36 - 31 Child Psychiatry 01 - 31.1 Introduction Infant, Child, and Adolesce 31.1 Introduction: Infant, Child, and Adolescent Development Child Psychiatry 31.1 Introduction: Infant, Child, and Adolescent Development The transactional nature of development in infancy, childhood, and adolescence, consisting of a continuous interplay between biological predisposition and environmental experiences, forms the basis of current conceptualizations of development. There is much evidence that observed developmental outcomes evolve from interactions between particular biological substrates and specific environmental events. For example, the serotonin transporter gene sensitizes a child with early adverse experiences of abuse or neglect to increased risk for later development of a depressive disorder. In addition, the degree of resilience and adaptation, that is, the ability to withstand adversity without negative effects, is likely to be mediated by endogenous glucocorticoids, cytokines, and neurotrophins. Thus, allostasis, the process of achieving stability in the face of adverse environmental events, results from interactions between specific environmental challenges and particular genetic backgrounds that combine to result in a response. It is widely accepted that adverse childhood experiences (ACEs) are likely to alter the trajectory of development in a given individual, and that during early development the brain is especially vulnerable to injury. Future studies may uncover windows of plasticity in older children and adolescents that affect vulnerability as well. Changes in both white matter and gray matter in the brains of adolescents are linked to increased acquisition of subtle social skills. Adolescents’ keen abilities, competencies, and interests in a host of technological advances—including the Internet, social media sites such as Facebook, Twitter, and Instagram, and smart phones, to name a few—shed some light on their potential to adapt to new and challenging demands. PRENATAL, INFANT, AND CHILD The phases of development described in this section are defined as follows: prenatal is the time frame from conception to 8 weeks; the fetus, from 8 weeks to birth; infancy, from birth to 15 months; the toddler period, from 15 months to 2½ years; the preschool period, from 2½ years to 6 years; and the middle years, from 6 to 12 years. PRENATAL Historically, the analysis of human development began with birth. The influence of endogenous and exogenous in utero factors, however, now requires that developmental schemes take intrauterine events into consideration. The infant is not a tabula rasa, a smooth slate upon which outside influences etch patterns. To the contrary, the newborn has already been influenced by myriad factors that have occurred in the safety of the womb, the result of which has produced wide individual differences among infants. For example, the studies of Stella Chess and Alexander Thomas (described later) have demonstrated a wide range of temperamental differences among newborns. Maternal stress, through the production of adrenal hormones, also influences behavioral characteristics of newborns. The time frame in which the development of the embryo and fetus occurs is known as the prenatal period. After implantation, the egg begins to divide and is known as an embryo. Growth and development occur at a rapid pace; by the end of 8 weeks, the shape is recognizably human, and the embryo has become a fetus. Figure 31.1-1 illustrates a sonogram of a 9-week and 15-week fetus in utero. FIGURE 31.1-1 A. Sonogram of fetus at 9 weeks. B. Same fetus at 15 weeks. (Courtesy K.C. Attwell, M.D.) The fetus maintains an internal equilibrium that, with variable effects, interacts continuously with the intrauterine environment. In general, most disorders that occur are multifactorial—the result of a combination of effects, some of which can be additive. Damage at the fetal stage usually has a more global impact than damage after birth, because rapidly growing organs are the most vulnerable. Boys are more vulnerable to developmental damage than girls are; geneticists recognize that in humans and animals, female fetuses show a propensity for greater biological vigor than male fetuses, possibly because of the second X chromosome in the female. Prenatal Life Much biological activity occurs in utero. A fetus is involved in a variety of behaviors that are necessary for adaptation outside the womb. For example, a fetus sucks on thumb and fingers; folds and unfolds its body, and eventually assumes a position in which its occiput is in an anterior vertex position, which is the position in which fetuses usually exit the uterus. Behavior. Pregnant women are extraordinarily sensitive to prenatal movements. They describe their unborn babies as active or passive, as kicking vigorously or rolling around, as quiet when the mothers are active, but as kicking as soon as the mothers try to rest. Women usually detect fetal movements 16 to 20 weeks into the pregnancy; the fetus can be artificially set into total body motion by in utero stimulation of its ventral skin surfaces by the 14th week. The fetus may be able to hear by the 18th week, and it responds to loud noises with muscle contractions, movements, and an increased heart rate. Bright light flashed on the abdominal wall of the 20-week pregnant woman causes changes in fetal heart rate and position. The retinal structures begin to function at that time. Eyelids open at 7 months. Smell and taste are also developed at this time, and the fetus responds to substances that may be injected into the amniotic sac, such as contrast medium. Some reflexes present at birth exist in utero: the grasp reflex, which appears at 17 weeks; the Moro (startle) reflex, which appears at 25 weeks; and the sucking reflex, which appears at about 28 weeks. Nervous System. The nervous system arises from the neural plate, which is a dorsal ectodermal thickening that appears on about day 16 of gestation. By the sixth week, part of the neural tube becomes the cerebral vesicle, which later becomes the cerebral hemispheres (Fig. 31.1-2). FIGURE 31.1-2 Formation of the neural tube and neural crest. These schematic illustrations follow the early development of the nervous system in the embryo. The drawings above are dorsal views of the embryo; those below are cross sections. A. The primitive embryonic central nervous system (CNS) begins as a thin sheet of ectoderm. B. The first important step in the development of the nervous system is the formation of the neural groove. C. The walls of the groove, called neural folds, come together and fuse, forming the neural tube. D. The bits of neural ectoderm that are pinched off when the tube rolls up are called the neural crest, from which the peripheral nervous system (PNS) will develop. The somites are mesoderm that will give rise to much of the skeletal system and the muscles. (Reprinted from Bear MF, Conners BW, Paradiso MA, eds. Neuroscience: Exploring the Brain. 2nd ed. Philadelphia: Lippincott Williams & Wilkins. 2001:179, with permission.) The cerebral cortex begins to develop by the 10th week, but layers do not appear until the sixth month of pregnancy; the sensory cortex and the motor cortex are formed before the association cortex. Some brain function has been detected in utero by fetal encephalographic responses to sound. The human brain weighs about 350 g at birth and 1,450 g at full adult development, a fourfold increase, mainly in the neocortex. This increase is almost entirely because of the growth in the number and branching of dendrites establishing new connections. After birth, the number of new neurons is negligible. Uterine contractions can contribute to fetal neural development by causing the developing neural network to receive and transmit sensory impulses. Pruning Pruning refers to the programmed elimination during development of neurons, synapses, axons, and other brain structures from the original number, present at birth, to a lesser number. Thus, the developing brain contains structures and cellular elements that are absent in the older brain. The fetal brain generates more neurons than it will need for adult life. For example, in the visual cortex, neurons increase in number from birth to 3 years of age, at which point they diminish in number. Another example is that the adult brain contains fewer neural connections than were present during the early and middle years of childhood. Approximately twice as many synapses are present in certain parts of the cerebral cortex during early postnatal life than during adulthood. Pruning occurs to rid the nervous system of cells that have served their function in the development of the brain. Some neurons, for example, exist to produce neurotrophic or growth factors and are programmed to die—a process called apoptosis—when that function is fulfilled. The implication of these observations is that the immature brain can be vulnerable in locations that lack sensitivity to injury later on. The developing white matter of the human brain before 32 weeks of gestation is especially sensitive to damage from hypoxic and ischemic injury and metabolic insults. Neurotransmitter receptors located on synaptic terminals are subject to injury from excessive stimulation by excitatory amino acids, (e.g., glutamate, aspartate), a process referred to as excitotoxicity. Research is proceeding on the implications of such events in the etiology of child and adult neuropsychiatric disorders such as schizophrenia. Maternal Stress Maternal stress correlates with high levels of stress hormones (epinephrine, norepinephrine, and adrenocorticotropic hormone) in the fetal bloodstream, which act directly on the fetal neuronal network to increase blood pressure, heart rate, and activity level. Mothers with high levels of anxiety are more likely to have babies who are hyperactive, irritable, and of low birth weight, and who have problems feeding and sleeping than are mothers with low anxiety levels. A fever in the mother causes the fetus’s temperature to rise. Genetic Disorders In many cases, genetic counseling depends on prenatal diagnosis. The diagnostic techniques used include amniocentesis (transabdominal aspiration of fluid from the amniotic sac), ultrasound examinations, x-ray studies, fetoscopy (direct visualization of the fetus), fetal blood and skin sampling, chorionic villus sampling, and α-fetoprotein screening. In about 2 percent of women tested, the results are positive for some abnormality, including X-linked disorders, neural tube defects (detected by high levels of α-fetoprotein), chromosomal disorders (e.g., trisomy 21), and various inborn errors of metabolism (e.g., Tay-Sachs disease and lipoidoses). Figure 31.1-3 illustrates hypertelorism of the eyes. FIGURE 31.1-3 Hypertelorism. Note the wide distance between the eyes, flat nasal bridge, and external strabismus. (Courtesy of Michael Malone, M.D. Children’s Hospital, Washington, D.C.) Some diagnostic tests carry a risk; for instance, about 5 percent of women who undergo fetoscopy miscarry. Amniocentesis, which is usually performed between the 14th and 16th weeks of pregnancy, causes fetal damage or miscarriage in less than 1 percent of women tested. Fully 98 percent of all prenatal tests in pregnant women reveal no abnormality in the fetus. Prenatal testing is recommended for women older than 35 years of age and for those with a family history of a congenital defect. Parental reactions to birth defects can include feelings of guilt, anxiety, or anger as their worst fears during the pregnancy are realized. Some degree of depression over the loss of the fantasized perfect child may be observed before the parents develop more active coping strategies. Termination of a pregnancy because of a known or suspected birth defect is an option chosen by some women. Maternal Drug Use Alcohol. Alcohol use in pregnancy is a major cause of serious physical and mental birth defects in children. Each year, up to 40,000 babies are born with some degree of alcohol-related damage. The National Institute on Drug Abuse (NIDA) reports that 19 percent of pregnant women used alcohol during their pregnancy, the highest rate being among white women. Fetal alcohol syndrome (Fig. 31.1-4) affects about one third of all infants born to alcoholic women. The syndrome is characterized by growth retardation of prenatal origin (height, weight); minor anomalies, including microphthalmia (small eyeballs), short palpebral fissures, midface hypoplasia (underdevelopment), a smooth or short philtrum, and a thin upper lip; and central nervous system (CNS) manifestations, including microcephaly (head circumference below the third percentile), a history of delayed development, hyperactivity, attention deficits, learning disabilities, intellectual deficits, and seizures. The incidence of infants born with fetal alcohol syndrome is about 0.5 per 1,000 live births. FIGURE 31.1-4 Photographs of children with “fetal-alcohol syndrome.” A. Severe case. B. Slightly affected child. Note in both children the short palpebral fissures and hypoplasia of the maxilla. Usually, the defect includes other craniofacial abnormalities. Cardiovascular defects and limb deformities are also common symptoms of the fetal alcohol syndrome. (From Langman J. Medical Embryology. 7th ed. Philadelphia: Williams & Wilkins; 1995:108, with permission.) Some studies suggest that alcohol use during pregnancy may contribute to attentiondeficit/hyperactivity disorder (ADHD). Animal experiments have shown that alcohol reduces the number of active dopamine neurons in the midbrain area, and ADHD is associated with reduced dopaminergic activity in the brain. Smoking. Smoking during pregnancy is associated with both premature births and below-average infant birth weight. Some reports have associated sudden infant death syndrome (SIDS) with mothers who smoke. Other Substances. Marijuana (used by 3 percent of all pregnant women) and cocaine (used by 1 percent) are the two most commonly abused illegal drugs, followed by heroin. Chronic marijuana use is associated with low infant birth weight, prematurity, and withdrawal-like symptoms, including excessive crying, tremors, and hyperemesis (severe and chronic vomiting). Crack cocaine use by women during pregnancy has been correlated with behavioral abnormalities such as increased irritability and crying and decreased desire for human contact. Infants born to mothers dependent on narcotics go through a withdrawal syndrome at birth. Prenatal exposure to various prescribed medications can also result in abnormalities. Common drugs with teratogenic effects include antibiotics (tetracyclines), anticonvulsants (valproate [Depakene], carbamazepine [Tegretol], phenytoin [Dilantin]), progesterone-estrogens, lithium (Eskalith), and warfarin (Coumadin). Table 31.1-1 outlines the etiologies of malformations that may emerge during the first year of life. Table 31.1-1 Causes of Human Malformations Observed During the First Year of Life INFANCY The delivery of the fetus marks the start of infancy. The average newborn weighs about 3,400 g (7.5 lb.). Small fetuses, defined as those with a birth weight below the 10th percentile for their gestational age, occur in about 7 percent of all pregnancies. At the 26th to the 28th week of gestation, the prematurely born fetus has a good chance of survival. Arnold Gesell described developmental landmarks that are widely used in both pediatrics and child psychiatry. These landmarks outline the sequence of children’s motor, adaptive, and personal–social behavior from birth to 6 years (Table 31.1-2). Table 31.1-2 Landmarks of Normal Behavioral Development Premature infants are defined as those with a gestation of less than 34 weeks or a birth weight less than 2,500 g (5.5 lb.). Such infants are at increased risk for learning disabilities, such as dyslexia, emotional and behavioral problems, mental retardation, and child abuse. With each 100 g increment of weight, beginning at about 1,000 g (2.2 lb.), infants have a progressively better chance of survival. A 36-week-old fetus has less chance of survival than a 3,000 g (6.6 lb) fetus born close to term. The differences between full-term and infants born prematurely are shown in Figure 31.1-5. FIGURE 31.1-5 Contrast between full-term (A and B) and premature (C and D) infants. Note the limp sprawl of the baby in C and the difficulty in raising the head to clear the nose and mouth in D. (Reprinted from Stone LJ, Church J. Childhood and Adolescence. 4th ed. New York: Random House; 1979:7, with permission.) Postmature infants are defined as infants born 2 weeks or more beyond the expected date of birth. Because pregnancy at term is calculated as extending 40 weeks from the last menstrual period and the exact time of fertilization varies, the incidence of postmaturity is high if based on menstrual history alone. The postmature baby typically has long nails, scanty lanugo, more scalp hair than usual, and increased alertness. Developmental Milestones in Infants Reflexes and Survival Systems at Birth. Reflexes are present at birth. They include the rooting reflex (puckering of the lips in response to perioral stimulation), the grasp reflex, the plantar (Babinski) reflex, the knee reflex, the abdominal reflexes, the startle (Moro) reflex (Fig. 31.1-6), and the tonic neck reflex. In normal children, the grasp reflex, the startle reflex, and the tonic neck reflex disappear by the fourth month. The Babinski reflex usually disappears by the 12th month. FIGURE 31.1-6 Moro reflex. (Reprinted with permission from Stone LJ, Church J. Childhood and Adolescence. 4th ed. New York: Random House 1979:14 with permission.) Survival systems—breathing, sucking, swallowing, and circulatory and temperature homeostasis—are relatively functional at birth, but the sensory organs are incompletely developed. Further differentiation of neurophysiological functions depends on an active process of stimulatory reinforcement from the external environment, such as persons touching and stroking the infant. The newborn infant is awake for only a short period each day; rapid eye movement (REM) and non-REM sleep are present at birth. Other spontaneous behaviors include crying, smiling, and penile erection in males. Infants 1 day old can detect the smell of their mother’s milk, and those 3 days old distinguish their mother’s voice. Language and Cognitive Development. At birth, infants can make noises, such as crying, but they do not vocalize until about 8 weeks. At that time, guttural or babbling sounds occur spontaneously, especially in response to the mother. The persistence and further evolution of children’s vocalizations depend on parental reinforcement. Language development occurs in well-delineated stages as outlined in Table 31.1-3. Table 31.1-3 Language Development By the end of infancy (about 2 years), infants have transformed reflexes into voluntary actions that are the building blocks of cognition. They begin to interact with the environment, to experience feedback from their own bodies, and to become intentional in their actions. By the end of the second year of life, children begin to use symbolic play and language. Jean Piaget (1896–1980), a Swiss psychologist, observed the growing capacity of young children (including his own) to think and to reason. An outline of the Piaget’s stages of cognitive development is presented in Table 31.1-4. Table 31.1-4 Piaget’s Stages of Cognitive Development Emotional and Social Development. By the age of 3 weeks, infants imitate the facial movements of adult caregivers. They open their mouths and thrust out their tongues in response to adults who do the same. By the third and fourth months of life, these behaviors are easily elicited. These imitative behaviors are believed to be the precursors of the infant’s emotional life. The smiling response occurs in two phases: the first phase is endogenous smiling, which occurs spontaneously within the first 2 months and is unrelated to external stimulation; the second phase is exogenous smiling, which is stimulated from the outside, usually by the mother, and occurs by the 16th week. The stages of emotional development parallel those of cognitive development. Indeed, the caregiving person provides the major stimulus for both aspects of mental growth. Human infants depend totally on adults for survival. Through warm and predictable interactions, an infant’s social and emotional repertoire expands with the interplay of caregivers’ social responses (Table 31.1-5). Table 31.1-5 Emotional Development In the first year, infants’ moods are highly variable and intimately related to internal states such as hunger. Toward the second two thirds of the first year, infants’ moods grow increasingly related to external social cues; a parent can get even a hungry infant to smile. When the infant is internally comfortable, a sense of interest and pleasure in the world and in its primary caregivers should prevail. Prolonged separation from the mother (or other primary caregiver) during the second 6 months of life can lead to depression that may persist into adulthood as part of an individual’s character. Temperamental Differences There are strong suggestions of inborn differences and wide variability in autonomic reactivity and temperament among individual infants. Chess and Thomas identified nine behavioral dimensions, in which reliable differences among infants can be observed (Table 31.1-6). Table 31.1-6 Temperament—Newborn to 6 Years Most temperamental dimensions of individual children showed considerable stability over a 25-year follow-up period, but some temperamental traits did not persist. This finding was attributed to genetic and environmental effects on personality. A complex interplay exists among the initial characteristics of infants, the mode of parental interactions, and children’s subsequent behavior. Observations of the stability and plasticity of certain temperamental traits support the importance of interactions between genetic endowment (nature) and environmental experience (nurture) in behavior. Attachment Bonding is the term used to describe the intense emotional and psychological relationship a mother develops for her baby. Attachment is the relationship the baby develops with its caregivers. Infants in the first months after birth become attuned to social and interpersonal interaction. They show a rapidly increasing responsivity to the external environment and an ability to form a special relationship with significant primary caregivers—that is, to form an attachment. Table 31.1-7 lists the commonly observed attachment styles. Table 31.1-7 Types of Attachment Harry Harlow. Harry Harlow studied social learning and the effects of social isolation in monkeys. Harlow placed newborn rhesus monkeys with two types of surrogate mothers—one a wire-mesh surrogate with a feeding bottle and the other a wire-mesh surrogate covered with terry cloth. The monkeys preferred the terry-cloth surrogates, which provided contact and comfort, to the feeding surrogate. (When hungry, the infant monkeys would go to the feeding bottle but then would quickly return to the terry-cloth surrogate.) When frightened, monkeys raised with terry-cloth surrogates showed intense clinging behavior and appeared to be comforted, whereas those raised with wire-mesh surrogates gained no comfort and appeared to be disorganized. The results of Harlow’s experiments were widely interpreted as indicating that infant attachment is not simply the result of feeding. Both types of surrogate-reared monkeys were subsequently unable to adjust to life in a monkey colony and had extraordinary difficulty learning to mate. When impregnated, the female monkeys failed to mother their young. These behavioral peculiarities were attributed to the isolates’ lack of mothering in infancy. John Bowlby. John Bowlby studied the attachment of infants to mothers and concluded that early separation of infants from their mothers had severe negative effects on children’s emotional and intellectual development. He described attachment behavior, which develops during the first year of life, as the maintenance of physical contact between the mother and child when the child is hungry, frightened, or in distress. Mary Ainsworth. Mary Ainsworth expanded on Bowlby’s observations and found that the interaction between mother and baby during the attachment period influences the baby’s current and future behavior significantly. Many observers believe that patterns of infant attachment affect future adult emotional relationships. Patterns of attachment vary among babies; for example, some babies signal or cry less than others. Sensitive responsiveness to infant signals, such as cuddling the baby when it cries, causes infants to cry less in later months. Close bodily contact with the mother when the baby signals for her is also associated with the growth of self-reliance, rather than clinging dependence, as the baby grows older. Unresponsive mothers produce anxious babies. Ainsworth also confirmed that attachment serves to reduce anxiety. What she called the secured base effect enables a child to move away from the attachment figure and explore the environment. Inanimate objects, such as a teddy bear or a blanket (called the transitional object by Donald Winnicott), also serve as a secure base, one that often accompanies children as they investigate the world. A growing body of literature derived from direct observation of mother–infant interactions and longitudinal studies has expanded on, and refined, Ainsworth’s original descriptions. Maternal sensitivity and responsiveness are the main determinants of secure attachment. But when the attachment is insecure, the type of insecurity (avoidant, anxious, or ambivalent) is determined by infant temperament. Overall, male infants are less likely to have secure attachments and are more vulnerable to changes in maternal sensitivity than are female infants. The attachment of the firstborn child is decreased by the birth of a second, but it is decreased much more when the firstborn is 2 to 5 years of age when the younger sibling is born than when the firstborn is younger than 24 months. Not surprisingly, the extent of the decrease also depends on the mother’s own sense of security, confidence, and mental health. Social Deprivation Syndromes and Maternal Neglect. Investigators, especially René Spitz, have long documented the severe developmental retardation that accompanies maternal rejection and neglect. Infants in institutions characterized by low staff-to-infant ratios and frequent turnover of personnel tend to display marked developmental retardation, even with adequate physical care and freedom from infection. The same infants, placed in adequate foster or adoptive care, exhibit marked acceleration in development. Fathers and Attachment. Babies become attached to fathers as well as to mothers, but the attachment is different. Generally, mothers hold babies for caregiving, and fathers hold babies for purposes of play. Given a choice of either parent after separation, infants usually go to the mother, but if the mother is unavailable they turn to the father for comfort. Babies raised in extended families or with multiple caregivers are able to establish many attachments. Stranger Anxiety. A developmentally expected fear of strangers is first noted in infants at about 26 weeks of age, and more fully developed by 32 weeks (8 months). At the approach of a stranger, infants cry and cling to their mothers. Babies exposed to only one caregiver are more likely to have stranger anxiety than babies exposed to a variety of caregivers. Stranger anxiety is believed to result from a baby’s growing ability to distinguish caregivers from all other persons. Separation anxiety, which occurs between 10 and 18 months of age, is related to stranger anxiety but is not identical to it. Separation from the person to whom the infant is attached precipitates separation anxiety. Stranger anxiety, however, occurs even when the infant is in the mother’s arms. The infant learns to separate as it starts to crawl and move away from the mother, but the infant constantly looks back and frequently returns to the mother for reassurance. Margaret Mahler (1897–1985) proposed a theory to describe how young children acquire a sense of identity separate from that of their mothers’. Her theory of separation–individuation was based on observations of the interactions of children and their mothers. Mahler’s stages of separation–individuation are outlined in Table 31.1-8. Table 31.1-8 Stages of Separation-Individuation Proposed by Mahler Infant Care Clinicians are now beginning to view infants as important actors in the family drama, ones who partly determine its course. Infants’ behavior controls mothers’ behavior, just as mothers’ behavior modulates infants’ behavior. A calm, smiling, predictable infant is a powerful reward for tender maternal care. A jittery, irregular, irritable infant tries a mother’s patience. When a mother’s capacity for giving is marginal, such infant traits may cause her to turn away from her child and thus complicate the child’s alreadytroubled beginnings. Parental Fit Parental fit describes how well the mother or father relates to the newborn or developing infant; the idea takes into account temperamental characteristics of both parent and child. Each newborn has innate psychophysiological characteristics, which are known collectively as temperament. Chess and Thomas identified a range of normal temperamental patterns, from the difficult child at one end of the spectrum to the easy child at the other end. Difficult children, who make up 10 percent of all children, have a hyperalert physiological makeup. They react intensely to stimuli (cry easily at loud noises), sleep poorly, eat at unpredictable times, and are difficult to comfort. Easy children, who make up 40 percent of all children, are regular in eating, eliminating, and sleeping; they are flexible, can adapt to change and new stimuli with a minimum of distress, and are easily comforted when they cry. The other 50 percent of children are mixtures of these two types. The difficult child is harder to raise and places greater demands on the parent than the easy child. Chess and Thomas used the term goodness of fit to characterize the harmonious and consonant interaction between a mother and a child in their motivations, capacities, and styles of behavior. Poor fit is likely to lead to distorted development and maladaptive functioning. A difficult child must be recognized, because parents of such infants often have feelings of inadequacy and believe that they are doing something wrong to account for the child’s difficulty in sleeping and eating and their problems comforting the child. In addition, most difficult children have emotional disturbances later in life. Good-Enough Mothering. Winnicott believed that infants begin life in a state of nonintegration, with unconnected and diffuse experiences, and that mothers provide the relationship that enables infants’ incipient selves to emerge. Mothers supply a holding environment in which infants are contained and experienced. During the last trimester of pregnancy and for the first few months of a baby’s life, the mother is in a state of primary maternal preoccupation, absorbed in fantasies about, and experiences with, her baby. The mother need not be perfect, but she must provide good-enough mothering. She plays a vital role in bringing the world to the child and offering empathic anticipation of the infant’s needs. If the mother can resonate with the infant’s needs, the baby can become attuned to its own bodily functions and drives that are the basis for the gradually evolving sense of self. TODDLER PERIOD The second year of life is marked by accelerated motor and intellectual development. The ability to walk gives toddlers some control over their own actions; this mobility enables children to determine when to approach and when to withdraw. The acquisition of speech profoundly extends their horizons. Typically, children learn to say “no” before they learn to say “yes.” Toddlers’ negativism is vital to the development of independence, but if it persists, oppositional behavior connotes a problem. Learning language is a crucial task in the toddler period. Vocalizations become distinct, and toddlers can name a few objects and make needs known in one or two words. Near the end of the second year and into the third year, toddlers sometimes use short sentences. The pace of language development varies considerably from child to child, and although a small number of children are truly late developers, most child experts recommend a hearing test if the child is not making two-word sentences by age 2. Developmental Milestones in Toddlers Language and Cognitive Development. Toddlers begin to listen to explanations that can help them tolerate delay. They create new behaviors from old ones (originality) and engage in symbolic activities, for instance, using words and playing with dolls when the dolls represent something, such as a feeding sequence. Toddlers have varied capacities for concentration and self-regulation. Emotional and Social Development. In the second year, pleasure and displeasure become further differentiated. Social referencing is often apparent at this age; the child looks to parents and others for emotional cues about how to respond to novel events. Toddlers show exploratory excitement, assertive pleasure, and pleasure in discovery and in developing new behavior (e.g., new games), including teasing and surprising or fooling the parent (e.g., hiding). The toddler has capacities for an organized demonstration of love, as when the toddler runs up and hugs, smiles, and kisses the parent at the same time, and of protest when the toddler turns away, cries, bangs, bites, hits, yells, and kicks. Comfort with family and apprehension with strangers may increase. Anxiety appears to be related to disapproval and the loss of a loved caregiver and can be disorganizing. Sexual Development. Sexual differentiation is evident from birth, when parents start dressing and treating infants differently because of the expectations evoked by sex typing. Through imitation, reward, and coercion, children assume the behaviors that their cultures define as appropriate for their sexual roles. Children exhibit curiosity about anatomical sex. When their curiosity is recognized as healthy and is met with honest, age-appropriate replies, children acquire a sense of the wonder of life and are comfortable with their own roles. If the subject of sex is taboo and children’s questions are rebuffed, shame and discomfort may result. Gender identity, the conviction of being male or female, begins to manifest at 18 months of age and is often fixed by 24 to 30 months. It was once widely believed that gender identity was primarily a function of social learning. John Money reported on children with ambiguous or damaged external genitalia who were raised as the sex opposite to their chromosomal sex. Long-term follow-up of those individuals suggests that the major part of gender identity is innate and that rearing may not affect the genetic diathesis. Gender role describes the behavior that society deems appropriate for one sex or another, and it is not surprising that significant cultural differences exist. There may be different expectations for boys and girls in what and with whom they play, their tone of voice, the expression of emotions, and how they dress. Nevertheless, some generalizations are possible. Boys are more likely than girls to engage in rough and tumble play. Mothers talk more to girls than to boys, and by the time the child is 2 years of age, fathers generally pay more attention to boys. Many educated, middle-class parents determined to raise nonsexist children are startled to see their children’s determined preference for sex-stereotyped toys: girls want to play with dolls, and boys with guns. Toilet Training. The second year of life is a period of increasing social demands on children. Toilet training serves as a paradigm of the family’s general training practices; that is, the parent who is overly severe in the area of toilet training is likely to be punitive and restrictive in other areas also. Control of daytime urination is usually complete by the age of 2½, and control of nighttime urination is usually complete by the age of 4 years, when bowel control is usually accomplished. Since 1900, the pendulum has swung between extremes of permissiveness and control in toilet training. The trend in the United States has been toward delayed training, but in the last few years this trend appears to be shifting back to early training. Toddlers may have sleep difficulties related to fear of the dark, which can often be managed by using a nightlight. Most toddlers generally sleep about 12 hours a day, including a 2-hour nap. Parents must be aware that children of this age may need reassurance before going to bed and that the average 2-year-old takes about 30 minutes to fall asleep. Parenting Challenges. In infancy, the major responsibility for parents is to meet the infant’s needs in a sensitive and consistent fashion. The parental task in the toddler stage requires firmness about the boundaries of acceptable behavior and encouragement of the child’s progressive emancipation. Parents must be careful not to be too authoritarian at this stage; children must be allowed to operate for themselves and to learn from their mistakes and must be protected and assisted when challenges are beyond their abilities. During the toddler period, children are likely to struggle for the exclusive affection and attention of their parents. This struggle includes rivalry, both with siblings and with one or another parent for the star role in the family. Although children are beginning to be able to share, they do so reluctantly. When the demands for exclusive possession are not resolved effectively, the result is likely to be jealous competitiveness in relationships with peers and lovers. The fantasies aroused by the struggle lead to fear of retaliation and to displacement of fear onto external objects. In an equitable, loving family a child elaborates a moral system of ethical rights. Parents need to balance between punishment and permissiveness and set realistic limits on a toddler’s behavior. PRESCHOOL PERIOD The preschool period is characterized by marked physical and emotional growth. Generally, between 2 and 3 years of age, children reach half their adult height. The 20 baby teeth are in place at the beginning of the stage, and by the end they begin to fall out. Children are ready to enter school by the time the stage ends at age 5 or 6. They have mastered the tasks of primary socialization—to control their bowels and urine, to dress and feed themselves, and to control their tears and temper outbursts, at least most of the time. The term preschool for the age group of 2½ to 6 years may be a misnomer; many children are already in school-like settings, such as preschool nurseries and day care centers, where working mothers must often place their children. Preschool education can be valuable, but stressing academic advancement too far beyond a child’s capabilities can be counterproductive. Developmental Milestones in Preschoolers Language and Cognitive Development. In the preschool period, children’s use of language expands, and they use sentences. Individual words have regular and consistent meanings at the beginning of the period, and children begin to think symbolically. In general, however, their thinking is egocentric; they cannot place themselves in the position of another child and are incapable of empathy. Children think intuitively and prelogically and do not understand causal relations. Emotional and Social Behavior. At the start of the preschool period, children can express such complex emotions as love, unhappiness, jealousy, and envy, both preverbally and verbally. Their emotions are still easily influenced by somatic events, such as tiredness and hunger. Although they still think mostly egocentrically, children’s capacity for cooperation and sharing is emerging. Anxiety is related to loss of a person who was loved and depended on and to loss of approval and acceptance. Although still potentially disorganizing, anxiety can be tolerated better than in the past. Four-yearolds are learning to share and to have concern for others. Feelings of tenderness are sometimes expressed. Anxiety over bodily injury and the loss of a loved person’s approval is sometimes disruptive. By the end of the preschool period, children have many relatively stable emotions. Expansiveness, curiosity, pride, and gleeful excitement related to the self and the family are balanced with coyness, shyness, fearfulness, jealousy, and envy. Shame and humiliation are evident. Capacities for empathy and love are developed but are fragile and easily lost if competitive or jealous strivings intervene. Anxiety and fears are related to bodily injury and loss of respect, love, and emerging self-esteem. Guilt feelings are possible. Children between the ages of 3 and 6 years are aware of their bodies, and of differences between the sexes. In their play, doctor–nurse games allow children to act out their sexual fantasies. Their awareness of their bodies extends beyond the genitalia; they show a preoccupation with illness or injury, so much so that the period has been called “the Band-Aid phase.” Every injury must be examined and taken care of by a parent. Children develop a division between what they want and what they are told to do. The division increases until a gap grows between their set of expanded desires, their exuberance at unlimited growth, and their parents’ restrictions; they gradually turn parental values into self-obedience, self-guidance, and self-punishment. At the end of the preschool stage, the child’s conscience is evolving. The development of a conscience sets the tone for the moral sense of “right and wrong.” Until about 7 years of age, children typically experience rules as “absolute” and as existing for their own sake. They do not understand that more than one point of view on a moral issue may exist; a violation of the rules calls for absolute retribution—that is, children have the notion of immanent justice. SIBLING RIVALRY. In the preschool period, children relate to others in new ways. The birth of a sibling (a common occurrence during this time) tests a preschool child’s capacity for further cooperation and sharing but may also evoke sibling rivalry, which is most likely to occur at this time. Sibling rivalry depends on child-rearing practice. Favoritism for any reason commonly aggravates such rivalry. Children who get special treatment because they are gifted, are defective in some way, or have a preferred gender are likely to receive angry feelings from their siblings. Experiences with siblings can influence growing children’s relationships with peers and authority; for example, a problem may result if the needs of a new baby prevent the mother from attending to a firstborn child’s needs. If not handled properly, the displacement of the firstborn can be a traumatic event. PLAY. In the preschool years, children begin to distinguish reality from fantasy, and play reflects this growing awareness. Pretend games are popular and help test real-life situations in a playful manner. Dramatic play in which children act out a role, such as a housewife or a truck driver, is common. One-to-one play relationships advance to complicated patterns with rivalries, secrets, and two-against-one intrigues. Children’s play behavior reflects their level of social development. Between 2½ and 3 years, children commonly engage in parallel play, solitary play alongside another child with no interaction between them. By age 3, play is often associative, that is, playing with the same toys in pairs or in small groups, but still with no real interaction among them. By age 4, children are usually able to share and engage in cooperative play. Real interactions and taking turns become possible. Between 3 and 6 years of age, growth can be traced through drawings. A child’s first drawing of a human being is a circular line with marks for the mouth, nose, and eyes; ears and hair are added later; arms and stick-like fingers appear next; and then legs appear. Last to appear is a torso in proportion to the rest of the body. Intelligent children can deal with details in their art. Drawings express creativity throughout a child’s development: They are representational and formal in early childhood, make use of perspective in middle childhood, and become abstract and affect-laden in adolescence. Drawings also reflect children’s body image concepts and sexual and aggressive impulses. IMAGINARY COMPANIONS. Imaginary companions most often appear during preschool years, usually in children with above-average intelligence and usually in the form of persons. Imaginary companions may also be things, such as toys that are anthropomorphized. Some studies indicate that up to 50 percent of children between the ages of 3 and 10 years have imaginary companions at one time or another. Their significance is not clear, but these figures are usually friendly, relieve loneliness, and reduce anxiety. In most instances, imaginary companions disappear by age 12, but they can occasionally persist into adulthood. MIDDLE YEARS The period between age 6 and puberty is often called the middle years. During this time, children enter elementary school. The formal demands for academic learning and accomplishment become major determinants of further personality development. Developmental Milestones in School-age Children Language and Cognitive Development. In the middle years, language expresses complex ideas with relations among several elements. Logical exploration tends to dominate fantasy, and children show an increased interest in rules and orderliness and an increased capacity for self-regulation. During this period, children’s conceptual skills develop, and thinking becomes organized and logical. The ability to concentrate is well established by age 9 or 10, and by the end of the period, children begin to think in abstract terms. Improved gross motor coordination and muscle strength enable children to write fluently and draw artistically. They are also capable of complex motor tasks and activities, such as tennis, gymnastics, golf, baseball, and skateboarding. Recent evidence has shown that changes in thinking and reasoning during the middle years result from maturational changes in the brain. Children are now capable of increased independence, learning, and socialization. Theorists consider moral development a gradual, stepwise process spanning childhood, adolescence, and young adulthood. In the middle years, both girls and boys make new identifications with other adults, such as teachers and counselors. These identifications may so influence girls that their goals of wanting to marry and have babies, as their mothers did, may be combined with a desire for a career or may be postponed or abandoned entirely. Girls who cannot identify with their mothers or whose fathers are overly attached may become fixated at about a 6-year-old level; as a result, they may fear men or women or both or become seductively close to them. In either case, such girls may not be seen as normal during the school-age years. A similar situation can occur in boys who have been unable to identify successfully with fathers who were aloof, brutal, or absent. Perhaps his mother prevented a boy from identifying with his father by being overprotective or by binding the son too closely to her. As a result, boys may enter this period with a variety of problems. They may be fearful of men, unsure of their sense of masculinity, or unwilling to leave their mothers (sometimes manifested by a school phobia); they may lack initiative and be unable to master school tasks, thus incurring academic problems. The school-age period is a time when peer interaction assumes major importance. Interest in relationships outside the family takes precedence over those within the family. Nevertheless, a special relationship exists with the same-sex parent, with whom children identify and who is now an ideal and a role model. Empathy and concern for others begin to emerge early in the middle years; by the time children are 9 or 10, they have well-developed capacities for love, compassion, and sharing. They have a capacity for long-term, stable relationships with family, peers, and friends, including best friends. Emotions about sexual differences begin to emerge as either excitement or shyness with the opposite sex. School-age children prefer to interact with children of the same sex. Although the middle years have sometimes been referred to as a latency period—a moratorium on psychosexual exploration and play until the eruption of sexual impulses with puberty—it is now recognized that a considerable amount of sexual interest continues through these years. Sex play and curiosity are common, especially among boys, but also among girls. Boys compare genitals and sometimes engage in group or mutual masturbation. An interest in anal humor and toilet jokes is often seen. Children this age often start using sexual and excretory words as expletives. BEST FRIEND. Harry Stack Sullivan postulated that a buddy, or best friend, is an important phenomenon during the school years. By about 10 years of age, children develop a close same-sex relationship, which Sullivan believed is necessary for further healthy psychological growth. Moreover, Sullivan believed that the absence of a chum during the middle years of childhood is an early harbinger of schizophrenia. SCHOOL REFUSAL. Some children refuse to go to school at this time, generally because of separation anxiety. A fearful mother may transmit her own fear of separation to a child, or a child who has not resolved dependence needs panics at the idea of separation. School refusal is usually not an isolated problem; children with the problem typically avoid many other social situations. Sex Role Development Persons’ sex roles are similar to their gender identity; persons see themselves as male or female. The sex role also involves identification with culturally acceptable masculine or feminine ways of behaving; but changing expectations in society (particularly in the United States) of what constitutes masculine and feminine behavior can create ambiguity. Parents react differently to their male and female children. Independence, physical play, and aggressiveness are encouraged in boys; dependence, verbalization, and physical intimacy are encouraged in girls. Nowadays, however, boys are encouraged to verbalize their feelings and to pursue interests traditionally associated with girls, whereas girls are encouraged to pursue careers traditionally dominated by men and to participate in competitive sports. As society grows more tolerant in its expectations of the sexes, roles become less rigid, and opportunities for boys and girls enlarge and broaden. Biologically, boys are more physically aggressive than girls; and parental expectations, particularly the expectations of fathers, reinforce this trait. Differences also exist between boys and girls in the influence of persons outside the family. Girls tend to respond to the expectations and opinions of girls and of teachers of either sex, but to ignore boys. Boys, on the other hand, tend to respond to other boys, but to ignore girls and teachers. Dreams and Sleep Children’s dreams can have a profound effect on behavior. During the first year of life, when reality and fantasy are not yet fully differentiated, dreams may be experienced as if they were, or could be, true. At age 3, many children believe dreams are shared directly by more than one person, but most 4-year-olds understand that dreams are unique to each person. Children view dreams either with pleasure or, as is most often reported, with fear. The dream content should be seen in connection with children’s life experience, developmental stage, mechanisms used during dreaming, and sex. Disturbing dreams peak when children are 3, 6, and 10 years of age. Two-year-old children may dream about being bitten or chased; at the age of 4, they may have many animal dreams and also dream of persons who either protect or destroy. At age 5 or 6, dreams of being killed or injured, of flying and being in cars, and of ghosts become prominent; the role of conscience, moral values, and increasing conflicts are concerned with these themes. In early childhood, aggressive dreams rarely seem to occur; instead, dreamers are in danger, a state that perhaps reflects children’s dependent position. By about the age of 5, children realize that their dreams are not real; before then, they believed them to be real events. By age 7, children know that they create their dreams themselves. Between the ages of 3 and 6 years, children normally want to keep their bedroom door open or to have a nightlight, so that they can either maintain contact with their parents or view the room in a realistic, nonfearful way. At times, children resist going to sleep to avoid dreaming. Disorders associated with falling asleep, therefore, are often connected with dreaming. Children often create rituals to protect themselves in the withdrawal from the world of reality into the world of sleep. Parasomnias, such as sleepwalking, sleep talking, enuresis (bed-wetting), and night terrors, are common at this age. They usually occur during stage 4 sleep when dreaming is minimal, and they do not indicate emotional trouble or underlying psychopathology. Most children grow out of parasomnias by adolescence. Periods of REM occur about 60 percent of the time during the first few weeks of life, a period when infants sleep two thirds of the time. Premature babies sleep even longer than full-term babies, and a greater proportion of their sleep is REM sleep. The sleep– wake cycle of newborns is about 3 hours long. Among adults, the dream-to-sleep ratio is stable: 20 percent of sleeping time is spent dreaming. Even newborns have brain activity similar to that of the dreaming state. Birth Spacing For women in the United States, 10 percent of conceptions that lead to live births are considered unwanted, and 20 percent are wanted but considered ill timed. Children born close together have higher rates of premature or underweight births, and malnutrition; they develop more slowly and are at increased risk of contracting and dying from childhood infectious diseases. Studies have shown when a child is born 3 to 5 years after a previous birth, health risks are reduced for both mother and child. Compared with 24- to 29-month intervals, children born in 36- to 41-month intervals are associated with a 28 percent reduction in stunting and a 29 percent reduction of low birth weight. Women who have children at 27- to 32-month intervals are 1.3 times more likely to avoid anemia, 1.7 times more likely to avoid third-trimester bleeding, and 2.5 times more likely to survive childbirth. Birth Order The effects of birth order vary. Firstborn children are often more highly valued and given more attention than subsequent children. Firstborn children appear to be more achievement oriented and motivated to please their parents than subsequent children born to the same parents. Some studies show that people in certain competitive occupational areas, such as architecture, accounting, and engineering, tend to be firstborn children. Second and third children have the advantage of their parents’ previous experience. Younger children also learn from their older siblings. For example, they may show more sophisticated use of pronouns at an earlier age than firstborns did. When children are spaced too closely, however, there may not be enough time for each child. The arrival of new children in the family affects not only the parents but also the siblings. Firstborn children may resent the birth of a new sibling, who threatens their sole claim on parental attention. In some cases, regressive behavior, such as enuresis or thumbsucking, occurs. According to Frank Sulloway, firstborn children tend to be conservative and conformists; by contrast, youngest children tend to be independent and rebellious in regard to family and cultural norms. Sulloway found that a high proportion of prominent persons were lastborn children. He ascribes these differences to birth order and suggests that each child develops personality traits to fit an unfilled slot in the family. His findings need to be replicated. Children and Divorce Many children live in homes in which divorce has occurred. Approximately 30 to 50 percent of all children in the United States live in homes in which one parent (usually the mother) is the sole head of the household, and 61 percent of all children born in any given year can expect to live with only one parent before they reach the age of 18 years. A child’s age at the time of the parents’ divorce affects the child’s reaction to the divorce. Immediately after a divorce, an increase in behavioral and emotional disorders appears in all age groups. Infants do not understand anything about separation or divorce; however, they do notice changes in their parents’ responses to them and may experience changes in their eating or sleeping patterns; have bowel problems; and seem more fretful, fearful, or anxious. Children 3- to 6-years of age may not understand what is happening, and those who do understand often assume that they are somehow responsible for the divorce. Older children, especially adolescents, comprehend the situation and may believe that they could have prevented the divorce had they intervened in some way, but they are still hurt, angry, and critical of their parents’ behavior. Some children harbor the fantasy that their parents will be reunited in the future. Such children may show animosity toward a parent’s real or potential new mate because they are faced with the reality that reconciliation between their parents is not taking place. Adaptation to the effects of divorce in children typically takes several years; however, up to about one third of children from divorced homes may have lasting psychological trauma. Among boys, physical aggression is a common sign of distress. Adolescents tend to spend more time away from the parental home after the divorce. Children who adapt best to divorce are typically in a situation in which both parents make genuine efforts to spend time and relate to the child despite the child’s potential anger about the divorce. To facilitate adaptation in children, a divorced couple who are amicable, and avoid arguing with one another is most likely to succeed. Table 31.1-9 lists potential psychological effects of divorce on children. Table 31.1-9 Effects of Divorce on Children Stepparents. Although there are many different scenarios that may occur after a divorce and remarriage, several potential scenarios have been outlined in Table 31.1-10. These include: (1) Neo-traditional, (2) Romantic, and (3) Matriarchal. When remarriage occurs, children must learn to adapt to the stepparent and to the “blended” family. Adaptation is often challenging, especially when a child feels that a stepparent is nonsupportive, resents the stepchild, or favors his or her own natural children. Of stepfamilies, 25 percent tend to dissolve within the first 2 years, whereas 75 percent grow to find a new balance in their blended family. A biological child born to a new couple with a stepchild already in the home may receive more attention than the stepchild, leading to of sibling rivalry. After 5 years, about 20 percent of adolescents in step-families suggest that they move out and try living with their other biological parent. Table 31.1-10 Types of Step-Families Family Factors in Child Development Family Stability. Parents and children living under the same roof in harmonious interaction is the expected cultural norm in Western society. Within this framework, childhood development presumably proceeds most expeditiously. Deviations from the norm, such as divorced- and single-parent families, are associated with a broad range of problems in children, including low self-esteem, increased risk of child abuse, and increased incidence of divorce when they eventually marry, and increased incidence of mental disorders, particularly depressive disorders and antisocial personality disorder as adults. Why some children from unstable homes are less affected than others (or even immune to these deleterious effects) is of great interest. Michael Rutter has postulated that vulnerability is influenced by sex (boys are more affected than girls), age (older children are less vulnerable than younger ones), and inborn personality characteristics. For example, children who have a placid temperament are less likely to be victims of abuse within a family than are hyperactive children; by virtue of their placidity, they may be less affected by the emotional turmoil surrounding them. Adverse Events. It is now well known that significant adverse events, especially in early childhood such as sexual and physical abuse, neglect, or loss of a parent, interact with genetic background in a given child and influences the trajectory of development. For example, as mentioned earlier early severe maltreatment such as sexual abuse increases the risk of multiple psychosocial difficulties and emergence of many psychiatric disorders. Among young maltreated children, those with particular genetics, that is, who have the “short” variant of the serotonin transporter gene (short 5-HTTLPR polymorphism) are significantly more vulnerable to chronic depression in adulthood. This example of specific gene–environment interaction plays an important role in a child’s development as well as in the risk for future psychopathology. Current investigations are also seeking insight into what factors lead to resilience in youth who have been exposed to adverse events, yet maintain allostasis, that is, stability in the face of stressful events. Hormones of the adrenal glands, thyroid, gonads, as well as metabolic hormones play a role on the brain’s ability to maintain stability upon exposure to stress, and the prefrontal cortex, hippocampus, and amygdala play critical roles in regulating emotionality, aggression, and resilience. Day Care Centers. The role of day care centers for children is under continuous investigation, and various studies have produced different results. One study found that children placed in day care centers before the age of 5 are less assertive and less effectively toilet trained than home-reared children. Another study found children in day care to be more advanced in social and cognitive development than children who were not in day care. The National Institute of Child Health and Human Development reported that 4½ year olds who had spent more than 30 hours a week in child care were more demanding, more aggressive, and more noncompliant than those raised at home and showed higher cognitive skills, particularly in math and reading. These same children who were tracked through the third grade continued to score higher in math and reading skills but had poorer work habits and social skills. The researchers were careful to note that this behavior was within the normal range, however. All studies of day care must take into account the quality of both the day care center and the homes from which children come. For example, a child from a disadvantaged home may be better off at a day care center than a child from an advantaged home. Similarly, a woman who wishes to leave the home to work for financial or other reasons and cannot do so may resent being forced to remain in the home in a child-rearing role, which may adversely affect the child. Parenting Styles. The ways in which children are raised vary considerably between and within cultures. Rutter has clustered the diversity into four general styles. Subsequent research has confirmed that certain styles tend to correlate with certain behavior in the children, although the outcomes are by no means absolute. The authoritarian style, characterized by strict, inflexible rules, can lead to low self-esteem, unhappiness, and social withdrawal. The indulgent-permissive style, which includes little or no limit setting coupled with unpredictable parental harshness, can lead to low self-reliance, poor impulse control, and aggression. The indulgent-neglectful style, one of noninvolvement in the child’s life and rearing, puts the child at risk for low selfesteem, impaired self-control, and increased aggression. The authoritative-reciprocal style, marked by firm rules and shared decision-making in a warm, loving environment, is believed to be the style most likely to result in self-reliance, self-esteem, and a sense of social responsibility. Development and Expression of Psychopathology The expression of psychopathology in children can be related to both age and developmental level. Specific developmental disorders, particularly developmental language disorders, often are diagnosed in the preschool years. Delayed development of language is a common parental concern. Children who do not use words by 18 months or phrases by 2½ to 3 years may need assessment, particularly if they do not appear to understand normal verbal cues or much language at all. Mild mental retardation or specific learning problems often are not diagnosed until after the child begins elementary school. Disruptive behavior disorder will become apparent at that time as the child begins to interact with peers. Similarly, attention-deficit disorders are only diagnosed when the demands for sustained attention are made in school. Other conditions, particularly schizophrenia and bipolar disorder, are rare in preschool and school-aged children. ADOLESCENCE Adolescence, marked by the physiological signs and surging sexual hormones of puberty, is the period of maturation between childhood and adulthood. Adolescence is a transitional period in which peer relationships deepen, autonomy in decision-making grows, and intellectual pursuits and social belonging are sought. Adolescence is largely a time of exploration and making choices, a gradual process of working toward an integrated concept of self. Adolescents can best be described as “works in progress,” characterized by increasing ability for mastery over complex challenges of academic, interpersonal, and emotional tasks, while searching for new interests, talents, and social identities. A body of growing literature of the specific mechanisms of brain development in adolescence has increased our understanding of broadening social skills in adolescents, in addition to the three expected developmental changes in adolescence: increased risk taking, increased sexual behavior, and a move toward peer affiliation rather than primary family attachment. The total cortical gray matter is at its peak at about age 11 years in girls and 13 years in boys, which enhances the ability to understand subtle social situations, control impulses, make long range plans, and think ahead. White matter volume increases throughout childhood and adolescence, which may allow for increased “connectivity,” thereby enhancing the abilities of adolescents to acquire new competencies, such as those needed to master today’s technology. What is Normal Adolescence? The concept of normality in adolescent development refers to the degree of psychological adaptation that is achieved while navigating the hurdles and meeting the milestones characteristic of this period of growth. For up to approximately 75 percent of youth, adolescence is a period of successful adaptation to physical, cognitive, and emotional changes, largely continuous with their previous functioning. Psychological maladjustment, self-loathing, disturbance of conduct, substance abuse, affective disorders, and other impairing psychiatric disorders emerge in approximately 20 percent of the adolescent population. Adolescent adjustment is continuous with previous psychological function; thus, psychologically disturbed children are at greater risk for psychiatric disorders during adolescence. Adolescents with psychiatric disorders are at increased risk for greater conflicts with families and for feeling alienated from their families. Although up to 60 percent of adolescents endorse occasional distress, or a psychiatric symptom, this group of adolescents functions well academically and with peers and describes themselves as generally satisfied with their lives. The developmentalist Erik Erikson characterizes the normative task of adolescence as identity versus role confusion. The integration of past experiences with current changes takes place in what Erikson terms ego identity. Adolescents explore various aspects of their psychological selves by becoming fans of heroes, or other well-known musical or political idols. Some adolescents appear consumed by their identification with a particular idol, whereas others are more moderate in their expression. Adolescents who feel accepted by a peer group and are involved in a variety of activities are less likely to become consumed by adoration of an idol. Adolescents who are socially isolated, feel socially rejected, and become overly identified with an idol to the exclusion of all other activities are at greater risk for serious emotional problems and require psychiatric intervention. Erickson uses the term moratorium to describe that interim period between the concrete thinking of childhood and a more evolved complex ethical development. Erikson defines identity crisis as a normative part of adolescence in which adolescents pursue alternative behaviors and styles and, then, successfully mold these different experiences into a solid identity. A failure to do so would result in identity diffusion, or role confusion, in which the adolescent lacks a cohesive or confident sense of identity. Adolescence is the time to bond with peers, experiment with new beliefs and styles, fall in love for the first time, and explore creative ideas for future endeavors. Most adolescents go through this developmental process with optimism, develop good self-esteem, maintain good peer relationships, and sustain basically harmonious relationships with their families. Stages of Adolescence Early Adolescence. Early adolescence, from 12 to 14 years of age, is the period in which the most striking initial changes are noticed—physically, attitudinally, and behaviorally. Growth spurts often begin in these years for boys, whereas girls may have already had rapid growth for 1 to 2 years. At this stage, boys and girls begin to criticize usual family habits, insist on spending time with peers with less supervision, have a greater awareness of style and appearance, and may question previously accepted family values. A new awareness of sexuality may be displayed by increased modesty and embarrassment with their current physical development or may exhibit itself in an increased interest in the opposite sex. Early adolescents engage in subtle or overt displays of their growing desire for autonomy, sometimes with challenging behaviors toward authority figures, including teachers and school administrators, and exhibit disdain for rules themselves. At this age, some adolescents begin to experiment with cigarettes, alcohol, and marijuana. During early adolescence, there is normal variation in when new defining behaviors are acquired. Overall, although many early adolescents make new friends and modify their public image, most maintain positive connections to family members, old friends, and their family’s values. However, early adolescence has been viewed as a time of overwhelming turmoil, during which there is a dramatic rejection of family, friends, and lifestyle, resulting in a powerful alienation of the adolescent. Jake, a 13-year-old adolescent, had just started the 8th grade. In the past, he has been a jovial, fun-loving, and cooperative student, but this year he found the school rules increasingly irritating and felt that his teachers were too strict. He had always been a good student while putting in a minimum of work. His older brother Sean, now in 11th grade, had established himself as a compliant, well-liked, and well-behaved student who always put maximal effort into school projects in the same school, so Jake was compared with his brother on a regular basis by many teachers. Jake resented these comparisons because, unlike his brother, whom Jake felt was a “nerd,” Jake was more rebellious, took more risks, and made friends with more popular peers. To distinguish himself from his older brother in school and at home, Jake began to challenge the rules at school, stating that they were “stupid” and “meaningless.” Jake began to cut classes, to stay out late, and to experiment with alcohol and marijuana. He rejected his best friends from 6th and 7th grade, and began to hang out with peers who were more daring. When Jake was at home, he was able to relate to his older brother Sean only when they played basketball and video games. Jake’s grades began to deteriorate only slightly, but his parents noticed that on his report cards, his effort and behavior were rated as unsatisfactory. During the second month of school, Jake’s parents received a phone call that Jake was going to be suspended due to possession of a small amount of marijuana on the school grounds during recess. During a subsequent meeting with the assistant principal and school counselor, Jake argued that the suspension was unfair because his grades were still good, and did not understand why his marijuana possession had triggered a suspension. When confronted with the fact that he had not only broken the school rules, but also violated the law, and that he was fortunate because the school did not involve the police, Jake became angry and continued to insist that he was being treated unfairly. He also blurted out that all of his teachers and his parents favored his older brother Sean, and treated him like a second-class citizen. Jake was suspended for 5 days, but the school indicated that they would report the incident to the police unless Jake and his family initiated immediate counseling. Jake begrudgingly began psychotherapy and entered into a weekly therapy group specializing in substance use, for teens. Jake’s parents also sought therapy to work on becoming more unified in their parenting. Jake remained in psychotherapy for the next 1½ years, during which time his attitude and reasoning style changed and evolved considerably. At age 15, Jake was able to understand why his school had suspended him for possession of marijuana and came to appreciate their willingness to give him the chance to seek counseling, rather than be turned over to the police. Over time, Jake was able to admit the dangers of using drugs, and took responsibility for his ill-advised behaviors. Alcohol and drug use continued to be a focus of his therapy and, by 15, Jake had virtually lost interest in alcohol, and admitted to smoking marijuana rarely at parties. Jake became more open to making friends with a variety of peers, and he disclosed that he liked himself better now than when he was 13. He now treated his brother respectfully when alone or with friends, and he felt that his parents appreciated him for “who he was.” (Courtesy of Caroly S. Pataki, M.D.). Middle Adolescence. During the middle phase of adolescence (roughly between the ages of 14 and 16), adolescents’ lifestyles may reflect their efforts to pursue their own stated goals of being independent. Their abilities to combine abstract reasoning with realistic decision-making and the application of social judgment is put to the test in this phase of adolescent development. In this phase, sexual behavior intensifies, making romantic relationships more complicated, and self-esteem becomes a pivotal influence on positive and negative risk-taking behaviors. In this phase of development, adolescents tend to identify with a group of peers who become highly influential in their choices of activities, styles, music, idols, and role models. Adolescents’ underestimation of the risks associated with a variety of recreational behaviors and their sense of “omnipotence,” mixed with their drive to be autonomous, frequently cause some conflict with parental requests and expectations. For most teens, the process of defining themselves as unique and different from their families can be achieved while still maintaining alliances with family members. Jenna, a 16-year-old junior in high school, had just gotten her driver’s license. She realized that she was lucky to have been given a brand new car at 16, because many of her friends did not yet have cars, she was upset that her parents disapproved of her agreement to drive all of her friends to places that she did not even want to go. Jenna was an attractive and well-liked adolescent who had always been an “A” and “B” student, and she and her family had never had conflicts about school. She played the flute in the school’s orchestra, and was not involved in any team sports. Jenna started “going out” with a boy in her grade at school, Brett, who was also 16 years old, shortly after she got her license, and even though they didn’t know each other that well, she felt that they had a close relationship. Since he did not yet have a car, she was the “identified driver” whenever they went out or to parties. Jenna was glad about this, because she didn’t really like alcohol and was relieved that Brett would not be driving, given that he like to drink quite a bit at parties. Jenna got along fairly well with her parents, who were considered very “easy-going” by her friends, and she felt that she and her parents had similar values and ideas. Things were going well until Brett began to pressure her to go further in their sexual relationship. When Jenna told him that she wasn’t ready, Brett hounded her more. When the subject of sex had come up with her parents “hypothetically” in the past, they had dismissed the subject, indicating that when it was the right time for her, Jenna would know. Jenna knew that she was not ready to have sex, although many of her classmates were sexually active. Jenna was not an impulsive person and liked to plan things carefully so that they would feel right to her. Jenna realized that she could not agree with Brett’s request but she was confident that she could make him understand. One of Jenna’s friends suggested that Brett might break up with her if she didn’t have sex with him, but Jenna was willing to take that risk. Jenna carefully told Brett that she loved him but she was not yet ready for sex. Jenna was slightly surprised that instead of pressuring her more, or breaking up with her, Brett accepted her decision, in fact, he seemed a little relieved. Jenna and Brett continued their relationship into their senior year of high school, and, toward the end of her senior year, Jenna desired to be sexually active with him. They decided to go to a community clinic known for its positive attitude toward adolescents, to learn about birth control methods and pick one, without the including their parents. Jenna and Brett took the time to learn about a variety of birth control methods and chose to use condoms. When they left the clinic, Jenna and Brett felt closer than they had before, and realized how they had both grown in their relationship. Jenna and Brett both felt that they were doing the right thing. (Courtesy of Caroly S. Pataki, M.D.). Late Adolescence. Late adolescence (between the ages of 17 and 19) is a time when continued exploration of academic pursuits, musical and artistic tastes, athletic participation, and social bonds lead a teen toward greater definition of self and a sense of belonging to certain groups or subcultures within mainstream society. Well-adjusted adolescents can be comfortable with current choices of activities, tastes, hobbies, and friendships, yet remain aware that their “identities” will continue to be refined during young adulthood. Joey was in his second semester of his freshman year of college, living away from home, and had just turned 18 years of age. He reflected on the fact that he was no longer a “minor” and could make almost any decision for himself without his parents being involved. Joey felt liberated, but at the same time, he was confused and a little lost. Since 10th grade, Joey had planned to pursue a career in medicine like his father, so he had taken a heavy load of science courses in the first semester, all of which he had despised. This semester, however, he had signed up only for liberal arts classes. He did not mention this to his father. He was now enrolled in classes that ranged from art history to architectural drafting to sociology, philosophy, and music. He had been influenced, he believed, by his roommate Tony, who was in the architecture program, and by his girlfriend, Lisa, who was majoring in studio art. As the semester progressed, Joey found that his favorite course was the drafting class, just like Tony had predicted. Tony was in a more advanced drafting class than Joey, and Joey couldn’t help but wonder whether he liked the drafting class so much because of how much he idolized Tony, or because he really enjoyed the class. He talked this over with Lisa, who suggested that he chill out and not figure out the rest of his life right now. She recommended that he take at least two more semesters of varied classes including those in the architecture curriculum before making a final decision about a career. Joey realized that Lisa’s approach to college, and to life was so relaxed, the opposite of his approach, following his parents’ pressure to plan ahead, make commitments early, and see them through, regardless of how it felt. Lisa’s approach left more room for reflecting on experiences, and then making a choice, rather than jumping into what he was “supposed” to do. Joey took her advice and allowed himself another year to try out majors and then decide on a career. After experiencing courses in many varied subjects, Joey decided that he did truly enjoy architecture and was able to switch his focus from premed to architecture. (Courtesy of Caroly S. Pataki M.D.) Components of Adolescence Physical Development. Puberty is the process by which adolescents develop physical and sexual maturity, along with reproductive ability. The first signs of the pubertal process are an increased rate of growth in both height and weight. This process begins in girls by approximately 10 years of age. By the age of 11 or 12, many girls noticeably tower over their male classmates, who do not experience a growth spurt, on average, until they reach 13 years of age. By age 13, many girls have experienced menarche, and most have developed breasts and pubic hair. Wide variation exists in the normal range of onset and timing of pubertal development and its components. A set sequence occurs, however, in the order in which pubertal development proceeds. Thus, secondary sexual characteristics in boys, such as increased length and width of the penis, for example, will occur after the release of androgens from developed enlarged testes. Sexual maturity ratings (SMR), also referred to as Tanner Stages, range from SMR 1 (prepuberty) to SMR 5 (adult). The SMR ratings include stages of genital maturity in boys and breast development in girls, as well as pubic hair development. Table 31.1-11 outlines sexual maturity ratings for boys and girls. Table 31.1-11 Sexual Maturity Ratings for Male and Female Adolescents The primary female sex characteristic is ovulation, the release of eggs from ovarian follicles, approximately once every 28 days. When adolescent girls reach SMR 3 to 4, ovarian follicles are producing enough estrogen to result in menarche, the onset of menstruation. When adolescent girls reach SMR 4 to 5, an ovarian follicle matures on a monthly basis and ovulation occurs. Estrogen and progesterone promote sexual maturation, including further development of fallopian tubes and breasts. For adolescent boys, the primary sex characteristic is the development of sperm by the testes. In boys, sperm development occurs in response to follicle-stimulating hormone acting on the seminiferous tubules within the testes. The pubertal process in boys is marked by the growth of the testes stimulated by luteinizing hormone. An adolescent boy’s ability to ejaculate generally emerges within 1 year of reaching SMR 2. Secondary sexual characteristics in boys include thickening of skin, broadening of the shoulders, and the development of facial hair. Cognitive Maturation. Cognitive maturation in adolescence encompasses a wide range of expanded abilities that fall within the global category of executive functions of the brain. These include the transition from concrete thinking to more abstract thinking; an increased ability to draw logical conclusions in scientific pursuits, with peer interactions and in social situations; and new abilities for self-observation and selfregulation. Adolescents acquire increased awareness of their own intellectual, artistic, and athletic gifts and talents; yet it often takes many more years into young adulthood to establish a practical application for these abilities. The central cognitive change that occurs gradually during adolescence is the shift from concrete thinking (concrete operational thinking, according to Jean Piaget) to the ability to think abstractly (formal operational thinking, in Piaget’s terminology). This evolution occurs as an adaptation to stimuli that demand an adolescent to produce hypothetical responses, as well as in response to the adolescent’s expanded abilities to provide generalizations from specific situations. The development of abstract thinking is not a sudden epiphany but, rather, a gradual process of expanding logical deductions beyond concrete experiences and achieving the capacity for idealistic and hypothetical thinking based on everyday life. Adolescents often use an omnipotent belief system that reinforces their sense of immunity from danger, even when confronted with logical risks. Some degree of childlike magical thinking continues to coexist with more mature abstract thinking in many adolescents. Despite the persistence of magical thinking into adolescence, adolescent cognition departs from that of younger children insofar as the increased ability for selfobservation and development of strategies to promote strengths and compensate for weaknesses. One of the important cognitive tasks in adolescence is to identify and gravitate toward those pursuits that seem to match the adolescent’s cognitive strengths, in academic courses and in thinking about future aspirations. Piaget believed that cognitive adaptation in adolescence is profoundly influenced by social relationships and the dialogue between adolescents and peers, making social cognition an integral part of cognitive development in adolescence. Socialization. Socialization in adolescence encompasses the ability to find acceptance in peer relationships, as well as the development of more mature social cognition. The skills to develop a sense of belonging to a peer group are of central importance to a sense of well-being. Being viewed as socially competent by peers is a critical component in building good self-esteem for most early adolescents. Peer influences are powerful and can foster positive social interactions, as well as apply pressure in less socially accepted behaviors or even high-risk behavior. Belonging to a peer group is, in general, a sign of adaptation and a developmentally appropriate step in separating from parents and turning the focus of loyalty toward friends. Children between the ages of 6 and 12 are able to engage in exchanges of ideas and opinions and acknowledge feelings of peers, but the relationships often wax and wane in a discontinuous way on the basis of altercations and good times. Friendships deepen with repeated good times but, for some school-aged children, a variety of peers are often interchangeable—that is, a companion is sought when a given child has free time, rather than out of a desire to spend time with a specific friend. As adolescence ensues, friendships become more individualized, and personal secrets are likely shared with a friend rather than a family member. A comfort level is achieved with one or several early adolescent peers, and the group may “stick together,” spending most free time together. In early adolescence, a blend of the above two social modes may emerge, small “cliques” arise, and, even within the cliques, competition and jealousies regarding which dyads are “preferred” or higher ranked within the clique may result in some discontinuities in the relationships. In later adolescence, the peer group solidifies, leading to increased stability in the friendships and a greater mutuality in the quality of the interactions. Moral Development. Morality is a set of values and beliefs about codes of behavior that conform to those shared by others in society. Adolescents, as do younger children, tend to develop patterns of behaviors characteristic of their family and educational environments and by imitation of specific peers and adults whom they admire. Moral development is not strictly tied to chronological age but, rather, is an outgrowth from cognitive development. Piaget described moral development as a gradual process parallel to cognitive development, with expanded abilities in differentiating the best interests for society from those of individuals occurring during late adolescence. Preschool children simply follow rules set forth by the parents; in the middle years, children accept rules but show an inability to allow for exceptions; and during adolescence, young persons recognize rules in terms of what is good for the society at large. Lawrence Kohlberg integrated Piaget’s concepts and described three major levels of morality. The first level is preconventional morality, in which punishment and obedience to the parent are the determining factors. The second level is morality of conventional role-conformity, in which children try to conform to gain approval and to maintain good relationships with others. The third and highest level is morality of selfaccepted moral principles, in which children voluntarily comply with rules on the basis of a concept of ethical principles and make exceptions to rules in certain circumstances. Although Kohlberg’s and Piaget’s notions of moral development focus on a unified theory of cognitive maturation for both sexes, Carol Gilligan emphasizes the social context of moral development leading to divergent patterns in moral development. Gilligan points out that, in women, compassion and the ethics of caring are dominant features of moral decision-making, whereas, for men, predominant features of moral judgments are related more to a perception of justice, rationality, and a sense of fairness. Self-Esteem. Self-esteem is a measure of one’s sense of self-worth based on perceived success and achievements, as well as a perception of how much one is valued by peers, family members, teachers, and society in general. The most important correlates of good self-esteem are one’s perception of positive physical appearance and high value to peers and family. Secondary features of self-esteem relate to academic achievement, athletic abilities, and special talents. Adolescent self-esteem is mediated, to a significant degree, by positive feedback received from a peer group and family members, and adolescents often seek out a peer group that offers acceptance, regardless of negative behaviors associated with that group. Adolescent girls have more of a problem maintaining self-esteem than do boys. Girls continued to rate themselves with generally lower self-esteem into adulthood. Current Environmental Influences and Adolescence Adolescent Sexual Behavior. Sexual experimentation in adolescents often begins with fantasy and masturbation in early adolescence followed by noncoital genital touching with the opposite sex or, in some cases, same-sex partners, oral sex with partners, and initiation of sexual intercourse at a later point in development. By high school, most male adolescents report experience with masturbation, and more than half of adolescent girls report masturbation. The balance between healthy adolescent sexual experimentation and emotionally and physically safe sexual practices is one of the major challenges for society. Estimates vary, but about 50 percent of 9th to 12th grade students reported having had sexual intercourse. The median age at first intercourse is about 16 years for boys and 17 years for girls. Boys generally have more sexual partners than do girls, and boys are less likely than girls to seek emotional attachments with their sexual partners. FACTORS INFLUENCING ADOLESCENT SEXUAL BEHAVIOR. Factors that affect sexual behavior in adolescents include personality traits, gender, cultural and religious background, racial factors, family attitudes, and sexual education and prevention programs. Personality factors have been found to be associated with sexual behavior, as well as sexual risk-taking. Higher levels of impulsivity are associated with a younger age at first experience of sexual intercourse; higher number of sexual partners; sexual intercourse without the use of contraception, including condoms; and a history of sexually transmitted disease (chlamydia). Historically, male adolescents have initiated sexual intercourse at a younger age than female adolescents. The younger a teenage girl is when she has sex for the first time, the more likely she is to have had unwanted sexual activity. Close to four of ten girls who had first intercourse at 13 or 14 years of age report it was either not voluntary or unwanted. Three of four girls and over half of boys report that girls who have sex do so because their boyfriends want them to. In general, adolescents who initiate sexual intercourse at younger ages are also more likely to have a greater number of sexual partners. The additive effects of more highly educated families, social and religious youth groups, and school-based educational programs can be credited with a decline in highrisk sexual behavior among adolescents. Responsible sexual behavior among adolescents has been determined as one of the ten leading health indicators for the next decade. The primary reason that teenage girls who have never had intercourse give for abstaining from sex is that having sex would be against their religious or moral values. Other reasons include desire to avoid pregnancy, fear of contracting a sexually transmitted disease (STD), and not having met the appropriate partner. CONTRACEPTIVES. Currently, 98 percent of teenagers 15 to 19 years are using at least one method of birth control. The two most common methods are condoms and birth control pills. STDs, despite use of condoms, are still at high levels in teens. Approximately one in four sexually active teens contracts an STD every year. Approximately half of all new human immunodeficiency virus (HIV) infections occur in people younger than age 25. PREGNANCY. Each year 750,000 to 850,000 teenage girls younger than age 19 become pregnant. Of this number, 432,000 give birth, a 19 percent decline from 532,000 in 1991; the rest (418,000) obtain abortions. The largest decline in teen pregnancy by race is for black women. Hispanic teen births have declined 20 percent, but continue to have the highest teen birth rates compared with other races. Teenage pregnancy creates a plethora of health risks for both mother and child. Children born to teenage mothers have a greater chance of dying before the age of 5 years. Those who survive are more likely to perform poorly in school and are at greater risk of abuse and neglect. Teenage mothers are less likely to gain adequate weight during pregnancy, increasing the risk of premature births and low-birth-weight infants. Low-birth-weight babies are more likely to have organs that are not fully developed, resulting in bleeding in the brain, respiratory distress syndrome, and intestinal problems. Teenage mothers are also less likely to seek regular prenatal care and to take recommended daily multivitamins, and they are more likely to smoke, drink, or use drugs during pregnancy. Only one third of teenage mothers obtain high school diplomas, and only 1.5 percent have a college degree by the age of 30. The average adolescent mother who cannot care for her child has the child either placed in foster care or raised by the teenager’s already overburdened parents or other relatives. Few teenage mothers marry the fathers of their children; the fathers, usually teenagers, cannot care for themselves, much less the mothers of their children. If the two do marry, they usually divorce. Many are more likely to end up on welfare. ABORTION. Nearly four of ten teen pregnancies end in abortion. Almost all the girls are unwed mothers from low socioeconomic groups; their pregnancies result from sex with boys to whom they felt emotionally attached. Most (61 percent) teenagers elect to have abortions with their parents’ consent, but laws of mandatory parental consent put two rights into competition: a girl’s claim to privacy and a parent’s need to know. Most adults believe that teenagers should have parental permission for an abortion; but when parents refuse to give their consent, most states prohibit parents from vetoing the teenager’s decision. The abortion rate in many European countries tends to be far lower than that in the United States. In the United States, the rate of abortion among girls between the ages of 15 and 19 is about 30 per 1,000 girls, according to the Centers for Disease Control and Prevention. In France, for instance, about 10.5 of every 1,000 girls under the age of 20 had an abortion, according to World Health Organization statistics. The rate of abortion in Germany was 6.8; in Italy, 6.3; and in Spain, 4.5. Britain has a higher rate, 18.5. Family planning experts believe that more sex education and availability of contraceptive devices help keep the number of abortions down. In Holland, where contraceptives are freely available in schools, the teenage pregnancy rate is among the lowest in the world. Risk-Taking Behavior. Reasonable risk-taking is a necessary endeavor in adolescence, leading to confidence both in forming new relationships and in sports and social situations. High-risk behaviors among adolescents are associated with serious negative consequences, however, and can take many forms, including drug and alcohol use, unsafe sexual practices, self-injurious behaviors, and reckless driving. Drug Use ALCOHOL. About 30 percent of 12th graders report having five or more drinks in a row within a 2-week period. The average age when youths first try alcohol is 11 years for boys and 13 years for girls. The national average age at which Americans begin drinking regularly is 15.9 years of age. People ages 18 to 25 show the highest prevalence of binge and heavy drinking. Drunk driving has declined since 2002. Alcohol dependence, along with other drugs, is associated with depression, anxiety, oppositional defiant disorder, antisocial personality disorder, and an increased rate of suicide. NICOTINE. The number of younger Americans who smoke has declined since 1990; however, the rate of smoking among teenagers is still as high as or higher than that of adults. According to the American Cancer Society, on average more than one of five students has smoked cigarettes. Each day, more than 4,000 teenagers try their first cigarette and another 2,000 become regular, daily smokers. Cigarette smokers are more likely to get into fights, carry weapons, attempt suicide, suffer from mental health problems such as depression, and engage in high-risk sexual behaviors. One of three will eventually die from smoking-related diseases. Cigarettes are the most common type of tobacco used among middle-school students followed by cigars, smokeless tobacco, and pipes. CANNABIS. Marijuana is the most popular illicit drug, with 14.6 million people using it (6.2 percent of the population), two thirds being under the age of 18. Its use, however, is slowly declining. About 6 percent of 12th graders report daily use of marijuana. One of the major reasons for such prevalence of marijuana use among teenagers is because many find that marijuana is easier to get than alcohol or cigarettes. This belief has declined in recent years. Once teenagers are dependent on marijuana, they often tumble into truancy, crime, and depression. COCAINE. About 13 percent of high school seniors use cocaine. exceeding the national average of 3.6 percent. In addition, about 1 percent of 12th graders admit to using phencyclidine (PCP). Crystal methamphetamine (ice) has an annual prevalence in 12th graders of about 2 percent. OPIOIDS. In recent years, the number of teens using prescription pain relievers for nonmedical reasons has increased. Prescription drug abuse by people ages 18 to 25 has increased 15 percent. Drugs of specific concern are the pain relievers oxycodone (OxyContin) and hydrocodone (Vicodin). OxyContin has gained ground among high school students since its emergence in 2001, with 5 percent of 12th graders, 3.5 percent of 10th graders, and 1.7 percent of 8th graders reporting use. Vicodin was used by 9.3 percent of 12th graders, 6.2 percent of 10th graders, and 2.5 percent of 8th graders. HEROIN. Heroin use is prevalent among adolescents, although less so than cocaine. The average age of use is 19, but it is used by almost 2 percent of 12th graders, the nasal route (snorting) being the most common method of use. Violence. Although rates of violent crime have decreased throughout the United States in recent years, violent crimes by young offenders are on the increase. Homicides are the second leading cause of death among persons ages 15 to 25. (Accidents are first; suicides are third.) Black male teenagers are far more likely to be murder victims than are boys from any other racial or ethnic group or girls of any race. The factor most strongly associated with violence among adolescent boys is growing up in a household without a father or father surrogate; this factor aside, race, socioeconomic status, and education show no effect on the propensity toward violence. BULLYING. Bullying is defined as the use of one’s strength or status to intimidate, injure, or humiliate another person of lesser strength or status. It can be categorized as physical, verbal, or social. Physical bullying involves physical injury or threat of injury to someone. Verbal bullying refers to teasing or insulting someone. Social bullying refers to the use of peer rejection or exclusion to humiliate or isolate a victim. Approximately 30 percent of 6th through 10th grade students are involved in some aspect of moderate-to-frequent bullying, either as a bully, the target of bullying, or both. Approximately 1.7 million children within this age group can be identified as bullies. Boys are more likely to be involved in bullying and violent behavior than girls. Girls tend to use verbal bullying rather than physical. An estimated 160,000 students miss school each day because of fear of attack or intimidation from peers; some are forced to drop out. Stresses of “victimization” can interfere with student’s engagement and learning in school. Children who bully other children are at risk for engaging in more serious violent behaviors, such as frequent fighting and carrying a weapon. Cyber Bullying. During the last decade, electronic or internet bullying has become of great concern to adolescents. Cyber bullying is defined broadly, to convey the use of electronic means to intentionally intimidate or harm someone. The reported prevalence of cyber bullying is variable, reports ranging from 1% to 62% of youth reporting that they were victims of cyber victimization. A study of about 700 Australian students, recruited at age 10 years, and followed until age 14 to 15 years, found that 15% had engaged in cyber bullying, 21% had engaged in traditional bullying, and 7% had engaged in both. Another study of self-reported information collected from 399 teens in the 8th to 10th grades, found that involvement in cyber bullying, either as a victim or a bully, specifically contributed to the prediction of depressive symptoms and suicidal ideation. This correlation of cyber bullying and depressive symptomatology was found to be stronger than the association of traditional bulling and affective disorder. Gangs. Gang violence is a problem in various communities throughout the United States. There are 2,000 different youth gangs around the country with more than 200,000 teens and young adults as members. Most members are between the ages of 12 and 24 years, with an average of 17 to 18 years. Gang membership is a brief phase for many teenagers; one half to two thirds leave the gang by the 1-year mark. Boys are more likely to join gangs than girls; however, female gang membership may be underrepresented. Female gang members are more likely to be found in small cities and rural areas and tend to be younger than male gang members. Female gang members are also involved in less delinquent or criminal activity than males and commit fewer violent crimes. WEAPONS. Each day, on average, nearly ten American children younger than the age of 18 years are killed in handgun suicides, homicides, and accidents. Many more are wounded. One in five youths in grades 9 to 12 carries a weapon: knife, gun, or club. By law, firearms cannot be sold to anyone younger than the age of 18 years. Two thirds of students in grades 6 to 12 say that they can get a firearm within 24 hours, however. More than 22 million children live in a home with a firearm. In 40 percent of these homes, at least one gun is kept unlocked and 13 percent are kept unlocked and loaded. Two of three students involved in school shootings acquired their guns from their own home or that of a relative. At least 60 percent of suicide deaths in teens involve the use of a handgun. SCHOOL VIOLENCE. According to the CDC of all youth homicides in 2010 about 2 percent occurred in schools. Approximately 7 percent of teachers report they have been threatened with injury or physically attacked by a student from their school. In addition, among students in grades 9 through 12, about 6 percent reported carrying a weapon on school property on one or more days in the 30 days before the survey. Many factors can lead to violent acts in teenagers. Some inherited traits include impulsivity, learning difficulties, low IQ, or fearlessness. A correlation also exists between witnessing violent acts and involvement in violence. Children who witness violent acts are more aggressive and grow up more likely to become involved in violence—either as a victimizer or as a victim. Table 31.1-12 lists some of the early and imminent warning signs of school violence. Table 31.1-12 Warning Signs of School Violence On April 20, 1999, two teenage boys, ages 17 and 18 years, went on a shooting rampage through Columbine High School of Littleton, Colorado. Armed with shotguns, a semiautomatic rifle, and a pistol, they laughed and hollered as they shot classmates and teachers at point-blank range while hurling homemade explosives. Fifteen were killed, including the two gunmen, and 25 were injured. The gunmen were members of the “trench coat mafia” at the high school, a clique of social misfits who stood out at the school for their gothic style of dress and nihilistic attitude. The two gunmen were obsessed with violent video games and intrigued with Nazi culture, even though one was part Jewish. The date of the attack was picked because it was Adolf Hitler’s birthday. On March 21, 2005, a 16-year-old boy went on a shooting rampage at Red Lake High School on the Red Lake Indian Reservation in far northern Minnesota. He began his shooting spree by killing his grandfather and the grandfather’s companion. He then donned his grandfather’s police-issue gun belt and bulletproof vest before heading to the school, where he killed a security guard, a teacher, five students, and then himself. About 15 others were injured. The gunman had a troubled childhood; his father committed suicide in 1997 and his mother suffered head injuries in an auto accident. He expressed admiration for Adolf Hitler on a neo-Nazi website, using the handle “Todesengel,” which is German for “Angel of Death.” He had bouts of depression, suicide ideation, and was taking fluoxetine (Prozac). He was a member of a clique of about five students known as “The Darkers,” who wore black clothes and chains, spiked or dyed their hair, and loved heavy-metal music. The gunman was usually seen in a long black trench coat, eyeliner, and combat boots, and was described as a quiet teenager. SEXUAL OFFENSE. Adolescents younger than age 18 years account for 20 percent of arrests for all sexual offenses (excluding prostitution), 20 to 30 percent of rape cases, 14 percent of aggravated sexual assault offenses, and 27 percent of child sexual homicides. These adolescent offenders account for the victimization of approximately one half of boys and one fourth of girls who are molested or sexually abused. Most instances have involved adolescent male perpetrators. There appear to be two types of juvenile sex offenders: those who target children and those who offend against peers or adults. The main distinction between the two groups is based on the age difference between the victim and the offender. Table 31.1-13 lists the differences and similarities of these two groups. Table 31.1-13 Juvenile Sex Offender Subtypes Etiological factors of juvenile sex offending include maltreatment experiences, exposure to pornography, substance abuse, and exposure to aggressive role models. A significant number of offending adolescents have a childhood history of physical abuse (25 to 50 percent) or sexual abuse (10 to 80 percent). Half of adolescent offenders lived with both parents and one other juvenile at the time of their offending. Evidence also suggests that most juvenile sex offenders are likely to become adult sex offenders. The most common psychosocial deficits of adolescent sexual offenders include low selfesteem, few social skills, minimal assertive skills, and poor academic performance. The most common psychiatric diagnoses are conduct disorder, substance abuse disorder, adjustment disorder, attention-deficit/hyperactivity disorder, specific phobia, and mood disorders. Male offenders are more often diagnosed with paraphilias and antisocial behavior, whereas female offenders are more likely to be diagnosed with mood disorders and engage in self-mutilation. Prostitution. Teenagers constitute a large portion of all prostitutes, with estimates ranging up to 1 million teenagers involved in prostitution. The average age of a new recruit is 13 years; however, some are as young as 9 years of age. Most adolescent prostitutes are girls, but boys are involved as homosexual prostitutes. Most teenagers who enter a life of prostitution come from broken homes; however, a growing number of teenage prostitutes come from middle- to upper middle-class homes. Many have been victims of rape, or were abused as children. Most teenagers ran away from home and were taken in by pimps and substance abusers; the adolescents themselves then became substance abusers. Twenty-seven percent of teenage prostitution occurs in large cities, and incidents usually take place at an outside location, such as highways, roads, alleys, fields, woods, or parking lots. Teenage prostitutes are at high risk for acquired immunodeficiency syndrome (AIDS), and many (up to 70 percent in some studies) are infected with HIV. As many as 17,500 individuals are smuggled into the United States each year as “sex slaves.” They are brought under the pretenses of a better life and job opportunities, but once they are in the United States, they are forced into prostitution, making little money while traffickers make thousands of dollars from their services. Many times they are raped and abused. Tattoos and Body Piercing. Body piercing and tattoos have become more prevalent among adolescents since the 1980s. 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Kicking the digital dog: A longitudinal investigation of young adults’ victimization and cyber-displaced aggression. Cyberpsychol Behav Soc Netw. 2012;15:448–454. 31.2 Assessment, Examination, and Psychological Testing A comprehensive evaluation of a child is composed of interviews with the parents, the child, and other family members; gathering information regarding the child’s current school functioning; and often, a standardized assessment of the child’s intellectual level and academic achievement. In some cases, standardized measures of developmental level and neuropsychological assessments are useful. Psychiatric evaluations of children are rarely initiated by the child, so clinicians must obtain information from the family and the school to understand the reasons for the evaluation. In some cases, the court or a child protective service agency may initiate a psychiatric evaluation. Children can be excellent informants about symptoms related to mood and inner experiences, such as psychotic phenomena, sadness, fears, and anxiety, but they often have difficulty with the chronology of symptoms and are sometimes reticent about reporting behaviors that have gotten them into trouble. Very young children often cannot articulate their experiences verbally and do better showing their feelings and preoccupations in a play situation. Assessment of a child or adolescent includes identifying the reasons for referral; assessing the nature and extent of the child’s psychological and behavioral difficulties; and determining family, school, social, and developmental factors that may be influencing the child’s emotional well-being. The first step in the comprehensive evaluation of a child or adolescent is to obtain a full description of the current concerns and a history of the child’s previous psychiatric and medical problems. This is often done with the parents for school-aged children, whereas adolescents may be seen alone first, to get their perception of the situation. Direct interview and observation of the child is usually next, followed by psychological testing, when indicated. Clinical interviews offer the most flexibility in understanding the evolution of problems and in establishing the role of environmental factors and life events, but they may not systematically cover all psychiatric diagnostic categories. To increase the breadth of information generated, the clinician may use semistructured interviews such as the Kiddie Schedule for Affective Disorders and Schizophrenia for School-Age Children (KSADS); structured interviews such as the National Institute for Mental Health Diagnostic Interview Schedule for Children Version IV (NIMH DISC-IV); and rating scales, such as the Child Behavior Checklist and Connors Parent or Teacher Rating Scale for ADHD. It is not uncommon for interviews from different sources, such as parents, teachers, and school counselors, to reflect different or even contradictory information about a given child. When faced with conflicting information, the clinician must determine whether apparent contradictions actually reflect an accurate picture of the child in different settings. Once a complete history is obtained from the parents, the child is examined, the child’s current functioning at home and at school is assessed, and psychological testing is completed, the clinician can use all the available information to make a best-estimate diagnosis and can then make recommendations. Once clinical information is obtained about a given child or adolescent, it is the clinician’s task to determine whether criteria are met for one or more psychiatric disorders according to the Fifth Edition of the Diagnostic and Statistical Manual of Mental Disorders (DSM-5). This most current version is a categorical classification reflecting the consensus on constellations of symptoms believed to comprise discrete and valid psychiatric disorders. Psychiatric disorders are defined by the DSM-5 as a clinically significant set of symptoms that is associated with impairment in one or more areas of functioning. Whereas clinical situations requiring intervention do not always fall within the context of a given psychiatric disorder, the importance of identifying psychiatric disorders when they arise is to facilitate meaningful investigation of childhood psychopathology. CLINICAL INTERVIEWS To conduct a useful interview with a child of any age, clinicians must be familiar with normal development to place the child’s responses in the proper perspective. For example, a young child’s discomfort on separation from a parent and a school-age child’s lack of clarity about the purpose of the interview are both perfectly normal and should not be misconstrued as psychiatric symptoms. Furthermore, behavior that is normal in a child at one age, such as temper tantrums in a 2-year-old, takes on a different meaning, for example, in a 17-year-old. The interviewer’s first task is to engage the child and develop a rapport so that the child is comfortable. The interviewer should inquire about the child’s concept of the purpose of the interview and should ask what the parents have told the child. If the child appears to be confused about the reason for the interview, the examiner may opt to summarize the parents’ concerns in a developmentally appropriate and supportive manner. During the interview with the child, the clinician seeks to learn about the child’s relationships with family members and peers, academic achievement and peer relationships in school, and the child’s pleasurable activities. An estimate of the child’s cognitive functioning is a part of the mental status examination. The extent of confidentiality in child assessment is correlated with the age of the child. In most cases, almost all specific information can appropriately be shared with the parents of a very young child, whereas privacy and permission of an older child or adolescent are mandated before sharing information with parents. School-age and older children are informed that if the clinician becomes concerned that any child is dangerous to himself or herself or to others, this information must be shared with parents and, at times, additional adults. As part of a psychiatric assessment of a child of any age, the clinician must determine whether that child is safe in his or her environment and must develop an index of suspicion about whether the child is a victim of abuse or neglect. Whenever there is a suspicion of child maltreatment, the local child protective service agency must be notified. Toward the end of the interview, the child may be asked in an open-ended manner whether he or she would like to bring up anything else. Each child should be complimented for his or her cooperation and thanked for participating in the interview, and the interview should end on a positive note. Infants and Young Children Assessments of infants usually begin with the parents present, because very young children may be frightened by the interview situation; the interview with the parents present also allows the clinician to assess the parent–infant interaction. Infants may be referred for a variety of reasons, including high levels of irritability, difficulty being consoled, eating disturbances, poor weight gain, sleep disturbances, withdrawn behavior, lack of engagement in play, and developmental delay. The clinician assesses areas of functioning that include motor development, activity level, verbal communication, ability to engage in play, problem-solving skills, adaptation to daily routines, relationships, and social responsiveness. The child’s developmental level of functioning is determined by combining observations made during the interview with standardized developmental measures. Observations of play reveal a child’s developmental level and reflect the child’s emotional state and preoccupations. The examiner can interact with an infant age 18 months or younger in a playful manner by using such games as peek-a-boo. Children between the ages of 18 months and 3 years can be observed in a playroom. Children ages 2 years or older may exhibit symbolic play with toys, revealing more in this mode than through conversation. The use of puppets and dolls with children younger than 6 years of age is often an effective way to elicit information, especially if questions are directed to the dolls, rather than to the child. School-Age Children Some school-age children are at ease when conversing with an adult; others are hampered by fear, anxiety, poor verbal skills, or oppositional behavior. School-age children can usually tolerate a 45-minute session. The room should be sufficiently spacious for the child to move around, but not so large as to reduce intimate contact between the examiner and the child. Part of the interview can be reserved for unstructured play, and various toys can be made available to capture the child’s interest and to elicit themes and feelings. Children in lower grades may be more interested in the toys in the room, whereas by the sixth grade, children may be more comfortable with the interview process and less likely to show spontaneous play. The initial part of the interview explores the child’s understanding of the reasons for the meeting. The clinician should confirm that the interview was not set up because the child is “in trouble” or as a punishment for “bad” behavior. Techniques that can facilitate disclosure of feelings include asking the child to draw peers, family members, a house, or anything else that comes to mind. The child can then be questioned about the drawings. Children may be asked to reveal three wishes, to describe the best and worst events of their lives, and to name a favorite person to be stranded with on a desert island. Games such as Donald W. Winnicott’s “squiggle,” in which the examiner draws a curved line and then the child and the examiner take turns continuing the drawing, may facilitate conversation. Questions that are partially open-ended with some multiple choices may elicit the most complete answers from school-age children. Simple, closed (yes or no) questions may not elicit sufficient information, and completely open-ended questions can overwhelm a school-age child who cannot construct a chronological narrative. These techniques often result in a shoulder shrug from the child. The use of indirect commentary—such as, “I once knew a child who felt very sad when he moved away from all his friends”—is helpful, although the clinician must be careful not to lead the child into confirming what the child thinks the clinician wants to hear. School-age children respond well to clinicians who help them compare moods or feelings by asking them to rate feelings on a scale of 1 to 10. Adolescents Adolescents usually have distinct ideas about why the evaluation was initiated, and can usually give a chronological account of the recent events leading to the evaluation, although some may disagree with the need for the evaluation. The clinician should clearly communicate the value of hearing the story from an adolescent’s point of view and must be careful to reserve judgment and not assign blame. Adolescents may be concerned about confidentiality, and clinicians can assure them that permission will be requested from them before any specific information is shared with parents, except in situations involving danger to the adolescent or others, in which case confidentiality must be sacrificed. Adolescents can be approached in an open-ended manner; however, when silences occur during the interview, the clinician should attempt to reengage the patient. Clinicians can explore what the adolescent believes the outcome of the evaluation will be (change of school, hospitalization, removal from home, removal of privileges). Some adolescents approach the interview with apprehension or hostility, but open up when it becomes evident that the clinician is neither punitive nor judgmental. Clinicians must be aware of their own responses to adolescents’ behavior (countertransference) and stay focused on the therapeutic process even in the face of defiant, angry, or difficult teenagers. Clinicians should set appropriate limits and should postpone or discontinue an interview if they feel threatened or if patients become destructive to property or engage in self-injurious behavior. Every interview should include an exploration of suicidal thoughts, assaultive behavior, psychotic symptoms, substance use, and knowledge of safe sexual practices along with a sexual history. Once rapport has been established, many adolescents appreciate the opportunity to tell their side of the story and may reveal things that they have not disclosed to anyone else. Family Interview An interview with parents and the patient may take place first or may occur later in the evaluation. Sometimes, an interview with the entire family, including siblings, can be enlightening. The purpose is to observe the attitudes and behavior of the parents toward the patient and the responses of the children to their parents. The clinician’s job is to maintain a nonthreatening atmosphere in which each member of the family can speak freely without feeling that the clinician is taking sides with any particular member. Although child psychiatrists generally function as advocates for the child, the clinician must validate each family member’s feelings in this setting, because lack of communication often contributes to the patient’s problems. Parents The interview with the patient’s parents or caretakers is necessary to get a chronological picture of the child’s growth and development. A thorough developmental history and details of any stressors or important events that have influenced the child’s development must be elicited. The parents’ view of the family dynamics, their marital history, and their own emotional adjustment are also elicited. The family’s psychiatric history and the upbringing of the parents are pertinent. Parents are usually the best informants about the child’s early development and previous psychiatric and medical illnesses. They may be better able to provide an accurate chronology of past evaluations and treatment. In some cases, especially with older children and adolescents, the parents may be unaware of significant current symptoms or social difficulties of the child. Clinicians elicit the parents’ formulation of the causes and nature of their child’s problems and ask about their expectations for the current assessment. DIAGNOSTIC INSTRUMENTS The two main types of diagnostic instruments used by clinicians are diagnostic interviews and questionnaires. Diagnostic interviews are administered to either children or their parents and typically are designed to elicit sufficient information on various aspects of functioning in order to determine whether DSM-5 criteria are met. Semistructured interviews, or “interviewer-based” interviews, such as K-SADS and the Child and Adolescent Psychiatric Assessment (CAPA), serve as guides for the clinician. They help the clinician clarify answers to questions about symptoms. Structured interviews, or “respondent-based” interviews, such as NIMH DISC-IV, the Children’s Interview for Psychiatric Syndromes (ChIPS), and the Diagnostic Interview for Children and Adolescents (DICA), provide a script for the interviewer without interpretation of the patient responses during the interview process. Two other diagnostic instruments, the Dominic-R and the Pictorial Instrument for Children and Adolescents (PICA-III-R), use pictures as cues along with an accompanying question to elicit information about symptoms, which can be especially useful for young children as well as for adolescents. Diagnostic instruments aid the collection of information in a systematic way. Diagnostic instruments, even the most comprehensive, however, cannot replace clinical interviews, because clinical interviews are superior in understanding the chronology of symptoms, the interplay between environmental stressors and emotional responses, and developmental issues. Clinicians often find it helpful to combine data from diagnostic instruments with clinical material gathered in a comprehensive evaluation. Questionnaires can cover a broad range of symptom areas, such as the Achenbach Child Behavior Checklist, or they can be focused on a particular type of symptomatology, such as the Connors Parent Rating Scale for ADHD. Semistructured Diagnostic Interviews Kiddie Schedule for Affective Disorders and Schizophrenia for SchoolAge Children. The K-SADS can be used for children and adolescents from 6 to 18 years of age. It contains multiple items with some space for further clarification of symptoms. It elicits information on current diagnosis and on symptoms present in the previous year. Another version can also ascertain lifetime diagnoses. This instrument has been used extensively, especially in evaluation of mood disorders, and includes measures of impairment caused by symptoms. The schedule comes in a form for parents to give information about their child and in a version for use directly with the child. The schedule takes about 1 to 1.5 hours to administer. The interviewer should have some training in the field of child psychiatry, but need not be a psychiatrist. Child and Adolescent Psychiatric Assessment. The CAPA is an “interviewerbased” instrument that can be used for children from 9 to 17 years of age. It comes in modular form so that certain diagnostic entities can be administered without having to give the entire interview. It covers disruptive behavior disorders, mood disorders, anxiety disorders, eating disorders, sleep disorders, elimination disorders, substance use disorders, tic disorders, schizophrenia, posttraumatic stress disorder, and somatization symptoms. It focuses on the 3 months before the interview, called the “primary period.” In general, it takes about 1 hour to administer. It has a glossary to help clarify symptoms, and it provides separate ratings for presence and severity of symptoms. The CAPA can be used to obtain information that is applicable to making diagnoses according to the DSM-5. Training is necessary to administer this interview, and the interviewer must be prepared to use some clinical judgment in interpreting elicited symptoms. Structured Diagnostic Interviews National Institute of Mental Health Interview Schedule for Children Version IV. The NIMH DISC-IV is a highly structured interview designed to assess more than 30 DSM-IV diagnostic entities administered by trained “laypersons.” Although it was formulated to match diagnostic criteria in DSM-IV, information from this interview can be utilized, along with clinical information for diagnoses in DSM-5. It is available in parallel child and parent forms. The parent form can be used for children from 6 to 17 years of age, and the direct child form of the instrument was designed for children from 9 to 17 years of age. A computer scoring algorithm is available. This instrument assesses the presence of diagnoses that have been present within the last 4 weeks, and also within the last year. Because it is a fully structured interview, the instructions serve as a complete guide for the questions, and the examiner need not have any knowledge of child psychiatry to administer the interview correctly. Children’s Interview for Psychiatric Syndromes. The ChIPS is a highly structured interview designed for use by trained interviewers with children from 6 to 18 years of age. It is composed of 15 sections, and it elicits information on psychiatric symptoms as well as psychosocial stressors targeting 20 psychiatric disorders, according to DSM-IV criteria; however, it can also be applied to diagnoses in DSM-5. There are parent and child forms. It takes approximately 40 minutes to administer the ChIPS. Diagnoses covered include depression, mania, attention-deficit/hyperactivity disorder (ADHD), separation disorder, obsessive-compulsive disorder (OCD), conduct disorder, substance use disorder, anorexia, and bulimia. The ChIPS was designed for use as a screening instrument for clinicians and a diagnostic instrument for clinical and epidemiological research. Diagnostic Interview for Children and Adolescents. The current version of the DICA was developed in 1997 to assess information resulting in diagnoses according to either DSM-IV or DSM-III-R. This instrument can be used to help obtain information that can be applied to DSM-5 as well. Although the DICA was originally designed to be a highly structured interview, it can now be used in a semistructured format. This means that, although interviewers are allowed to use additional questions and probes to clarify elicited information, the method of probing is standardized so that all interviewers will follow a specific pattern. When using the interview with younger children, more flexibility is built in, allowing interviewers to deviate from written questions to ensure that the child understands the question. Parent and child interviews are expected to be used. The DICA is designed for use with children 6 to 17 years of age and generally takes 1 to 2 hours to administer. It covers externalizing behavior disorders, anxiety disorders, depressive disorders, and substance abuse disorders, among others. Pictorial Diagnostic Instruments Dominic-R. The Dominic-R is a pictorial, fully structured interview designed to elicit psychiatric symptoms from children 6 to 11 years of age. The pictures illustrate abstract emotional and behavioral content of diagnostic entities according to the DSMIII-R; however, information gleaned from this instrument can also be applied in conjunction with clinical information to the DSM-5. The instrument uses a picture of a child called “Dominic” who is experiencing the symptom in question. Some symptoms have more than one picture, with a brief story that is read to the child. Along with each picture is a sentence asking about the situation being shown and asking the child if he or she has experiences similar to the one that Dominic is having. Diagnostic entities covered by the Dominic-R include separation anxiety, generalized anxiety, depression and dysthymia, ADHD, oppositional defiant disorder, conduct disorder, and specific phobia. Although symptoms of the preceding diagnoses can be fully elicited from the Dominic-R, no specific provision within the instrument inquires about frequency of the symptom, duration, or age of onset. The paper version of this interview takes about 20 minutes, and the computerized version of this instrument takes about 15 minutes. Trained lay-interviewers can administer this interview. Computerized versions of this interview are available with pictures of a child who is white, black, Latino, or Asian. Pictorial Instrument for Children and Adolescents. PICA-III-R is composed of 137 pictures organized in modules and designed to cover five diagnostic categories, including disorders of anxiety, mood, psychosis, disruptive disorders, and substance use disorder. It is designed to be administered by clinicians and can be used for children and adolescents ranging from 6 to 16 years of age. The PICA-III_R provides a categorical (diagnosis present or absent) and a dimensional (range of severity) assessment. This instrument presents pictures of a child experiencing emotional, behavioral, and cognitive symptoms. The child is asked, “How much are you like him/her?” and a fivepoint rating scale with pictures of a person with open arms in increasing degrees is shown to the child to help him or her identify the severity of the symptoms. It takes about 40 minutes to 1 hour to administer the interview. This instrument is currently keyed to the DSM-III-R, but can be used along with clinical information to make diagnoses according to the DSM-5. This assessment can be used to aid in clinical interviews and in research diagnostic protocols. QUESTIONNAIRES AND RATING SCALES Achenbach Child Behavior Checklist The parent and teacher versions of the Achenbach Child Behavior Checklist were developed to cover a broad range of symptoms and several positive attributes related to academic and social competence. The checklist presents items related to mood, frustration tolerance, hyperactivity, oppositional behavior, anxiety, and various other behaviors. The parent version consists of 118 items to be rated 0 (not true), 1 (sometimes true), or 2 (very true). The teacher version is similar, but without the items that apply only to home life. Profiles were developed based on normal children of three different age groups (4 to 5, 6 to 11, and 12 to 16). Such a checklist identifies specific problem areas that might otherwise be overlooked, and it may point out areas in which the child’s behavior deviates from that of normal children of the same age group. The checklist is not used specifically to make diagnoses. Revised Achenbach Behavior Problem Checklist Consisting of 150 items that cover a variety of childhood behavioral and emotional symptoms, the Revised Achenbach Behavior Problem Checklist discriminates between clinicreferred and nonreferred children. Separate subscales have been found to correlate in the appropriate direction with other measures of intelligence, academic achievement, clinical observations, and peer popularity. As with the other broad rating scales, this instrument can help elicit a comprehensive view of a multitude of behavioral areas, but it is not designed to make psychiatric diagnoses. Connors Abbreviated Parent-Teacher Rating Scale for ADHD In its original form, the Connors Abbreviated Parent-Teacher Rating Scale for ADHD consisted of 93 items rated on a 0 to 3 scale and was subgrouped into 25 clusters, including problems with restlessness, temper, school, stealing, eating, and sleeping. Over the years, multiple versions of this scale were developed and used to aid in systematic identification of children with ADHD. A highly abbreviated form of this rating scale, the Connors Abbreviated Parent-Teacher Questionnaire, was developed for use with both parents and teachers by Keith Connors in 1973. It consists of ten items that assess both hyperactivity and inattention. Brief Impairment Scale A newly validated 23-item instrument suitable to obtain information on children ranging from 4 years to 17 years, the Brief Impairment Scale (BIS) evaluates three domains of functioning: interpersonal relations, school/work functioning, and care/selffulfillment. This scale is administered to an adult informant about his or her child, does not take long to administer, and provides a global measure of impairment along the above three dimensions. This scale cannot be used to make clinical decisions on individual patients, but it can provide information on the degree of impairment that a given child is experiencing in a certain area. COMPONENTS OF THE CHILD PSYCHIATRIC EVALUATION Psychiatric evaluation of a child includes a description of the reason for the referral, the child’s past and present functioning, and any test results. An outline of the evaluation is given in Table 31.2-1. Table 31.2-1 Child Psychiatric Evaluation Identifying Data Identifying data for a child includes the child’s gender, age, as well as the family constellation surrounding the child. History A comprehensive history contains information about the child’s current and past functioning from the child’s report, from clinical and structured interviews with the parents, and from information from teachers and previous treating clinicians. The chief complaint and the history of the present illness are generally obtained from both the child and the parents. Naturally, the child will articulate the situation according to his or her developmental level. The developmental history is more accurately obtained from the parents. Psychiatric and medical histories, current physical examination findings, and immunization histories can be augmented with reports from psychiatrists and pediatricians who have treated the child in the past. The child’s report is critical in understanding the current situation regarding peer relationships and adjustment to school. Adolescents are the best informants regarding knowledge of safe sexual practices, drug or alcohol use, and suicidal ideation. The family’s psychiatric and social histories, and family function are best obtained from the parents. Mental Status Examination A detailed description of the child’s current mental functioning can be obtained through observation and specific questioning. An outline of the mental status examination is presented in Table 31.2-2. Table 31.2-3 lists components of a comprehensive neuropsychiatry mental status. Table 31.2-2 Mental Status Examination for Children Table 31.2-3 Neuropsychiatric Mental Status Examination* Physical Appearance. The examiner should document the child’s size, grooming, nutritional state, bruising, head circumference, physical signs of anxiety, facial expressions, and mannerisms. Parent–Child Interaction. The examiner can observe the interactions between parents and child in the waiting area before the interview and in the family session. The manner in which parents and child converse and the emotional overtones are pertinent. Separation and Reunion. The examiner should note both the manner in which the child responds to the separation from a parent for an individual interview and the reunion behavior. Either lack of affect at separation and reunion or severe distress on separation or reunion can indicate problems in the parent–child relationship or other psychiatric disturbances. Orientation to Time, Place, and Person. Impairments in orientation can reflect organic damage, low intelligence, or a thought disorder. The age of the child must be kept in mind, however, because very young children are not expected to know the date, other chronological information, or the name of the interview site. Speech and Language. The examiner should evaluate the child’s speech and language acquisition. Is it appropriate for the child’s age? A disparity between expressive language usage and receptive language is notable. The examiner should also note the child’s rate of speech, rhythm, latency to answer, spontaneity of speech, intonation, articulation of words, and prosody. Echolalia, repetitive stereotypical phrases, and unusual syntax are important psychiatric findings. Children who do not use words by age 18 months or who do not use phrases by age 2.5 to 3 years, but who have a history of normal babbling and responding appropriately to nonverbal cues, are probably developing normally. The examiner should consider the possibility that a hearing loss is contributing to a speech and language deficit. Mood. A child’s sad expression, lack of appropriate smiling, tearfulness, anxiety, euphoria, and anger are valid indicators of mood, as are verbal admissions of feelings. Persistent themes in play and fantasy also reflect the child’s mood. Affect. The examiner should note the child’s range of emotional expressivity, appropriateness of affect to thought content, ability to move smoothly from one affect to another, and sudden labile emotional shifts. Thought Process and Content. In evaluating a thought disorder in a child, the clinician must always consider what is developmentally expected for the child’s age and what is deviant for any age group. The evaluation of thought form considers loosening of associations, excessive magical thinking, perseveration, echolalia, the ability to distinguish fantasy from reality, sentence coherence, and the ability to reason logically. The evaluation of thought content considers delusions, obsessions, themes, fears, wishes, preoccupations, and interests. Suicidal ideation is always a part of the mental status examination for children who are sufficiently verbal to understand the questions and old enough to understand the concept. Children of average intelligence who are older than 4 years of age usually have some understanding of what is real and what is make-believe and may be asked about suicidal ideation, although a firm concept of the permanence of death may not be present until several years later. Aggressive thoughts and homicidal ideation are assessed here. Perceptual disturbances, such as hallucinations, are also assessed. Very young children are expected to have short attention spans and may change the topic and conversation abruptly without exhibiting a symptomatic flight of ideas. Transient visual and auditory hallucinations in very young children do not necessarily represent major psychotic illnesses, but they do deserve further investigation. Social Relatedness. The examiner assesses the appropriateness of the child’s response to the interviewer, general level of social skills, eye contact, and degree of familiarity or withdrawal in the interview process. Overly friendly or familiar behavior may be as troublesome as extremely retiring and withdrawn responses. The examiner assesses the child’s self-esteem, general and specific areas of confidence, and success with family and peer relationships. Motor Behavior. The motor behavior part of the mental status examination includes observations of the child’s coordination and activity level and ability to pay attention and carry out developmentally appropriate tasks. It also involves involuntary movements, tremors, motor hyperactivity, and any unusual focal asymmetries of muscle movement. Cognition. The examiner assesses the child’s intellectual functioning and problemsolving abilities. An approximate level of intelligence can be estimated by the child’s general information, vocabulary, and comprehension. For a specific assessment of the child’s cognitive abilities, the examiner can use a standardized test. Memory. School-age children should be able to remember three objects after 5 minutes and to repeat five digits forward and three digits backward. Anxiety can interfere with the child’s performance, but an obvious inability to repeat digits or to add simple numbers may reflect brain damage, mental retardation, or learning disabilities. Judgment and Insight. The child’s view of the problems, reactions to them, and suggested solutions may give the clinician a good idea of the child’s judgment and insight. In addition, the child’s understanding of what he or she can realistically do to help and what the clinician can do adds to the assessment of the child’s judgment. Neuropsychiatric Assessment A neuropsychiatric assessment is appropriate for children who are suspected of having a psychiatric disorder that coexists with neuropsychiatric impairment, or psychiatric symptoms that may be caused by neuropsychiatric dysfunction, or a neurologic disorder. Although a neuropsychiatric assessment is not sufficient in most cases to make a psychiatric diagnosis, neuropsychological profiles have been, in some cases correlated with particular psychiatric symptoms and syndromes. For example, neuropsychological differences in executive function, language and memory functions, as well as measures of mood and anxiety, have been found between youth with histories of childhood maltreatment and those without it. The neuropsychiatric evaluation combines information from neurological, neuropsychological testing, and mental status examinations. The neurological examination can identify asymmetrical abnormal signs (hard signs) that may indicate lesions in the brain. A physical examination can evaluate the presence of physical stigmata of particular syndromes in which neuropsychiatric symptoms or developmental aberrations play a role (e.g., fetal alcohol syndrome, Down syndrome). In a study of 119 youth with either early onset schizophrenia or schizoaffective disorder, by Hooper and colleagues, significantly high rates of deficits in intellectual function and academic skills were found, and the severity of these deficits was mildly correlated with severity of their psychiatric illness. A neuropsychiatric examination also includes neurological soft signs and minor physical anomalies. The term neurological soft signs was first noted by Loretta Bender in the 1940s in reference to nondiagnostic abnormalities in the neurological examinations of children with schizophrenia. Soft signs do not indicate focal neurological disorders, but they are associated with a wide variety of developmental disabilities and occur frequently in children with low intelligence, learning disabilities, and behavioral disturbances. Soft signs may refer to both behavioral symptoms (which are sometimes associated with brain damage, such as severe impulsivity and hyperactivity), physical findings (including contralateral overflow movements), and a variety of nonfocal signs (e.g., mild choreiform movements, poor balance, mild incoordination, asymmetry of gait, nystagmus, and the persistence of infantile reflexes). Soft signs can be divided into those that are normal in a young child, but become abnormal when they persist in an older child, and those that are abnormal at any age. The Physical and Neurological Examination for Soft Signs (PANESS) is an instrument used with children up to the age of 15 years. It consists of 15 questions about general physical status and medical history and 43 physical tasks (e.g., touch your finger to your nose, hop on one foot to the end of the line, tap quickly with your finger). Neurological soft signs are important to note, but they are not useful in making a specific psychiatric diagnosis. Minor physical anomalies or dysmorphic features occur with a higher than usual frequency in children with developmental disabilities, learning disabilities, speech and language disorders, and hyperactivity. As with soft signs, the documentation of minor physical anomalies is part of the neuropsychiatric assessment, but it is rarely helpful in the diagnostic process and does not imply a good or bad prognosis. Minor physical anomalies include a high-arched palate, epicanthal folds, hypertelorism, low-set ears, transverse palmar creases, multiple hair whorls, a large head, a furrowed tongue, and partial syndactyl of several toes. When a seizure disorder is being considered in the differential diagnosis or a structural abnormality in the brain is suspected, electroencephalography (EEG), computed tomography (CT), or magnetic resonance imaging (MRI) may be indicated. Developmental, Psychological, and Educational Testing Psychological testing, structured developmental assessments and achievement testing are valuable in evaluating a child’s developmental level, intellectual functioning, and academic difficulties. A measure of adaptive functioning (including the child’s competence in communication, daily living skills, socialization, and motor skills) is the most definitive way to determine the level of intellectual disability in a child. Table 31.2-4 outlines the general categories of psychological tests. Table 31.2-4 Commonly Used Child and Adolescent Psychological Assessment Instruments Development Tests for Infants and Preschoolers. The Gesell Infant Scale, the Cattell Infant Intelligence Scale, Bayley Scales of Infant Development, and the Denver Developmental Screening Test include developmental assessments of infants as young as 2 months of age. When used with very young infants, the tests focus on sensorimotor and social responses to a variety of objects and interactions. When these instruments are used with older infants and preschoolers, emphasis is placed on language acquisition. The Gesell Infant Scale measures development in four areas: motor, adaptive functioning, language, and social. An infant’s score on one of these developmental assessments is not a reliable way to predict a child’s future intelligence quotient (IQ) in most cases. Infant assessments are valuable, however, in detecting developmental deviation and mental retardation and in raising suspicions of a developmental disorder. Whereas infant assessments rely heavily on sensorimotor functions, intelligence testing in older children and adolescents includes later-developing functions, including verbal, social, and abstract cognitive abilities. Intelligence Tests for School-Age Children and Adolescents. The most widely used test of intelligence for school-age children and adolescents is the third edition of the Wechsler Intelligence Scale for Children (WISC-III-R). It can be given to children from 6 to 17 years of age and yields a verbal IQ, a performance IQ, and a combined full-scale IQ. The verbal subtests consist of vocabulary, information, arithmetic, similarities, comprehension, and digit span (supplemental) categories. The performance subtests include block design, picture completion, picture arrangement, object assembly, coding, mazes (supplemental), and symbol search (supplemental). The scores of the supplemental subtests are not included in the computation of IQ. Each subcategory is scored from 1 to 19, with 10 being the average score. An average full-scale IQ is 100; 70 to 80 represents borderline intellectual function; 80 to 90 is in the low average range; 90 to 109 is average; 110 to 119 is high average; and above 120 is in the superior or very superior range. The multiple breakdowns of the performance and verbal subscales allow great flexibility in identifying specific areas of deficit and scatter in intellectual abilities. Because a large part of intelligence testing measures abilities used in academic settings, the breakdown of the WISC-III-R can also be helpful in pointing out skills in which a child is weak and may benefit from remedial education. The Stanford-Binet Intelligence Scale covers an age range from 2 to 24 years. It relies on pictures, drawings, and objects for very young children and on verbal performance for older children and adolescents. This intelligence scale, the earliest version of an intelligence test of its kind, leads to a mental age score as well as an intelligence quotient. The McCarthy Scales of Children’s Abilities and the Kaufman Assessment Battery for Children are two other intelligence tests that are available for preschool and school-age children. They do not cover the adolescent age group. LONG-TERM STABILITY OF INTELLIGENCE. Although a child’s intelligence is relatively stable throughout the school-age years and adolescence, some factors can influence intelligence and a child’s score on an intelligence test. The intellectual functions of children with severe mental illnesses and of those from deprived and neglectful environments may decrease over time, whereas the IQs of children with intensively enriched environments, may increase over time. Factors that influence a child’s score on a given test of intellectual functioning and, thus, affect the accuracy of the test are motivation, emotional state, anxiety, and cultural milieu. The interactions between cognitive ability and anxiety, and depression and psychosis are complex. One study of 4,405 youth from the Canadian National Longitudinal Study of Children and Youth (NLSCY), by Weeks and colleagues (2013) found that greater cognitive ability was associated with less risk for anxiety and depressive symptoms in youth from 12 years to 13 years of age, however, by age 14 years to 15 years, cognitive ability had no effect on the odds of anxiety or depression. Perceptual and Perceptual Motor Tests. The Bender Visual Motor Gestalt Test can be given to children between the ages of 4 and 12 years. The test consists of a set of spatially related figures that the child is asked to copy. The scores are based on the number of errors. Although not a diagnostic test, it is useful in identifying developmentally age-inappropriate perceptual performances. Personality Tests. Personality tests are not of much use in making diagnoses, and they are less satisfactory than intelligence tests with regard to norms, reliability, and validity, but they can be helpful in eliciting themes and fantasies. The Rorschach test is a projective technique in which ambiguous stimuli—a set of bilaterally symmetrical inkblots—are shown to a child, who is then asked to describe what he or she sees in each. The hypothesis is that the child’s interpretation of the vague stimuli reflects basic characteristics of personality. The examiner notes the themes and patterns. A more structured projective test is the Children’s Apperception Test (CAT), which is an adaptation of the Thematic Apperception Test (TAT). The CAT consists of cards with pictures of animals in scenes that are somewhat ambiguous, but are related to parent– child and sibling issues, caretaking, and other relationships. The child is asked to describe what is happening and to tell a story about the scene. Animals are used because it was hypothesized that children might respond more readily to animal images than to human figures. Drawings, toys, and play are also applications of projective techniques that can be used during the evaluation of children. Dollhouses, dolls, and puppets have been especially helpful in allowing a child a nonconversational mode in which to express a variety of attitudes and feelings. Play materials that reflect household situations are likely to elicit a child’s fears, hopes, and conflicts about the family. Projective techniques have not fared well as standardized instruments. Rather than being considered tests, projective techniques are best considered as additional clinical modalities. Educational Tests. Achievement tests measure the attainment of knowledge and skills in a particular academic curriculum. The Wide-Range Achievement Test-Revised (WRAT-R) consists of tests of knowledge and skills and timed performances of reading, spelling, and mathematics. It is used with children from 5 years of age to adulthood. The test yields a score that is compared with the average expected score for the child’s chronological age and grade level. The Peabody Individual Achievement Test (PIAT) includes word identification, spelling, mathematics, and reading comprehension. The Kaufman Test of Educational Achievement, the Gray Oral Reading Test-Revised (GORT-R), and the Sequential Tests of Educational Progress (STEP) are achievement tests that determine whether a child has achieved the educational level expected for his or her grade level. Children whose achievement is significantly lower than expected for their grade level in one or more subjects, often exhibit a specific learning disorder. Biopsychosocial Formulation. A clinician’s task is to integrate all of the information obtained into a formulation that takes into account the biological predisposition, psychodynamic factors, environmental stressors, and life events that have led to the child’s current level of functioning. Psychiatric disorders and any specific physical, neuromotor, or developmental abnormalities must be considered in the formulation of etiologic factors for current impairment. The clinician’s conclusions are an integration of clinical information along with data from standardized psychological and developmental assessments. The psychiatric formulation includes an assessment of family function as well as the appropriateness of the child’s educational setting. A determination of the child’s overall safety in his or her current situation is made. Any suspected maltreatment must be reported to the local child protective service agency. The child’s overall well-being regarding growth, development, and academic and play activities is considered. Diagnosis Structured and semistructured (evidence-based) assessment tools often enhance a clinician’s ability to make the most accurate diagnoses. These instruments, described earlier, include the K-SADS, the CAPA, and the NIMH DISC-IV interviews. The advantages of including an evidence-based instrument in the diagnostic process include decreasing potential clinician bias to make a diagnosis without all of the necessary symptoms information, and serving as guides for the clinician to consider each symptom that could contribute to a given diagnosis. These data can enable the clinician to optimize his expertise to make challenging judgments regarding child and adolescent disorders, which may possess overlapping symptoms. The clinician’s ultimate task includes making all appropriate diagnoses according to the DSM-5. Some clinical situations do not fulfill criteria for DSM-5 diagnoses, but cause impairment and require psychiatric attention and intervention. Clinicians who evaluate children are frequently in the position of determining the impact of behavior of family members on the child’s well-being. In many cases, a child’s level of impairment is related to factors extending beyond a psychiatric diagnosis, such as the child’s adjustment to his or her family life, peer relationships, and educational placement. RECOMMENDATIONS AND TREATMENT PLAN The recommendations for treatment are derived by a clinician who integrates the data gathered during the evaluation into a coherent formulation of the factors that are contributing to the child’s current problems, the consequences of the problems, and strategies that may ameliorate the difficulties. The recommendations can be broken down into their biological, psychological, and social components. That is, identification of a biological predisposition to a particular psychiatric disorder may be clinically relevant to inform a psychopharmacologic recommendation. As part of the formulation, an understanding of the psychodynamic interactions between family members may lead a clinician to recommend treatment that includes a family component. Educational and academic problems are addressed in the formulation and may lead to a recommendation to seek a more effective academic placement. The overall social situation of the child or adolescent is taken into account when recommendations for treatment are developed. Of course, the physical and emotional safety of a child or adolescent is of the utmost importance and always at the top of the list of recommendations. The child or adolescent’s family, school life, peer interactions, and social activities often have a direct impact on the child’s success in overcoming his or her difficulties. The psychological education and cooperation of a child or adolescent’s family are essential ingredients in successful application of treatment recommendations. Communications from clinicians to parents and family members that balance the observed positive qualities of the child and family with the weak areas are often perceived as more helpful than a focus only on the problem areas. Finally, the most successful treatment plans are those developed cooperatively between the clinician, child, and family members during which each member of the team perceives that he or she has been given credit for positive contributions. REFERENCES Achenbach TM, Dumenci L, Rescorla LA. Ratings of relations between DSM-IV diagnostic categories and items of the CBCL/6–18, TRF, and YSR. 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J Am Acad Child Adolesc Psychiatry. 2005;44:712. 31.3 Intellectual Disability Intellectual disability, formerly known as mental retardation, can be caused by a range of environmental and genetic factors that lead to a combination of cognitive and social impairments. The American Association on Intellectual and Developmental Disability (AAIDD) defines intellectual disability as a disability characterized by significant limitations in both intellectual functioning (reasoning, learning, and problem solving) and in adaptive behavior (conceptual, social, and practical skills) that emerges before the age of 18 years. Wide acceptance of this definition has led to the international consensus that an assessment of both social adaptation and intelligence quotient (IQ) are necessary to determine the level of intellectual disability. Measures of adaptive function assess competency in social functioning, understanding of societal norms, and performance of everyday tasks, whereas measures of intellectual function focus on cognitive abilities. Although individuals with a given intellectual level do not all have identical levels of adaptive function, epidemiologic data suggest that prevalence of intellectual disability is largely determined by intellectual level and a level of adaptive function, which typically corresponds closely with cognitive ability. In the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5), various levels of severity of intellectual disability are determined on the basis of adaptive functioning, not on IQ scores. This change in emphasis from prior diagnostic manuals has been adopted by DSM-5 because adaptive functioning determines the level of support that is required. Furthermore, IQ scores are less valid in the lower portions of the IQ range. Making a determination of severity level of intellectual disability, according to DSM-5, includes assessment of functioning in a conceptual domain (e.g., academic skills), a social domain (e.g., relationships), and a practical domain (e.g., personal hygiene). Societal approaches to children with intellectual disability have shifted significantly over time. Historically, in the mid-1800s many children with intellectual disability were placed in residential educational facilities based on the belief that with sufficient intensive training, these children would be able to return to their families and function in society at a higher level. However, the expectation of educating these children in order to overcome their disabilities was not realized. Gradually, many residential programs increased in size, and eventually the focus began to shift from intensive education to more custodial care. Residential settings for children with intellectual disability received their maximal use in the mid-1900s, until public awareness of the crowded, unsanitary, and, in some cases, abusive conditions sparked the movement toward “deinstitutionalization.” An important force in the deinstitutionalization of children with intellectual disability was the philosophy of “normalization” in living situations, and “inclusion” in educational settings. Since the late 1960s, few children with intellectual disability have been placed in residences, and the concepts of normalization and inclusion remain prominent among advocacy groups and parents. The passage of Public Law 94–142 (the Education for all Handicapped Children Act) in 1975 mandates that the public school system provide appropriate educational service to all children with disabilities. The Individuals with Disabilities Act in 1990 extended and modified the above legislation. Currently, provision of public education for all children, including those with disabilities, “within the least restrictive environment” is mandated by law. In addition to the educational system, advocacy groups, including the Council for Exceptional Children (CEC) and the National Association for Retarded Citizens (NARC) are well known parental lobbying organizations for children with intellectual disability and were instrumental in advocating for Public Law 94–142. The American Association on Intellectual and Developmental Disabilities, formerly known as The American Association on Intellectual disability (AAMR), is the most prominent advocacy organization in this field. It has been very influential in educating the public about, and in supporting research and legislation relating to, intellectual disability. The AAIDD promotes a view of intellectual disability as a functional interaction between an individual and the environment, rather than a static designation of a person’s limitations. Within this conceptual framework, a child or adolescent with intellectual disability is determined to need intermittent, limited, extensive, or pervasive “environmental support” with respect to a specific set of adaptive function domains. These include communication, self-care, home living, social or interpersonal skills, use of community resources, self-direction, functional academic skills, work, leisure, health, and safety. The United Nations Convention on the Rights of Persons with Disabilities (2006) has created a forum to promote the full social inclusion of people with intellectual disability. Through its recognition and focus on social barriers, this international forum aims to provide protections for individuals with intellectual disability, and to seek more inclusion of those with intellectual disability in social, civic, and educational activities. NOMENCLATURE The accurate definition of intellectual disability has been a challenge for clinicians over the centuries. All current classification systems underscore that intellectual disability is based on more than cognitive deficits, that is, it also includes impaired social adaptive function. According to DSM-5, a diagnosis of intellectual disability should be made only when there are deficits in intellectual functioning and deficits in adaptive functioning (Table 31.3-1). Once intellectual disability is recognized, the level of severity is determined by the level of adaptive functional impairment. Table 31.3-1 DSM-5 Diagnostic Criteria for Intellectual Disability CLASSIFICATION DSM-5 criteria for intellectual disability include significantly subaverage general intellectual functioning associated with concurrent impairment in adaptive behavior, manifested before the age of 18. The diagnosis is made independent of coexisting physical or mental disorders. Table 31.3-2 presents an overview of developmental levels in communication, academic functioning, and vocational skills expected of persons with various degrees of intellectual disability. Table 31.3-2 Developmental Characteristics of Intellectual Disability If the clinician chooses to use a standardized test of intelligence—which is still common practice—the term significantly subaverage is defined as an IQ of approximately 70 or below or two standard deviations below the mean for the particular test. Adaptive functioning can be measured by using a standardized scale, such as the Vineland Adaptive Behavior Scale. This scale scores communications, daily living skills, socialization, and motor skills (up to 4 years, 11 months) and generates an adaptive behavior composite that is correlated with the expected skills at a given age. Approximately 85 percent of individuals who have intellectual disability fall within the DSM-5 mild intellectual disability category. This is typically defined by a Full Scale IQ between 50 and 70 and an adaptive function severity in the mild range. Adaptive function includes skills such as communication, self-care, social skills, work, leisure, and understanding of safety. Intellectual disability is influenced by genetic, environmental, and psychosocial factors. A host of subtle environmental and developmental factors, including subclinical lead intoxication and prenatal exposure to drugs, alcohol, and other toxins have been implicated as contributors to intellectual disability. Certain genetic syndromes associated with intellectual disability such as fragile X syndrome, Down syndrome, and Prader-Willi syndrome, have characteristic patterns of social, linguistic, and cognitive development and typical behavioral manifestations. DEGREES OF SEVERITY OF INTELLECTUAL DISABILITY The severity levels of intellectual disability are expressed in DSM-5 as mild, moderate, severe, and profound. “Borderline intellectual functioning,” a term previously used to describe individuals with a full scale IQ in the range of 70 to 80, is no longer described as a diagnosis in DSM-5. The term is used in DSM-5 as a condition that may be the focus of clinical attention; however, no criteria are given. Mild intellectual disability represents approximately 85 percent of persons with intellectual disability. Children with mild intellectual disability often are not identified until the first or second grade, when academic demands increase. By late adolescence, they often acquire academic skills at approximately a sixth-grade level. Specific causes for the intellectual disability are often unidentified in this group. Many adults with mild intellectual disability can live independently with appropriate support and raise their own families. IQ for this level of adaptive function may typically range from 50 to 70. Moderate intellectual disability represents about 10 percent of persons with intellectual disability. Most children with moderate intellectual disability acquire language and can communicate adequately during early childhood. They are challenged academically and often are not able to achieve above a second to third grade level. During adolescence, socialization difficulties often set these persons apart, and a great deal of social and vocational support is beneficial. As adults, individuals with moderate intellectual disability may be able to perform semiskilled work under appropriate supervision. IQ for this level of adaptive function may typically range from 35 to 50. Severe intellectual disability represents about 4 percent of individuals with intellectual disability. They may be able to develop communication skills in childhood and often can learn to count as well as recognize words that are critical to functioning. In this group, the cause for the intellectual disability is more likely to be identified than in milder forms of intellectual disability. In adulthood, persons with severe intellectual disability may adapt well to supervised living situations, such as group homes, and may be able to perform work-related tasks under supervision. IQ in individuals with this level of adaptive function may typically range from 20 to 35. Profound intellectual disability constitutes approximately 1 to 2 percent of individuals with intellectual disability. Most individuals with profound intellectual disability have identifiable causes for their condition. Children with profound intellectual disability may be taught some self-care skills and learn to communicate their needs given the appropriate training. IQ in individuals with this level of adaptive function may typically be less than 20. The DSM-5 also includes a disorder called “Unspecified Intellectual Disability” (Intellectual Developmental Disorder), reserved for individuals over the age of 5 years who are difficult to evaluate but are strongly suspected of having intellectual disability. Individuals with this diagnosis may have sensory or physical impairments such as blindness or deafness, or concurrent mental disorders, making it difficult to administer typical assessment tools. (e.g., Bayley Scales of Infant Development and Cattell Infant Scale) to aid in determining adaptive functional impairment. EPIDEMIOLOGY The majority of population-based prevalence estimates for intellectual disability in developing countries range from 10 to 15 per 1,000 children. The prevalence of intellectual disability at any one time is estimated to range from 1 to 3 percent of the population in Western societies. The incidence of intellectual disability is difficult to accurately calculate because mild disabilities may be unrecognized until middle childhood. In some cases, even when intellectual function is limited, social adaptive skills may not be challenged until late childhood or early adolescence, and the diagnosis is not made until that time. The highest incidence of intellectual disability is reported in school-age children, with the peak at ages 10 to 14 years. Intellectual disability is about 1.5 times more common among males than females. COMORBIDITY Prevalence Epidemiological surveys indicate that up to two thirds of children and adults with intellectual disability have comorbid psychiatric disorders; and this rate is several times higher than that in community samples without intellectual disability. The prevalence of psychopathology appears to be correlated with the severity of intellectual disability; the more severe the intellectual disability, the higher the risk for coexisting psychiatric disorders. An epidemiological study found that 40.7 percent of intellectually disabled children between 4 and 18 years of age met criteria for at least one additional psychiatric disorder. The severity of intellectual disability influenced the risk for particular comorbid psychiatric disorders. Disruptive and conduct-disorder behaviors occurred more frequently in those diagnosed with mild intellectual disability, whereas those with more severe intellectual disability were more likely to meet criteria for autism spectrum disorder and exhibited symptoms such as self-stimulation and selfmutilation. Comorbidity of psychiatric disorders with intellectual disability in children in this study was not correlated with age or gender. Children diagnosed with profound intellectual disability were less likely to exhibit comorbid psychiatric disorders. Psychiatric disorders among persons with intellectual disability are varied, and include mood disorders, schizophrenia, attention-deficit/hyperactivity disorder (ADHD), and conduct disorder. Children diagnosed with severe intellectual disability have a particularly high rate of comorbid autism spectrum disorder. Approximately 2 to 3 percent of those with intellectual disability meet diagnostic criteria for schizophrenia, which is several times higher than the rate for the general population. Up to 50 percent of children and adults with intellectual disability are found to meet criteria for a mood disorder when instruments such as the Kiddie Schedule for Affective Disorders and Schizophrenia (K-SADS), the Beck Depression Inventory, and the Children’s Depression Inventory were used in studies. However, a limitation of these studies is that these instruments have not been standardized within intellectual disability populations. Frequent psychiatric symptoms that occur in children with intellectual disability, outside the context of a full psychiatric disorder, include hyperactivity and short attention span, self-injurious behaviors (e.g., head-banging and self-biting), and repetitive stereotypical behaviors (hand-flapping and toe-walking). In children and adults with milder forms of intellectual disability, negative self-image, low self-esteem, poor frustration tolerance, interpersonal dependence, and a rigid problem-solving style are frequent. Neurological Disorders Seizure disorders occur more frequently in individuals with intellectual disability than in the general population, and prevalence rates for seizures increase proportionally to severity level of intellectual disability. A review of psychiatric disorders in children and adolescents with intellectual disability and epilepsy, found that approximately one third had comorbid autism spectrum disorder. The combination of intellectual disability, epilepsy, and autism spectrum disorder has been estimated to occur in 0.07 percent of the general population. Psychosocial Features A negative self-image and poor self-esteem are common features of mildly and moderately intellectually disabled persons who are aware of their social and academic differences from others. Given their experience of repeated failure and disappointment in being unable to meet their parents’ and society’s expectations, they may also be faced with falling progressively behind younger siblings. Communication difficulties further increase their vulnerability to feelings of ineptness and frustration. Inappropriate behaviors, such as withdrawal, are common. The perpetual sense of isolation and inadequacy has been linked to feelings of anxiety, anger, dysphoria, and depression. ETIOLOGY Etiological factors in intellectual disability can be genetic, developmental, environmental, or a combination. Genetic causes include chromosomal and inherited conditions; developmental and environmental factors include prenatal exposure to infections and toxins; and environmental or acquired factors include prenatal trauma (e.g., prematurity) and sociocultural factors. The severity of intellectual disability may be related to the timing and duration of a given trauma as well as to the degree of exposure to the central nervous system (CNS). In about three fourths of persons diagnosed with severe intellectual disability, the etiology is known, whereas the etiology is apparent in up to half of those diagnosed with mild intellectual disability. A study of 100 consecutive children diagnosed with intellectual disability admitted to a clinical genetics unit of a university pediatric hospital reported that in 41 percent of cases, a causative diagnosis was made. No cause is known for three fourths of persons with IQ ranging from 70 to 80 and variable adaptive functioning. Among chromosomal disorders, Down syndrome and fragile X syndrome are the most common disorders that usually produce at least moderate intellectual disability. A prototype of a metabolic disorder associated with intellectual disability is phenylketonuria (PKU). Deprivation of nutrition, nurturance, and social stimulation can potentially contribute to the development of at least mild forms of intellectual disability. Current knowledge suggests that genetic, environmental, biological, and psychosocial factors work additively in the emergence of intellectual disability. Genetic Etiological Factors in Intellectual Disability Single-Gene Causes. One of the most well-known single gene causes of intellectually disability is found in the FMR1 gene whose mutations cause fragile X syndrome. It is the most common and first X-linked gene to be identified as a direct cause of intellectual disability. Abnormalities in autosomal chromosomes are frequently associated with intellectual disability, whereas aberrations in sex chromosomes can result in characteristic physical syndromes that do not include intellectual disability (e.g., Turner’s syndrome with XO and Klinefelter’s syndrome with XXY, XXXY, and XXYY variations). Some children with Turner’s syndrome have normal to superior intelligence. Agreement exists on a few predisposing factors for chromosomal disorders —among them, advanced maternal age, increased age of the father, and X-ray radiation. Visible and Submicroscopic Chromosomal Causes of Intellectual Disability. Trisomy 21 (Down syndrome) is a prototype of a cytogenetically visible abnormality that accounts for about two-thirds of the 15 percent of intellectual disability attributable to visible abnormal cytogenetics. Other microscopically visible chromosomal abnormalities associated with intellectual disability include deletions, translocations, and supernumerary marker chromosomes. Typically, microscopic chromosome analysis is able to identify abnormalities of 5 to 10 million base pairs or greater. Submicroscopic identification requires the use of microarrays that can identify losses of chromosomal segments too small to be picked up by light microscopy. The altered copy number variants (CNVs) in submicroscopic segments of the chromosome have been identified to be associated with up to 13 to 20 percent of cases of intellectual disability. That is, the genes associated with a particular developmental abnormality have been identified to be located in the critical regions of the pathogenic copy number variants. Genetic Intellectual Disability and Behavioral Phenotype Specific and predictable behaviors have been found to be associated with certain genetically based cases of intellectual disability. These behavioral phenotypes are defined as a syndrome of observable behaviors that occur with a significantly greater probability than expected among those individuals with a specific genetic abnormality. Examples of behavioral phenotypes occur in genetically determined syndromes such as fragile X syndrome, Prader-Willi syndrome, and Down syndrome in which specific behavioral manifestations can be expected. Persons with fragile X syndrome have extremely high rates (up to three fourths of those studied) of ADHD. High rates of aberrant interpersonal behavior and language function often meet the criteria for autistic disorder and avoidant personality disorder. Prader-Willi syndrome is almost always associated with compulsive eating disturbances, hyperphagia, and obesity. Socialization is an area of weakness, especially in coping skills. Externalizing behavior problems—such as temper tantrums, irritability, and arguing—seem to be heightened in adolescence. Down Syndrome. The etiology of Down syndrome, known to be caused by an extra copy of the entire chromosome 21, makes it one of the more complex disorders. The original description of Down syndrome, first made by the English physician Langdon Down in 1866, was based on physical characteristics associated with subnormal mental functioning. Since then, Down syndrome has been the most investigated, and most discussed, syndrome in intellectual disability. Recent data have suggested that Down syndrome may be more amenable to postnatal interventions to address the cognitive deficits that it produces than was previously thought. Although still in the early stages of animal research, data from experiments with one mouse model, the Ts65Dn, indicates that pharmacologic interventions may influence learning and memory deficits known to occur in Down syndrome. Phenotypically, children with Down syndrome are observed to have characteristic physical attributes, including slanted eyes, epicanthal folds, and a flat nose. The etiology of Down syndrome is complicated by the recognition of three types of chromosomal aberrations in Down syndrome: Patients with trisomy 21 (three chromosomes 21, instead of the usual two) represent the overwhelming majority; they have 47 chromosomes, with an extra chromosome The mothers’ karyotypes are normal. A nondisjunction during meiosis, occurring for unknown reasons, is held responsible for the disorder. Nondisjunction occurring after fertilization in any cell division results in mosaicism, a condition in which both normal and trisomic cells are found in various tissues. In translocation, a fusion occurs of two chromosomes, usually 21 and 15, resulting in a total of 46 chromosomes, despite the presence of an extra chromosome 21. The disorder, unlike trisomy 21, is usually inherited, and the translocated chromosome may be found in unaffected parents and siblings. The asymptomatic carriers have only 45 chromosomes. Approximately 6,000 babies are affected with Down syndrome in the United States, which makes the incidence of Down syndrome 1 in every 700 births, or 15 per 10,000 live births. For women older than 32 years of age, the risk of having a child with Down syndrome (trisomy 21) is about 1 in 100 births, but when translocation is present, the risk is about 1 in Most children with Down syndrome are mildly to moderately intellectually disabled, with a minority having an IQ above Cognitive development appears to progress normally from birth to 6 months of age; IQ scores gradually decrease from near normal at 1 year of age to about 30 to 50 as development proceeds. The decline in intellectual function may not be readily apparent. Infant tests may not reveal the full extent of the deficits. According to anecdotal clinical reports, children with Down syndrome are typically placid, cheerful, and cooperative and adapt easily at home. With adolescence, the picture changes: youth with Down syndrome may experience more social and emotional difficulties and behavior disorders, and there is an increased risk for psychotic disorders. In Down syndrome, language function is a relative weakness, whereas sociability and social skills, such as interpersonal cooperation and conformity with social conventions, are relative strengths. Children with Down syndrome typically manifest deficits in scanning the environment; they are more likely to focus on a single stimulus, leading to difficulty noticing environmental changes. A variety of comorbid psychiatric disorders emerge in persons with Down syndrome; however, the rates appear to be lower than in children with intellectual disability and autism spectrum disorder. The diagnosis of Down syndrome is made with relative ease in an older child, but it is often difficult in newborn infants. The most important signs in a newborn include general hypotonia; oblique palpebral fissures; abundant neck skin; a small, flattened skull; high cheekbones; and a protruding tongue. The hands are broad and thick, with a single palmar transversal crease, and the little fingers are short and curved inward. Moro reflex is weak or absent. More than 100 signs or stigmata are described in Down syndrome, but rarely are all found in one person. Commonly occurring physical problems in Down syndrome include cardiac defects, thyroid abnormalities, and gastrointestinal problems. Life expectancy was once drastically limited to about the age of 40; however, currently it is vastly increased, although still not as long as those without intellectual disability. Down syndrome is characterized by deterioration in language, memory, self-care skills, and problem-solving by the third decade of life. Postmortem studies of individuals with Down syndrome older than age 40 have shown a high incidence of senile plaques and neurofibrillary tangles, similar to those seen in Alzheimer’s disease. Neurofibrillary tangles are known to occur in a variety of degenerative diseases, whereas senile plaques seem to be found most often in Alzheimer’s disease and in Down syndrome. Fragile X Syndrome. Fragile X syndrome is the second most common single cause of intellectual disability. The syndrome results from a mutation on the X chromosome at what is known as the fragile site (Xq27.3). The fragile site is expressed in only some cells, and it may be absent in asymptomatic males and female carriers. Much variability is present in both genetic and phenotypic expression. Fragile X syndrome is believed to occur in about 1 of every 1,000 males and 1 of every 2,000 females. The typical phenotype includes a large, long head and ears, short stature, hyperextensible joints, and postpubertal macroorchidism. Associated intellectual disability ranges from mild to severe. The behavioral profile of persons with the syndrome includes a high rate of ADHD, learning disorders, and autism spectrum disorder. Deficits in language function include rapid perseverative speech with abnormalities in combining words into phrases and sentences. Persons with fragile X syndrome seem to have relatively strong skills in communication and socialization; their intellectual functions seem to decline in the pubertal period. Female carriers are often less impaired than males with fragile X syndrome, but females can also manifest the typical physical characteristics and may have mild intellectual disability. Prader-Willi Syndrome. Prader-Willi syndrome is believed to result from a small deletion involving chromosome 15, occurring sporadically. Its prevalence is less than 1 in 10,000. Persons with the syndrome exhibit compulsive eating behavior and often obesity, intellectual disability, hypogonadism, small stature, hypotonia, and small hands and feet. Cat’s Cry (Cri-du-Chat) Syndrome. Children with cat’s cry syndrome have a deletion in chromosome 5. They are typically severely intellectually disabled and show many signs often associated with chromosomal aberrations, such as microcephaly, lowset ears, oblique palpebral fissures, hypertelorism, and micrognathia. The characteristic cat-like cry that gave the syndrome its name is caused by laryngeal abnormalities that gradually change and disappear with increasing age. Phenylketonuria. PKU was first described by Ivar Asbjörn Fölling in 1934 as an inborn error of metabolism. PKU is transmitted as a simple recessive autosomal Mendelian trait and occurs in about 1 of every 10,000 to 15,000 live births. For parents who have already had a child with PKU, the chance of having another child with PKU is 20 to 25 percent of successive pregnancies. PKU is reported predominantly in persons of North European origin; a few cases have been described in blacks, Yemenite Jews, and Asians. The basic metabolic defect in PKU is an inability to convert phenylalanine, an essential amino acid, to paratyrosine because of the absence or inactivity of the liver enzyme phenylalanine hydroxylase, which catalyzes the conversion. Therefore, PKU is largely preventable with a screening for it, which, if positive, should be followed with a low phenylalanine diet. Two other types of hyperphenylalaninemia have recently been described. One is caused by a deficiency of the enzyme dihydropteridine reductase, and the other to a deficiency of a cofactor, biopterin. The first defect can be detected in fibroblasts, and biopterin can be measured in body fluids. Both these rare disorders carry a high risk of fatality. Most patients with PKU are severely intellectually disabled, but some are reported to have borderline or normal intelligence. Eczema, vomiting, and convulsions occur in about one third of all patients. Although the clinical picture varies, typically, children with PKU are reported to be hyperactive and irritable. They frequently exhibit temper tantrums and often display bizarre movements of their bodies and upper extremities, including twisting hand mannerisms. Verbal and nonverbal communication is commonly severely impaired or nonexistent. The children’s coordination is poor, and they have many perceptual difficulties. Currently, the Guthrie inhibition assay is a widely applied screening test using a bacteriological procedure to detect phenylalanine in the blood. In the United States, newborn infants are routinely screened for PKU. Early diagnosis is important, because a low-phenylalanine diet, in use since 1955, significantly improves both behavior and developmental progress. The best results seem to be obtained with early diagnosis and the start of dietary treatment before the child is 6 months of age. Dietary treatment, however, is not without risk. Phenylalanine is an essential amino acid, and its omission from the diet can lead to such severe complications as anemia, hypoglycemia, or edema. Dietary treatment of PKU should be continued indefinitely. Children who receive a diagnosis before the age of 3 months and are placed on an optimal dietary regimen may have normal intelligence. A low-phenylalanine diet does not reverse intellectual disability in untreated older children and adolescents with PKU, but the diet does decrease irritability and abnormal electroencephalography (EEG) changes and does increase social responsiveness and attention span. The parents of children with PKU and some of the children’s normal siblings are heterozygous carriers. Rett syndrome. Rett syndrome, now diagnosed in the DSM-5 as a form of autism spectrum disorder, is believed to be caused by a dominant X-linked gene. It is degenerative and affects only females. In 1966, Andreas Rett reported on 22 girls with a serious progressive neurological disability. Deterioration in communications skills, motor behavior, and social functioning starts at about 1 year of age. Symptoms include ataxia, facial grimacing, teeth-grinding, and loss of speech. Intermittent hyperventilation and a disorganized breathing pattern are characteristic while the child is awake. Stereotypical hand movements, including hand-wringing, are typical. Progressive gait disturbance, scoliosis, and seizures occur. Severe spasticity is usually present by middle childhood. Cerebral atrophy occurs with decreased pigmentation of the substantia nigra, which suggests abnormalities of the dopaminergic nigrostriatal system. Neurofibromatosis. Also called von Recklinghausen’s, neurofibromatosis is the most common of the neurocutaneous syndromes caused by a single dominant gene, which may be inherited or occur as a new mutation. The disorder occurs in about 1 of 5,000 births and is characterized by café au lait spots on the skin and by neurofibromas, including optic gliomas and acoustic neuromas, caused by abnormal cell migration. Mild intellectual disability occurs in up to one third of those with the disease. Tuberous Sclerosis. Tuberous sclerosis is the second most common of the neurocutaneous syndromes; a progressive intellectual disability occurs in up to two thirds of all affected persons. It occurs in about 1 of 15,000 persons and is inherited by autosomal dominant transmission. Seizures are present in all those with intellectual disability, and in two thirds of those who are not. Infantile spasms may occur as early as 6 months of age. The phenotypic presentation includes adenoma sebaceum and ash-leaf spots that can be identified with a slit lamp. Lesch-Nyhan Syndrome. Lesch-Nyhan syndrome is a rare disorder caused by a deficiency of an enzyme involved in purine metabolism. The disorder is X-linked; patients have intellectual disability, microcephaly, seizures, choreoathetosis, and spasticity. The syndrome is also associated with severe compulsive self-mutilation by biting the mouth and fingers. Lesch-Nyhan syndrome is another example of a genetically determined syndrome with a specific, predictable behavioral pattern. Adrenoleukodystrophy The most common of several disorders of sudanophilic cerebral sclerosis, adrenoleukodystrophy is characterized by diffuse demyelination of the cerebral white matter resulting in visual and intellectual impairment, seizures, spasticity, and progression to death. The cerebral degeneration in adrenoleukodystrophy is accompanied by adrenocortical insufficiency. The disorder is transmitted by a sex-linked gene located on the distal end of the long arm of the X chromosome. The clinical onset is generally between 5 and 8 years of age, with early seizures, disturbances in gait, and mild intellectual impairment. Abnormal pigmentation reflecting adrenal insufficiency sometimes precedes the neurological symptoms, and attacks of crying are common. Spastic contractures, ataxia, and swallowing disturbances are also frequent. Although the course is often rapidly progressive, some patients may have a relapsing and remitting course. Maple Syrup Urine Disease. The clinical symptoms of maple syrup urine disease appear during the first week of life. The infant deteriorates rapidly and has decerebrate rigidity, seizures, respiratory irregularity, and hypoglycemia. If untreated, maple syrup urine disease is usually fatal in the first months of life, and the survivors have severe intellectual disability. Some variants have been reported with transient ataxia and only mild intellectual disability. Treatment follows the general principles established for PKU and consists of a diet very low in the three involved amino acids—leucine, isoleucine, and valine. Other Enzyme Deficiency Disorders. Several enzyme deficiency disorders associated with intellectual disability have been identified, and still more diseases are being added as new discoveries are made, including Hartnup disease, galactosemia, and glycogen-storage disease. Table 31.3-3 lists 30 important disorders with inborn errors of metabolism, hereditary transmission patterns, defective enzymes, clinical signs, and relation to intellectual disability. Table 31.3-3 Impairment in Disorders with Inborn Errors of Metabolism Acquired and Developmental Factors Prenatal Period. Important prerequisites for the overall development of the fetus include the mother’s physical, psychological, and nutritional health during pregnancy. Maternal chronic illnesses and conditions affecting the normal development of the fetus’s CNS include uncontrolled diabetes, anemia, emphysema, hypertension, and longterm use of alcohol and narcotic substances. Maternal infections during pregnancy, especially viral infections, have been known to cause fetal damage and intellectual disability. The extent of fetal damage depends on such variables as the type of viral infection, the gestational age of the fetus, and the severity of the illness. Although numerous infectious diseases have been reported to affect the fetus’s CNS, the following maternal illnesses have been identified to increase risk of intellectual disability in the newborn. Rubella (German measles). Rubella has replaced syphilis as the major cause of congenital malformations and intellectual disability caused by maternal infection. The children of affected mothers may show several abnormalities, including congenital heart disease, intellectual disability, cataracts, deafness, microcephaly, and microphthalmia. Timing is crucial, because the extent and frequency of the complications are inversely related to the duration of the pregnancy at the time of maternal infection. When mothers are infected in the first trimester of pregnancy, 10 to 15 percent of the children are affected, but the incidence rises to almost 50 percent when the infection occurs in the first month of pregnancy. The situation is often complicated by subclinical forms of maternal infection that go undetected. Maternal rubella can be prevented by immunization. Cytomegalic Inclusion Disease. In many cases, cytomegalic inclusion disease remains dormant in the mother. Some children are stillborn, and others have jaundice, microcephaly, hepatosplenomegaly, and radiographic findings of intracerebral calcification. Children with intellectual disability from the disease frequently have cerebral calcification, microcephaly, or hydrocephalus. The diagnosis is confirmed by positive findings of the virus in throat and urine cultures and the recovery of inclusionbearing cells in the urine. Syphilis. Syphilis in pregnant women was once the main cause of various neuropathological changes in their offspring, including intellectual disability. Today, the incidence of syphilitic complications of pregnancy fluctuates with the incidence of syphilis in the general population. Some recent alarming statistics from several major cities in the United States indicate that there is still no room for complacency. Toxoplasmosis. Toxoplasmosis can be transmitted by the mother to the fetus. It causes mild or severe intellectual disability and, in severe cases, hydrocephalus, seizures, microcephaly, and chorioretinitis. Herpes Simplex. The herpes simplex virus can be transmitted transplacentally, although the most common mode of infection is during birth. Microcephaly, intellectual disability, intracranial calcification, and ocular abnormalities may result. Human Immunodeficiency Virus (HIV). Cognitive impairments are well known to be associated with transmission of HIV from mothers to their babies. HIV may have both direct and indirect influences on the developing brain. A subset of infants born infected with HIV may develop progressive encephalopathy, intellectual disabilities, and seizures within the first year of life. Fortunately, over the last two decades, there has been a dramatic decrease in perinatal HIV transmission due to a combination of antiviral agents provided to mothers during pregnancy and delivery, obstetric interventions that reduce risk, and administration of zidovudine (ZDV) as a prophylaxis for six weeks to newborns exposed to HIV. In the United States, the highest rate of reported pediatric acquired immunodeficiency disease (AIDS) occurred in 1992, with 1,700 cases reported compared to less than 50 cases reported annually now. In the United States, fewer than 300 cases of HIV transmission from mother to child were reported in 2005. However, vertical transmission of HIV from mother to child around the world, especially in Africa, is considerable. Most babies born to HIV-infected mothers in the United States are not infected with the virus. Fetal Alcohol Syndrome. Fetal alcohol syndrome (FAS) results from prenatal alcohol exposure and can lead to a wide range of problems in the newborn. According to the Centers for Disease Control and Prevention, FAS in the United States occurs at a rate ranging from 0.2 to 1.5 per 1,000 live births. FAS is one of the leading preventable causes of intellectual disability and physical disabilities. The typical phenotypic picture of a child with FAS includes facial dysmorphism comprising hypertelorism, microcephaly, short palpebral fissures, inner epicanthal folds, and a short, turned-up nose. Often, the affected children have learning disorders and ADHD, and in some cases intellectual disability. Cardiac defects are also frequent. The entire syndrome occurs in up to 15 percent of babies born to women who regularly ingest large amounts of alcohol. Babies born to women who consume alcohol regularly during pregnancy have a high incidence of ADHD, learning disorders, and intellectual disability without the facial dysmorphism. Prenatal Drug Exposure. Prenatal exposure to opioids, such as heroin, often results in infants who are small for their gestational age, with a head circumference below the tenth percentile and withdrawal symptoms that appear within the first 2 days of life. The withdrawal symptoms of infants include irritability, hypertonia, tremor, vomiting, a high-pitched cry, and an abnormal sleep pattern. Seizures are unusual, but the withdrawal syndrome can be life-threatening to infants if it is untreated. Diazepam (Valium), phenobarbital (Luminal), chlorpromazine (Thorazine), and paregoric have been used to treat neonatal opioid withdrawal. The long-term sequelae of prenatal opioid exposure are not fully known; the children’s developmental milestones and intellectual functions may be within the normal range, but they have an increased risk for impulsivity and behavioral problems. Infants prenatally exposed to cocaine are at high risk for low birth weight and premature delivery. In the early neonatal period, they may have transient neurological and behavioral abnormalities, including abnormal results on EEG studies, tachycardia, poor feeding patterns, irritability, and excessive drowsiness. Rather than a withdrawal reaction, the physiological and behavioral abnormalities are a response to the cocaine, which may be excreted for up to a week postnatally. Complications of Pregnancy. Toxemia of pregnancy and uncontrolled maternal diabetes present hazards to the fetus and can potentially result in intellectual disability. Maternal malnutrition during pregnancy often results in prematurity and other obstetrical complications. Vaginal hemorrhage, placenta previa, premature separation of the placenta, and prolapse of the cord can damage the fetal brain by causing anoxia. The use of lithium during pregnancy was recently implicated in some congenital malformations, especially of the cardiovascular system (e.g., Ebstein’s anomaly). Perinatal Period. Some evidence indicates that premature infants and infants with low birth weight are at high risk for neurological and subtle intellectual impairments that may not be apparent until their school years. Infants who sustain intracranial hemorrhages or show evidence of cerebral ischemia are especially vulnerable to cognitive abnormalities. The degree of neurodevelopmental impairment generally correlates with the severity of the intracranial hemorrhage. Recent studies have documented that, among children with very low birth weight (less than 1,000 g), 20 percent had significant disabilities, including cerebral palsy, intellectual disability, autism, and low intelligence with severe learning problems. Very premature children and those who had intrauterine growth retardation were found to be at high risk for developing both social problems and academic difficulties. Socioeconomic deprivation can also affect the adaptive function of these vulnerable infants. Early intervention may improve their cognitive, language, and perceptual abilities. Acquired Childhood Disorders Infection. The most serious infections affecting cerebral integrity are encephalitis and meningitis. Measles encephalitis has been virtually eliminated by the universal use of measles vaccine, and the incidence of other bacterial infections of the CNS has been markedly reduced with antibacterial agents. Most episodes of encephalitis are caused by viruses. Sometimes a clinician must retrospectively consider a probable encephalitic component in a previous obscure illness with high fever. Meningitis that was diagnosed late, even when followed by antibiotic treatment, can seriously affect a child’s cognitive development. Thrombotic and purulent intracranial phenomena secondary to septicemia are rarely seen today except in small infants. Head Trauma. The best-known causes of head injury in children that produces developmental handicaps, including seizures, are motor vehicle accidents, but more head injuries are caused by household accidents, such as falls from tables, open windows, and on stairways. Child maltreatment is not infrequently implicated in head traumas or intracranial trauma such as bleeding due “shaken baby” syndrome. Asphyxia. Brain damage due to asphyxia associated with near drowning is not an uncommon cause of intellectual disability. Long-term Exposures. Long-term exposure to lead is a well-established cause of compromised intelligence and learning skills. Intracranial tumors of various types and origins, surgery, and chemotherapy can also adversely affect brain function. Environmental and Sociocultural Factors Mild intellectual disability has been associated with significant deprivation of nutrition and nurturance. Children who have endured these conditions are at risk for a host of psychiatric disorders including mood disorders, posttraumatic stress disorder, and attentional and anxiety disorders. Prenatal environment compromised by poor medical care and poor maternal nutrition may be contributing factors in the development of mild intellectual disability. Teenage pregnancies are at risk for mild intellectual disability in the baby due to the increased risk of obstetrical complications, prematurity, and low birth weight. Poor postnatal medical care, malnutrition, exposure to toxic substances such as lead, and potential physical trauma are additional risk factors for mild intellectual disabilities. Child neglect and inadequate caretaking may deprive an infant of both physical and emotional nurturances, leading to failure to thrive syndromes. DIAGNOSIS The diagnosis of intellectual disability can be made after the history is obtained, using information from a standardized intellectual assessment, and a standardized measure of adaptive function indicating that a child is significantly below the expected level in both areas. The severity of the intellectual disability will be determined on the basis of the level of adaptive function. A history and psychiatric interview are useful in obtaining a longitudinal picture of the child’s development and functioning. Examination of physical signs, neurological abnormalities, and in some cases, laboratory tests can be used to ascertain the cause and prognosis. History The clinician taking the history, which may elucidate pathways to intellectual disability, should pay particular attention to the mother’s pregnancy, labor, and delivery; the presence of a family history of intellectual disability; consanguinity of the parents; and known familial hereditary disorders. Psychiatric Interview A psychiatric interview of a child or adolescent with intellectual disability requires a high level of sensitivity in order to elicit information at the appropriate intellectual level while remaining respectful of the patient’s age and emotional development. The patient’s verbal abilities, including receptive and expressive language, can be initially screened by observing the communication between the caretakers and patient. If the patient communicates largely through gestures or sign language, the parents may serve as interpreters. Patients with milder forms of intellectual disability are often well aware of their differences from others and their failures, and may be anxious and ashamed during the interview. Approaching patients with a clear, supportive, concrete explanation of the diagnostic process, particularly patients with sufficiently receptive language ability, may allay anxiety and fears. Providing support and praise in language appropriate to the patient’s age and understanding is beneficial. Subtle direction, structure, and reinforcement may be necessary to keep patients focused on the task or topic. In general, the psychiatric examination of an intellectually disabled child or adolescent should reveal how the patient has coped with stages of development. Frustration tolerance, impulse control, and over-aggressive motor and sexual behavior are important areas of attention in the interview. It is equally important to elicit the patient’s self-image, areas of self-confidence, and an assessment of tenacity, persistence, curiosity, and willingness to explore the environment. Structured Instruments, Rating Scales and Psychological Assessment In children and adolescents who have acquired language, one of several standardized instruments that include numerous domains of cognitive function are used. For children ages 6 to 16 years, the Wechsler Intelligence Test for Children is typically administered, and for children ages 3 to 6 years, the Wechsler Preschool and Primary Scale of Intelligence-Revised is commonly used. The Stanford-Binet Intelligence Scale, Fourth Edition, has the advantage that it can be administered to children even younger, starting at age 2 years. The Kaufman Assessment Battery for Children can be used in children ages 2½ to 12½ years, whereas the Kaufman Adolescent and Adult Intelligence Test is applicable to a wide range of ages, from 11 to 85 years. All of the above standardized instruments evaluate cognitive abilities across multiple domains including verbal, performance, memory, and problem solving. Standardized instruments measuring adaptive function (functions of “everyday” life) are based on the construct that adaptive skills increase with age, and that adaptation may vary across different settings such as school, peer relationships, and family life. The Vineland Adaptive Behavior Scales can be used in infants through youth 18 years of age and includes four basic domains including Communication (Receptive, Expressive, and Written); Daily Living Skills (Personal, Domestic, and Community); Socialization (Interpersonal Relations, Play and Leisure, and Coping Skills); Motor Skills (Fine and Gross). Several behavioral rating scales have been developed for the population with intellectual disability. General behavioral ratings scales include the Aberrant Behavior Checklist (ABC) and the Developmental Behavior Checklist (DBC). The Behavior Problem Inventory (BPI) is a good screening instrument for self-injurious, aggressive, and stereotyped behaviors. The Psychopathology Inventory for Mentally Retarded Adults (PIMRA) is utilized to identify the presence of comorbid psychiatric symptoms and disorders. Examining clinicians can use several screening instruments for developmental and intellectual delay or disability in infants and toddlers. However, controversy over the predictive value of infant psychological tests is heated. Some report the correlation of abnormalities during infancy with later abnormal functioning as very low, and others report it to be very high. The correlation rises in direct proportion to the age of the child at the time of the developmental examination. Some exercises such as copying geometric figures, the Goodenough Draw-a-Person Test, the Kohs Block Test, and geometric puzzles all may be used as quick screening tests of visual-motor coordination. The Gesell and Bayley scales and the Cattell Infant Intelligence Scale are most commonly used with infants. The Peabody Vocabulary Test is the most widely used vocabulary test solely based on pictures. Other tests often found useful in detecting intellectual disability are the Bender Gestalt Test and the Benton Visual Retention Test. The psychological evaluation should assess perceptual, motor, linguistic, and cognitive abilities. Physical Examination Various parts of the body may demonstrate identifying characteristics of specific perinatal and prenatal events or conditions associated with intellectual disabilities. For example, the configuration and the size of the head may offer clues to a variety of conditions, such as microcephaly, hydrocephalus, or Down syndrome. A patient’s facial characteristics, for example, hypertelorism, a flat nasal bridge, prominent eyebrows, epicanthal folds may provide clues to a recognizable syndrome such as FAS. Additional facial characteristics including corneal opacities, retinal changes, low-set and small or misshapen ears, a protruding tongue, and disturbance in dentition may be stigmata of a variety of known syndromes. Facial expression, color and texture of the skin and hair, a high-arched palate, the size of the thyroid gland, and the proportions of a child’s trunk and extremities may offer clues for particular syndromes. The circumference of the head should be measured as part of the clinical investigation. Dermatoglyphics may offer another diagnostic tool, because uncommon ridge patterns and flexion creases on the hand are often found in persons who are intellectually disabled. Abnormal dermatoglyphics occur in chromosomal disorders and in persons who were prenatally infected with rubella. Table 31.3-4 lists syndromes with intellectual disability and their behavioral phenotypes. Table 31.3-4 Syndromes with Intellectual Disability and Behavioral Phenotypes Neurological Examination Sensory impairments occur frequently among persons with intellectual disabilities. For example, hearing impairment occurs in 10 percent of persons with intellectual disability, a rate that is four times that of the general population. Visual disturbances can range from blindness to disturbances of spatial concepts, design recognition, and concepts of body image. Seizure disorders occur in about 10 percent of intellectually disabled populations and in one third of those with severe intellectual disability. Neurological abnormalities increase in incidence and severity in direct proportion to the degree of intellectual disability. Disturbances in motor areas are manifested in abnormalities of muscle tone (spasticity or hypotonia), reflexes (hyperreflexia), and involuntary movements (choreoathetosis). Less disability may also be associated with clumsiness and poor coordination. CLINICAL FEATURES Mild intellectual disability may not be recognized or diagnosed in a child until school challenges the child’s social and communication skills. Cognitive deficits include poor ability to abstract and egocentric thinking, both of which become more easily evident as a child reaches middle childhood. Children with milder intellectual disabilities may function academically at the high elementary level and may acquire vocational skills sufficient to support themselves in some cases; however, social assimilation may be problematic. Communication deficits, poor self-esteem, and dependence may further contribute to a relative lack of social spontaneity. Moderate levels of intellectual disability are significantly more likely to be observed at a younger age, since communication skills develop more slowly and social isolation may ensue in the elementary school years. Academic achievement is usually limited to the middle-elementary level. Children with moderate intellectual deficits benefit from individual attention focused on the development of self-help skills. However, these children are aware of their deficits and often feel alienated from their peers and frustrated by their limitations. They continue to require a relatively high level of supervision but can become competent at occupational tasks in supportive settings. Severe intellectual disability is typically obvious in the preschool years; affected children have minimal speech and impaired motor development. Some language development may occur in the school-age years. By adolescence, if language has not improved significantly, poor, nonverbal forms of communication may have evolved. Behavioral approaches are useful means to promote some self-care, although those with severe intellectual disability generally need extensive supervision. Children with profound intellectual disability require constant supervision and are severely limited in both communication and motor skills. By adulthood, some speech development may be present, and simple self-help skills may be acquired. Clinical features frequently observed in populations with intellectual disability either in isolation or as part of a mental disorder, include hyperactivity, low frustration tolerance, aggression, affective instability, repetitive and stereotypic motor behaviors, and selfinjurious behaviors. Self-injurious behaviors occur more frequently and with greater intensity in more severe intellectual disability. Dylan was a full-term infant, the second child born to his 42-year-old mother, a medical technician, and 48-year-old father, a high school basketball coach. The pregnancy was unremarkable, and Dylan’s sister, who was two years older, was healthy and developing normally. The family lived in a rural town in the Midwest. Dylan was an extremely fussy, active newborn with extended periods of crying that the pediatrician labeled classic colic. As a newborn, it was noticed that Dylan seemed to have large ears and strabismus, which the pediatrician said would probably resolve spontaneously. At 2 months of age, at a regular pediatric visit, a systolic heart murmur was heard and electrocardiography (ECG) revealed a mitral valve prolapse. Because Dylan was not cyanotic and had no other cardiac symptoms, no treatment was recommended except monitoring. Although Dylan became less fussy over time, he remained very active, did not sleep through the night, and was a picky eater, refusing solid foods. Milestones were slightly delayed, with Dylan sitting unassisted at 10 months and walking at 18 months. Language was also delayed, and, although his first words appeared at 20 months, Dylan had always made his wants and needs known. Dylan’s parents were concerned about his activity level and his developmental delays compared with his sister; however, they were reassured by the pediatrician’s sense that boys often develop more slowly than girls in the first two years. When Dylan was 3 years of age, his preschool teacher noted that he was unable to pay attention and he was hyperactive compared to his classmates, prompting his parents to obtain a developmental evaluation. Results showed modest delays in cognitive, linguistic, and motor functioning, with a developmental quotient (DQ) of 74. Dylan was described as inattentive, shy, and anxious, and he had poor eye contact. Enrolled in a special kindergarten, Dylan remained in a combination of special education and mainstreamed classes throughout his academic life. At 7 years of age, the school psychologist evaluated Dylan and results indicated that he met criteria for a “learning disability” profile. Dylan had an overall IQ of 66, with close to average functioning in short-term memory and pronounced deficits in longterm memory, expressive language, and visual-spatial functioning. Dylan struggled with writing tasks and arithmetic, but loved science. Due to his significant problems with inattention and hyperactivity, Dylan was placed on Concerta, which was beneficial, and titrated up to 54 mg per day. He displayed transient, intense interests in unusual items, such as vacuum cleaners. When Dylan reached the older elementary grades, he began to have more difficulty socially, and he was bullied about being in special education, and teased for his long head and big ears. As he entered adolescence, Dylan became increasingly anxious, so much so that he occasionally rubbed his hands or rocked, and he “fretted” about day-to-day issues and what would happen next. His long-term sensitivities to loud sounds seemed to wane slightly, but he developed fears of storm clouds and dogs and refused to ride on elevators. He became tearful and upset after his older sister left for a party, and worried that she might have a car accident. Dylan was very shy and would occasionally pace with worry and complain of stomachaches, but he attended school and had a small group of acquaintances in the Special Olympics bowling league. He enjoyed activities that did not involve much talking or sustained attention. When Dylan was 17 years of age, his parents happened to watch a television documentary on genetic causes of intellectual disability. They were overwhelmed by the similarities between Dylan and some of the people described in the program. They later described the experience as a “jolt.” They had always accepted Dylan, quirks and all, and had stopped pushing their doctors for reasons “why” when Dylan was a preschooler. Nevertheless, they immediately called the informational number offered in the show, and within 2 months, they had the genetic tests done that confirmed a diagnosis of fragile X syndrome. Although Dylan’s day-to-day life did not change dramatically after the diagnosis, his parents reported a big difference in their approach to his shyness, restricted interests, and inattention. Dylan was later treated for anxiety with a selective serotonin reuptake inhibitor (SSRI) antidepressant, which decreased his social anxiety and facilitated activities with a few peers. Dylan’s parents reported a mixture of feelings at having such a late diagnosis—disappointment in their doctors, relief in finally knowing, and twinges of guilt. They were energized by Dylan’s positive responses to treatments for his attentional and anxiety symptoms and were pleased with Dylan’s recent increased interest in sharing activities with classmates and peers. LABORATORY EXAMINATION Laboratory tests that may elucidate the causes of intellectual disability include chromosomal analysis, urine and blood testing for metabolic disorders, and neuroimaging. Chromosomal abnormalities are the single most known common cause of intellectual disability. Chromosome Studies Chromosome analysis is commonly obtained when multiple physical anomalies, developmental delays, and intellectual disability present together. Current techniques are able to chromosomal regions with specific fluorescent in situ hybridization (FISH) markers, leading to microscopic deletions being identified in up to 7% of persons with moderate to severe intellectual disability. A history of growth retardation, the presence of microcephaly, a family history of intellectual disability, short stature, hypertelorism, and other facial abnormalities increase the risk for finding subtelomeric defects. Amniocentesis, in which a small amount of amniotic fluid is removed from the amniotic cavity transabdominally at about the 15 weeks of gestation, has been useful in diagnosing prenatal chromosomal abnormalities. Its use is considered when an increased fetal risk exists, such as with increased maternal age. Amniotic fluid cells, mostly fetal in origin, are cultured for cytogenetic and biochemical studies. Chronic villi sampling (CVS) is a screening technique to determine fetal chromosomal abnormalities. It is done at 8 to 10 weeks of gestation, 6 weeks earlier than amniocentesis is done. The results are available in a short time (hours or days) and, if the result is abnormal, the decision to terminate the pregnancy can be made within the first trimester. The procedure has a miscarriage risk between 2 and 5 percent; the risk in amniocentesis is lower (1 in 200). A non-invasive blood test called materniT21 is a proprietary prenatal test that detects abnormalities of chromosomes 21,18,13, X and Y. It is highly specific for Down syndrome (Figure 31.3-1). There is no risk of miscarriage. FIGURE 31.3-1 A. young child with Down syndrome. B. A young adult with fragile X syndrome. (Courtesy of L.S. Syzmanski, M.D., and A.C. Crocker, M.D.) Urine and Blood Analysis Lesch-Nyhan syndrome, galactosemia, PKU, Hurler’s syndrome (Figure 31.3-2), and Hunter’s syndrome (Figure 31.3-3) are examples of disorders characterized by intellectual disability that can be identified through assays of the appropriate enzyme or organic or amino acids. Enzymatic abnormalities in chromosomal disorders, particularly Down syndrome, promise to become useful diagnostic tools. FIGURE 31.3-3 Two brothers, age 6 and 8 years, with Hunter’s syndrome, shown with their normal older sister. They have had significant developmental delay, trouble with recurrent respiratory infection, and behavioral abnormalities. (Courtesy of L.S. Syzmanski, M.D., and A.C. Crocker, M.D.) FIGURE 31.3-2 A 6-year-old girl with Hurler’s syndrome. Her care has involved a class for seriously multihandicapped children, attention to cardiac problems, and special counseling for patients. (Courtesy of L.S. Syzmanski, M.D., and A.C. Crocker, M.D.) Electroencephalography Electroencephalography is indicated whenever a seizure disorder is considered. “Nonspecific” EEG changes, characterized by slow frequencies with bursts of spikes and sharp or blunt wave complexes are found with greater frequency among populations with intellectual disability than in the general population; however, these findings do not elucidate specific diagnoses. Neuroimaging Neuroimaging studies with populations of intellectually disabled patients using either computerized tomography (CT) or magnetic resonance imaging (MRI) have found high rates of abnormalities in those patients with microcephaly, significant delay, cerebral palsy, and profound disability. Among patients with intellectual disability, neuroimaging is indicated, accompanying findings that suggest seizures, microcephaly or macrocephaly, loss of previously acquired skills, or neurologic signs such as dystonia, spasticity, or altered reflexes. Although clinically not diagnostic, neuroimaging studies are currently also utilized to gather data that may eventually uncover biological mechanisms contributing to intellectual disability. Structural MRI, functional MRI (fMRI), and diffusion tensor imaging (DTI) are utilized in current research. For example, current data suggest that individuals with fragile X syndrome and concurrent attentional deficits are also more likely to show aberrant frontal-striatal pathways on MRI than those patients without attentional problems. MRI is also useful to elucidate myelination patterns. MRI studies can also provide a baseline for comparison of a later, potentially degenerative process in the brain. Hearing and Speech Evaluations Hearing and speech should be evaluated routinely. Speech development may be the most reliable criterion in investigating intellectual disability. Various hearing impairments often occur in persons who are intellectually disabled, but in some instances hearing impairments can simulate intellectual disability. The commonly used methods of hearing and speech evaluation, however, require the patient’s cooperation and, thus, are often unreliable in severely disabled persons. COURSE AND PROGNOSIS Although the underlying intellectual impairment does not improve, in most cases of intellectual disability, level of adaptation increases with age and can be influenced positively by an enriched and supportive environment. In general, persons with mild and moderate mental intellectual disabilities have the most flexibility in adapting to various environmental conditions. Comorbid psychiatric disorders negatively impact overall prognosis. When psychiatric disorders are superimposed on intellectual disability, standard treatments for the comorbid mental disorders are often beneficial; however, less robust responses and increased vulnerability to side effects of psychopharmacologic agents are often the case. DIFFERENTIAL DIAGNOSIS By definition, intellectual disability must begin before the age of 18. In some cases, severe child maltreatment in the form of neglect or abuse may contribute to delays in development, which can appear to be intellectual disability. However these damages are partially reversible when a corrective, enriched, and stimulating environment is provided in early childhood. Sensory disabilities, especially deafness and blindness, can be mistaken for intellectual disability when a lack of awareness of the sensory deficit leads to inappropriate testing. Expressive and receptive speech disorders may give the impression of intellectual disability in a child of average intelligence, and cerebral palsy may be mistaken for intellectual disability. Chronic, debilitating medical diseases may depress and delay a child’s functioning and achievement, despite normal intelligence. Seizure disorders, especially those that are poorly controlled, may contribute to persisting intellectual disability. Specific organic syndromes leading to isolated handicaps such as failure to read (alexia), failure to write (agraphia), or failure to communicate (aphasia), may occur in a child of normal and even superior intelligence. Children with learning disorders (which can coexist with intellectual disability) experience a delay or failure of development in a specific area, such as reading or mathematics, but they develop normally in other areas. In contrast, children with intellectual disability show general delays in most areas of development. Intellectual disability and autism spectrum disorder (ASD) often coexist; 70 to 75 percent of those with ASD have an IQ below 70. In addition, epidemiologic data indicate that ASD occurs in approximately 19.8% of persons with intellectual disability. Children with ASD have relatively more severe impairment in social relatedness and language than other children with the same level of intellectual disability. A child younger than the age of 18 years with significant adaptive functional impairment, with an IQ less than 70, who also meets diagnostic criteria for dementia, will receive both a diagnosis of dementia and intellectual disability. However, a child whose IQ drops below 70 after the age of 18 years with newly acquired cognitive impairment will receive only the diagnosis of dementia. TREATMENT Interventions for children and adolescents with intellectual disability are based on an assessment of social, educational, psychiatric, and environmental needs. Intellectual disability is associated with a variety of comorbid psychiatric disorders that often require specific treatment, in addition to psychosocial support. Of course, when preventive measures are available, the optimal approach includes primary, secondary, and tertiary interventions. Primary Prevention Primary prevention comprises actions taken to eliminate or reduce the conditions that lead to development of intellectual disability, as well as associated disorders. For example, screening babies for PKU, and administrating a low phenylalanine diet when PKU is present, significantly alters the emergence of intellectual disability in those affected children. Additional primary prevention steps include education of the general public about strategies to prevent intellectual disability, such as abstinence from alcohol during pregnancy; continuing efforts of health professionals to ensure and upgrade public health policies; and legislation to provide optimal maternal and child health care. Family and genetic counseling helps reduce the incidence of intellectual disability in a family with a history of a genetic disorder. Secondary and Tertiary Prevention Prompt attention to medical and psychiatric complications of intellectual disability can diminish their course (secondary prevention) and minimize the sequelae or consequent disabilities (tertiary prevention). Hereditary metabolic and endocrine disorders, such as PKU and hypothyroidism, can be treated effectively in an early stage by dietary control or hormone replacement therapy. Educational Interventions. Educational settings for children with intellectual disability should include a comprehensive program that addresses academics and training in adaptive skills, social skills, and vocational skills. Particular attention should focus on communication and efforts to improve the quality of life. Behavioral and Cognitive-Behavioral Interventions. The difficulties in adaptation among the intellectual disability populations are widespread and so varied that several interventions alone or in combination may be beneficial. Behavior therapy has been used for many years to shape and enhance social behaviors and to control and minimize aggressive and destructive behaviors. Positive reinforcement for desired behaviors and benign punishment (e.g., loss of privileges) for objectionable behaviors has been helpful. Cognitive therapy, such as dispelling false beliefs and relaxation exercises with self-instruction, has also been recommended for intellectually disabled persons who can follow the instructions. Psychodynamic therapy has been used with patients and their families to decrease conflicts about expectations that result in persistent anxiety, rage, and depression. Psychiatric treatment modalities require modifications that take into consideration the patient’s level of intelligence. Family Education. One of the most important areas that a clinician can address is educating the family of a child or adolescent with intellectual disability about ways to enhance competence and self-esteem while maintaining realistic expectations for the patient. The family often finds it difficult to balance the fostering of independence and the providing of a nurturing and supportive environment for an intellectually disabled child, who is likely to experience some rejection and failure outside the family context. The parents may benefit from continuous counseling or family therapy and should be allowed opportunities to express their feelings of guilt, despair, anguish, recurring denial, and anger about their child’s disorder and future. The psychiatrist should be prepared to give the parents all the basic and current medical information regarding causes, treatment, and other pertinent areas (e.g., special training and the correction of sensory defects). Social Intervention. One of the most prevalent problems among persons with intellectual disability is a sense of social isolation and social skills deficits. Thus, improving the quantity and quality of social competence is a critical part of their care. Special Olympics International is the largest recreational sports program geared for this population. In addition to providing a forum to develop physical fitness, Special Olympics also enhances social interactions, friendships, and (it is hoped) general selfesteem. A recent study confirmed positive effects of the Special Olympics on the social competence of the intellectually disabled adults who participated. Psychopharmacologic Interventions. Pharmacological approaches to the treatment of behavioral and psychological symptoms in children with intellectual disability follow the paradigms of the evidence-based literature on treatment for all children with psychiatric disorders. However, given the paucity of randomized trials in the childhood intellectual disability population, an empirical approach must also be taken. COMMON COMORBID PSYCHIATRIC SYMPTOMS AND DISORDERS Aggression, Irritability, and Self-injurious Behavior. Risperidone has been well documented as an efficacious treatment for irritability (aggression, self-injury, and severe tantrums) in children with ASD by the Research Units on Pediatric Psychopharmacology (RUPP, Autism Network 2002). Risperidone is helpful in treating disruptive behaviors in children with below-average intelligence, and has a good overall safety and tolerability profile. Cognitive testing has demonstrated small but significant improvement in cognitive ability with risperidone use. Children and adolescents with intellectual disability appear to be at higher risk for the development of tardive dyskinesia after use of antipsychotic medications; however, the atypical antipsychotics, including risperidone and clozapine (Clozaril), may provide some relief with a decreased risk of tardive dyskinesia. There is evidence to support the use of antipsychotic agents in the management of self-injurious behavior (SIB). Although data exist on the efficacy of thioridazine in improving SIB, a “black box” warning regarding QT prolongation with thioridazine has drastically diminished use of this drug, and atypical antipsychotic agents are currently preferred. Attention-Deficit/Hyperactivity Disorder. Estimates of attention deficit/hyperactivity (ADHD) and ADHD-like symptoms among children with sub average intelligence, genetic disorders, and developmental delay is estimated to be significantly higher than rates in the community. Randomized clinical trials of several psychopharmacologic agents have been done in children with sub-average intelligence. These include trials with methylphenidate, clonidine, and risperidone. The existing data for the treatment of ADHD and ADHD-like symptoms in youth with sub-average intelligence and developmental disorders suggest that agents, particularly stimulants used to treat ADHD in typically developing children, provide some degree of benefit to children with intellectual disability and ADHD. However, the occurrence of side effects within this population appears to be greater than in children with ADHD in the community. Thus, recommendations regarding treatment of ADHD in children and adolescents with comorbid ADHD include close monitoring for side effects. Studies of methylphenidate (Ritalin) treatment in those mildly intellectually disabled with ADHD have shown significant improvement in the ability to maintain attention and to stay focused on tasks. Methylphenidate treatment studies have not shown evidence of long-term improvement in social skills or learning. Risperidone also has been found to be beneficial in reducing symptoms of ADHD in this population; however, it may produce an increase in serum prolactin level. It is prudent to begin with a trial of a stimulant medication before the use of antipsychotic agents for the treatment of ADHD symptoms in intellectual disorder. A new extended release methylphenidate oral suspension (Quillivant XR, 2013) is currently available in 25 mg/5 ml preparation, and is taken once daily for the treatment of ADHD in children 6 to 12 years of age. Amphetamine-based preparations have been shown to be efficacious in treating ADHD in typically developing children; however, it does not appear that these stimulant preparations have been specifically studied in children with intellectual disability. Clonidine has been used clinically in this population, especially to ameliorate hyperactivity and impulsivity. Although there are scant data, clinical ratings by parents and clinicians suggest its efficacy. Atomoxetine has been shown to be efficacious in children diagnosed with ASD and prominent ADHD features, and it is used clinically in the intellectually disabled population. Depressive Disorders. The identification of depressive disorders among individuals with intellectual disability requires careful evaluation, since it may be inadvertently overlooked when behavioral problems are prominent. There have been anecdotal reports of disinhibition in response to SSRIs (e.g., fluoxetine [Prozac], paroxetine [Paxil], and sertraline [Zoloft]) in intellectually disabled individuals with ASD. Given the relative safety of SSRI antidepressants, a trial is indicated when a depressive disorder is diagnosed in a child or adolescent with intellectual disability. Stereotypical Motor Movements. Antipsychotic medications—historically, haloperidol (Haldol) and chlorpromazine, and currently, the atypical antipsychotics—are used in the treatment of repetitive self-stimulatory behaviors in children with intellectual disability when these behaviors are either harmful to the child or disruptive. Anecdotal reports indicate that these agents may diminish self-stimulatory behaviors; however, there is no improvement seen in adaptive behavior. Obsessive-compulsive symptoms often overlap with the repetitive stereotypical behaviors seen in children and adolescents with intellectual disability, particularly in those with comorbid ASD. SSRIs such as fluoxetine, fluvoxamine (Luvox), paroxetine, and sertraline have been shown to have efficacy in treating obsessive-compulsive symptoms in children and adolescents and may have some efficacy for stereotyped motor movements. Explosive Rage Behavior. Antipsychotic medications, particularly risperidone, have been shown to be efficacious for the treatment of explosive rage. Systematic controlled studies are indicated to confirm the efficacy of these drugs in the treatment of rage outbursts. βAdrenergic receptor antagonists (beta-blockers), such as propranolol (Inderal), have been reportedly anecdotally to result in fewer explosive rages in some children with intellectual disability and ASD. SERVICES AND SUPPORT FOR CHILDREN WITH INTELLECTUAL DISABILITY Early Intervention Early intervention programs serve individuals for the first 3 years of life. Such services are generally provided by the state and begin with a specialist visiting the home for several hours per week. Since the passage of Public Law 99–447, the Education of the Handicapped Amendments of 1986, early intervention services for the entire family are emphasized. Agencies are required to develop an Individualized Family Service Plan (IFSP) for each family, which identifies specific interventions to best help the family and child. School From ages 3 to 21 years, school is responsible by law to provide appropriate educational services to children and adolescents with intellectual disability in the United States. These mandates were created by the passage of Public Law 94–142, the Education for all Handicapped Children Act of 1975, and expanded with the addition of the Individuals with Disabilities Act (IDEA) of 1990. Through these laws, public schools must develop and provide an individualized educational program for each student with intellectual disability, determined at a meeting designated as the Individualized Education Plan (IEP) with school personnel and the family. The education must be provided for the child in the “least restrictive environment” that will allow the child to learn. Supports A wide variety of organized groups and services are available for children with intellectual disability and their families. These include short-term respite care, which allow families a break and is generally set up by state agencies. Other programs include the Special Olympics, which allows children with intellectual disability to participate in team sports and in sports competitions. Many organizations also exist for families who wish to connect with others who have children with intellectual disability. REFERENCES American Association on Intellectual and Developmental Disabilities. Overview of intellectual disability: Definition, classifications and systems of support. 2010. Arnold LE, Farmer C, Kraemer HC, Davies M, Witwer A, Chuang S, DiSilvestro R, McDougle CJ, McCracken J, Vitello B, Aman M, Scahill L, Posey DJ, Swiezy NB. Moderators, mediators, and other predictors of risperidone response in children with autistic disorder and irritability. J Child Adolesc Psychopharmacol. 2010;20:83–93,196–1205. Boulet S, Boyle C, Schieve L. Trends in health care utilization and health impact of developmental disabilities, 1997–2005. Arch Pediatr Adolesc Med. 2009;163:19–26. Correia Filho AG, Bodanase R, Silva TL, Alvarez JP, Aman M, Rohde LA. Comparison of risperidone and methylphenidate for reducing ADHD symptoms in children and adolescents with moderate intellectual disability. J Am Acad Child Adolesc Psychiatry. 2005;44:748. Ellison JW, Rosengeld JA, Shaffer LG. Genetic basis of intellectual disability. Annu Rev Med. 2013 Fowler MG, Gable AR, Lampe MA, Etima M Owor M. Perinatal HIV and its prevention: Progress toward an HIV-free generation. Clin Perinatol. 2010;37:699–719. Gothelf D, Furfaro JA, Penniman LC, Glover GH, Reiss AL. The contribution of novel brain imaging techniques to understanding the neurobiology of intellectual disability and developmental disabilities. Ment Retard Dev Disabil Res Rev. 04 - 31.4 Communication Disorders 31.4 Communication Disorders 2005;11:331. Ismail S, Buckley S, Budacki R, Jabbar A, Gallicano GI. Screening, diagnosing and prevention of fetal alcohol syndrome: Is this syndrome treatable? Dev Neurosci. 2010;32:91–100. Obi O, Braun KVN, Baio J, Drews-Botsch C, Devine O, Yeargin-Allsopp M. Effect of incorporating adaptive functioning scores on the prevalence of intellectual disability. Am J Intellect Dev Disabil. 2011;116:360–370. Reyes M, Croonenberghs J, Augustybs I, Eerdekens M. Long-term use of risperidone in children with disruptive behavior disorders and subaverage intelligence: Efficacy, safety, and tolerability. J Child Adolesce Psychopharmacol. 2006;16:60– 27. Rowles BM, Findling RL. Review of pharmacotherapy options for the treatment of attention-deficit/hyperactivity disorder (ADHD) and ADHD-like symptoms in children and adolescents with developmental disorders. Dev Disabil Res Rev. 2010;16:273–282. Stuart H. United Nations convention on the rights of persons with disabilities: A roadmap for change. Curr Opin Psychiatry.2012;25:365–369. Sturgeon X, Le T, Ahmed MM, Gardiner KJ. Pathways to cognitive deficits in Down syndrome. Prog Brain Res. 2012;197:73–100. United Nations General Assembly. Convention on the Rights of Persons with Disabilities (CRPD). Geneva: United Nations; December 13, 2006. Wijetunge LS, Chatterji S, Wyllie DJ, Kind PC. Fragile X syndrome: From targets to treatments. Neuropharmacology. 2013;68:83–96. Willen EJ. Neurocognitive outcomes in pediatric HIV. Ment Retard Dev Disabil Res Rev. 2006;12:223–228. 31.4 Communication Disorders Communication disorders range from mild delays in acquiring language to expressive or mixed receptive–expressive disorders, phonological disorders, and stuttering, which may remit spontaneously or persist into adolescence or even adulthood. Language delay is one of the most common very early childhood developmental delays, affecting up to approximately 7 percent of 5-year-olds. The rates of language disorders are understandably higher in preschoolers than in school-age children; rates were reported to be close to 20 percent of 4-year-olds in the Early Language in Victoria Study (ELVS). To communicate effectively, children must have a mastery of multiple aspects of language—that is, the ability to understand and express ideas—using words and speech, and express themselves in vernacular language. In the Fifth Edition of the American Psychiatric Association’s Diagnostic and Statistical Manual of Mental Disorders (DSM-5), Language Disorder includes both expressive and mixed receptive–expressive problems. DSM-5 speech disorders include Speech Sound Disorder (formerly known as Phonological Disorder) and Childhood-Onset Fluency Disorder (Stuttering). Children with expressive language deficits have difficulties expressing their thoughts with words and sentences at a level of sophistication expected for their age and developmental level in other areas. These children may struggle with limited vocabularies, speak in sentences that are short or ungrammatical, and often present descriptions of situations that are disorganized, confusing, and infantile. They may be delayed in developing an understanding and a memory of words compared with others their age. Children with language disorder are at higher risk for developing reading difficulties. Current expert consensus considers reading comprehension impairment a form of language impairment, distinct from other reading deficits such as dyslexia. Language and speech are pragmatically intertwined, despite the distinct categories of language disorders and speech disorders in DSM-5. Language competence spans four domains: phonology, grammar, semantics, and pragmatics. Phonology refers to the ability to produce sounds that constitute words in a given language and the skills to discriminate the various phonemes (sounds that are made by a letter or group of letters in a language). To imitate words, a child must be able to produce the sounds of a word. Grammar designates the organization of words and the rules for placing words in an order that makes sense in that language. Semantics refers to the organization of concepts and the acquisition of words themselves. A child draws from a mental list of words to produce sentences. Children with language impairments exhibit a wide range of difficulties with semantics that include acquiring new words, storage and organization of known words, and word retrieval. Speech and language evaluations that are sufficiently broad to test all of the preceding skill levels will be more accurate in evaluating a child’s remedial needs. Pragmatics has to do with skill in the actual use of language and the “rules” of conversation, such as pausing so that a listener can answer a question and knowing when to change the topic when a break occurs in a conversation. By age 2 years, toddlers without speech or language delay may know a few words or up to 200 words, and by age 3 years, most children understand the basic rules of language and can converse effectively. Table 31.4a-1 provides an overview of typical milestones in language and nonverbal development. Table 31.4a-1 Normal Development of Speech, Language, and Nonverbal Skills in Children Over the last decade there have been an increasing number of investigative studies of speech and language interventions with positive outcomes identified in numerous areas of language. These include improvements in expressive vocabulary, syntax usage, and overall phonologic development. Most interventions are targeted strategies for the child’s particular deficit, and delivered by speech and language therapists. 05 - 31.4a Language Disorder 31.4a Language Disorder 31.4a Language Disorder Language disorder consists of difficulties in the acquisition and use of language across many modalities, including spoken and written, due to deficits in comprehension or production based on both expressive and receptive skills. These deficits include reduced vocabulary, limited abilities in forming sentences using the rules of grammar, and impairments in conversing based on difficulties using vocabulary to connect sentences in descriptive ways. EXPRESSIVE LANGUAGE DEFICITS Expressive language deficits are present when a child demonstrates a selective deficit in expressive language development relative to receptive language skills and nonverbal intellectual function. Infants and young children with typically developing expressive language will laugh and coo by about 6 months of age, babble and verbalize syllables such as dadada or mamama by about 9 months, and by one year, babies imitate vocalizations and can often speak at least one word. Expressive speech and language generally continue to develop in a stepwise fashion, so that at a year and a half, children typically can say a handful of words, and by 2 years, children generally are combining words into simple sentences. By the age of 2½ years, children can name an action in a picture, and are able to make themselves understood through their verbalizations about half of the time. By 3 years, most children can speak understandably, and are able to name a color and describe what they see with several adjectives. At 4 years, children typically can name at least 4 colors, and can converse understandably. In the early years, prior to entering preschool, the development of proficiency in vocabulary and language usage is highly variable, and influenced by the amount and quality of verbal interactions with family members, and after beginning school, a child’s language skills are significantly influenced by the level of verbal engagement in school. A child with expressive language deficits may be identified using the Wechsler Intelligence Scale for Children III (WISC-III), in that verbal intellectual level may appear to be depressed compared with the child’s overall intelligence quotient (IQ). A child with expressive language problems is likely to function below the expected levels of acquired vocabulary, correct tense usage, complex sentence constructions, and word recall. Children with expressive language deficits often present verbally as younger than their age. Language disability can be acquired during childhood (e.g., secondary to a trauma or a neurological disorder), although less frequently, or it can be developmental; it is usually congenital, without an obvious cause. Most childhood language disorders fall into the developmental category. In either case, deficits in receptive skills (language comprehension) or expressive skills (ability to use language) can occur. Expressive language disturbance often appears in the absence of comprehension difficulties, whereas receptive dysfunction generally diminishes proficiency in the expression of language. Children with expressive language disturbance alone have better prognoses, and less interference with learning, than children with mixed receptive–expressive language disturbances. Although language use depends on both expressive and receptive skills, the degree of deficits in a given individual may be severe in one area, and hardly impaired at all in the other. Thus, language disorder can be diagnosed in children with expressive language disturbance in the absence of receptive language problems, or when both receptive and expressive language syndromes are present. In general, when receptive skills are sufficiently impaired to warrant a diagnosis, expressive skills are also impaired. In DSM-5 language disorder is not limited to developmental language disabilities; acquired forms of language disturbances are included. To meet the DSM-5 criteria for language disorder, patients must have scores on standardized measures of expressive or receptive language markedly below those of standardized nonverbal IQ subtests and standardized tests. Epidemiology The prevalence of expressive language disturbance decreases with a child’s increasing age, and overall it is estimated to be as high as 6 percent in children between the ages of 5 and 11 years of age. Surveys have indicated rates of expressive language as high as 20 percent in children younger than 4 years of age. In school-age children over the age of 11 years, the estimates are lower, ranging from 3 percent to 5 percent. The disorder is two to three times more common in boys than in girls and is most prevalent among children whose relatives have a family history of phonologic disorder or other communication disorders. Comorbidity Children with language disorder have above-average rates of comorbid psychiatric disorders. In one large study of children with speech and language disorders, the most common comorbid disorders were attention-deficit/hyperactivity disorder (ADHD; 19 percent), anxiety disorders (10 percent), oppositional defiant disorder, and conduct disorder (7 percent combined). Children with expressive language disorder are also at higher risk for a speech disorder, receptive difficulties, and other learning disorders. Many disorders—such as reading disorder, developmental coordination disorder, and other communication disorders—are associated with expressive language disturbance. Children with expressive language disturbance often have some receptive impairment, although not always sufficiently significant for the diagnosis of language disorder on this basis. Speech sound disorder, formerly known as phonologic disorder, is commonly found in young children with language disorder, and neurologic abnormalities have been reported in a number of children, including soft neurologic signs, depressed vestibular responses, and electroencephalography (EEG) abnormalities. Etiology The specific causes of the expressive components of language disorder are likely to be multifactorial. Scant data are available on the specific brain structure of children with language disorder, but limited magnetic resonance imaging (MRI) studies suggest that language disorders are associated with diminished left–right brain asymmetry in the perisylvian and planum temporale regions. Results of one small MRI study suggested possible inversion of brain asymmetry (right > left). Left-handedness or ambilaterality appears to be associated with expressive language problems with more frequency than right-handedness. Evidence shows that language disorders occur more frequently within some families, and several studies of twins show significant concordance for monozygotic twins with respect to language disorders. Environmental and educational factors are also postulated to contribute to developmental language disorders. Diagnosis Language disorder of the expressive disturbance type is diagnosed when a child has a selective deficit in language skills and is functioning well in nonverbal areas. Markedly below-age-level verbal or sign language, accompanied by a low score on standardized expressive verbal tests, is diagnostic of expressive deficits in language disorder. Although expressive language deficits are frequently exhibited in children with autism spectrum disorders, these disturbances also occur frequently in the absence of autism spectrum disorder and are characterized by the following features: limited vocabulary, simple grammar, and variable articulation. “Inner language” or the appropriate use of toys and household objects is present. One assessment tool, the Carter Neurocognitive Assessment, itemizes and quantifies skills in areas of social awareness, visual attention, auditory comprehension, and vocal communication even when there are compromised expressive language and motor skills in very young children—up to 2 years of age. To confirm the diagnosis, a child is given standardized expressive language and nonverbal intelligence tests. Observations of children’s verbal and sign language patterns in various settings (e.g., school yard, classroom, home, and playroom) and during interactions with other children help ascertain the severity and specific areas of a child’s impairment and aid in early detection of behavioral and emotional complications. Family history should include the presence or absence of expressive language disorder among relatives. Clinical Features Children with expressive language deficits are vague when telling a story and use many filler words such as “stuff” and “things” instead of naming specific objects. The essential feature of expressive deficits in language disorder is marked impairment in the development of age-appropriate expressive language, which results in the use of verbal or sign language markedly below the expected level in view of a child’s nonverbal intellectual capacity. Language understanding (decoding) skills remain relatively intact. When severe, the disorder becomes recognizable by about the age of 18 months, when a child fails to utter spontaneously or even echo single words or sounds. Even simple words, such as “Mama” and “Dada,” are absent from the child’s active vocabulary, and the child points or uses gestures to indicate desires. The child seems to want to communicate, maintains eye contact, relates well to the mother, and enjoys games such as pat-a-cake and peek-a-boo. The child’s vocabulary is severely limited. At 18 months, the child may be limited to pointing to common objects when they are named. When a child with expressive language deficits begins to speak, the language impairment gradually becomes apparent. Articulation is often immature; numerous articulation errors occur but are inconsistent, particularly with such sounds as th, r, s, z, y, and l, which are either omitted or are substituted for other sounds. By the age of 4 years, most children with expressive language disturbance can speak in short phrases, but may have difficulty retaining new words. After beginning to speak, they acquire language more slowly than do most children. Their use of various grammatical structures is also markedly below the age-expected level, and their developmental milestones may be slightly delayed. Emotional problems involving poor self-image, frustration, and depression may develop in school-age children. Damien was a friendly, alert, and hyperactive 2-year-old, whose expressive vocabulary was limited to only two words (mama, daddy). He used these words one at a time in inappropriate situations. He supplemented his infrequent verbal communications with pointing and other simple gestures to request desired objects or actions. He was unable to communicate for other purposes (e.g., commenting or protesting). Damien appeared to be developing normally in other areas, especially in gross motor skills, although his fine motor skills were also poor. Damien sat, stood, and walked, and played happily with other children, enjoying activities and toys that were appropriate for 2-year-olds. Although he had a history of frequent ear infections, a recent hearing test revealed normal hearing. Despite his expressive limitations, Damien exhibited age-appropriate comprehension for the names of familiar objects and actions and for simple verbal instructions (e.g., “Put that down.” “Get your shirt.” “Clap your hands.”). However, due to his hyperactivity and impulsivity, he often required multiple directions to complete a simple task. Despite Damien’s slow start in language development, his pediatrician had reassured his parents that most of the time, toddlers like Damien spontaneously overcome their initial slow start in language development. Fortunately, Damien’s language delay spontaneously remitted by the time he entered preschool at 3½ years of age, although he was diagnosed at that time with attention-deficit/hyperactivity disorder. Jessica was a sociable, active 5-year-old, who was diagnosed with language disorder. She was well liked in kindergarten despite her language deficits and played with many of her classmates. During an activity in which each student recounted the story of Little Red Riding Hood to her doll, Jessica’s classmate’s story began: “Little Red Riding Hood was taking a basket of food to her grandmother who was sick. A bad wolf stopped Red Riding Hood in the forest. He tried to get the basket away from her but she wouldn’t give it to him.” When it was Jessica’s turn, she tried to avoid being picked, but when she could not avoid her turn, Jessica’s story sounded quite different: Jessica struggled and came up with: “Riding Hood going to grandma house. Her taking food. Bad wolf in a bed. Riding Hood say, what big ears, and grandma? Hear you, dear. What big eyes, grandma? See you, dear. What big mouth, grandma? Eat you all up!” Jessica’s story was characteristic of expressive language deficits at her age: including short, incomplete sentences; simple sentence structures; omission of grammatical function words (e.g., is and the) and inflectional endings (e.g., possessives and present tense verbs); problems in question formation; and incorrect use of pronouns (e.g., her for she). Jessica, however, performed as well as her classmates in understanding the details and plot of the Riding Hood tale, as long as she was not required to retell the story verbally. Jessica also demonstrated adequate comprehension skills in her kindergarten classroom, where she readily followed the teacher’s complex, multistep verbal instructions (e.g., “After you write your name in the top left corner of your paper, get your crayons and scissors, put your library books under your chair, and line up at the back of the room.”). Ramon was a quiet, sullen 8-year-old boy whose expressive language problems had improved over time and were no longer obvious in play with peers. His speech now rarely contained the incomplete sentences and grammatical errors that were so evident when he was younger. Ramon’s expressive problems, however, were still impairing him in tasks involving abstract use of language, and he was struggling in his third-grade academic work. An example was Ramon’s explanation of a recent science experiment: “The teacher had stuff in some jars. He poured it, and it got pink. The other thing made it white.” Although each sentence was grammatical, his explanation was difficult to follow, because key ideas and details were vaguely explained. Ramon also showed problems in word finding, and he relied on vague and nonspecific terms, such as thing, stuff, and got. In the first and second grades, Ramon had struggled to keep up with his classmates in reading, writing, and other academic skills. By third grade, however, the increasing demands for written work were beyond his abilities. Ramon’s written work was characterized by poor organization and lack of specificity. In addition, classmates began to tease him about his difficulties, and he was ashamed of his disability and reacted quite aggressively, often leading to physical fighting. Nonetheless, Ramon continued to show relatively good comprehension of spoken language, including classroom teaching concerning abstract concepts. He also comprehended sentences that were grammatically and conceptually complex (e.g., “The car the truck hit had hubcaps that were stolen. Had it been possible, she would have notified us by mail or by phone.”) Differential Diagnosis Language disorders are associated with various psychiatric disorders including other learning disorders and ADHD, and in some cases, the language disorder is difficult to separate from another dysfunction. In mixed receptive–expressive language disorder, language comprehension (decoding) is markedly below the expected age-appropriate level, whereas in expressive language disorder, language comprehension remains within normal limits. In autism spectrum disorders, children often have impaired language, symbolic and imagery play, appropriate use of gesture, or capacity to form typical social relationships. In contrast, children with expressive language disorder become very frustrated with their disorder, and are usually highly motivated to make friends despite their disability. Children with acquired aphasia or dysphasia have a history of early normal language development; the disordered language had its onset after a head trauma or other neurologic disorder (e.g., a seizure disorder). Children with selective mutism have normal language development. Often these children will speak only in front of family members (e.g., mother, father, and siblings). Children affected by selective mutism are socially anxious and withdrawn outside the family. Pathology and Laboratory Examination Children with speech and language disorders should have an audiogram to rule out hearing loss. Course and Prognosis The prognosis for expressive language disturbance worsens the longer it persists in a child; prognosis is also dependent on the severity of the disorder. Studies of infants and toddlers who are “late talkers” concur that 50 to 80 percent of these children master language skills that are within the expected level during the preschool years. Most children who are delayed in acquiring language catch up during preschool years. Outcome of expressive language deficits is influenced by other comorbid disorders. If children do not develop mood disorders or disruptive behavior problems, the prognosis is better. The rapidity and extent of recovery depends on the severity of the disorder, the child’s motivation to participate in speech and language therapy, and the timely initiation of therapeutic interventions. The presence or absence of hearing loss, or intellectual disability, impedes remediation and leads to a worse prognosis. Up to 50 percent of children with mild expressive language disorder recover spontaneously without any sign of language impairment, but those children with severe expressive speech disorder may persist in exhibiting some symptoms into middle childhood or later. Current literature shows that children who demonstrate poor comprehension, poor articulation, or poor academic performance tend to continue to have problems in these areas at follow-up 7 years later. An association is also seen between particular language impairment profiles and persistent mood and behavior problems. Children with poor comprehension associated with expressive difficulties seem to be more socially isolated and impaired with respect to peer relationships. Treatment The primary goals for early childhood speech and language treatment are to guide children and their parents toward greater production of meaningful language. There are more data to support improvements through speech and language interventions for expressive language deficit in young school-aged children with primary deficits than in preschool children. A recent study investigating Parent-Child Interaction Therapy (PCIT) for school-aged children with expressive language impairment found that PCIT was particularly efficacious in improving a child’s verbal initiation, mean length of utterances, and the proportion of child-to-parent utterances. A large-scale randomized trial of a yearlong intervention targeting preschoolers with language delay in Australia found that a community-based program did not affect language acquisition in 2- and 3year-olds. Given the high rate of spontaneous remission of language deficits in preschoolers, and less than robust effects of interventions for children that young, treatment for expressive language disorder is generally not initiated unless it persists after the preschool years. Various techniques have been used to help a child improve use of such parts of speech as pronouns, correct tenses, and question forms. Direct interventions use a speech and language pathologist who works directly with the child. Mediated interventions, in which a speech and language professional teaches a child’s teacher or parent how to promote therapeutic language techniques, have also been efficacious. Language therapy is often aimed at using words to improve communication strategies and social interactions as well. Such therapy consists of behaviorally reinforced exercises and practice with phonemes (sound units), vocabulary, and sentence construction. The goal is to increase the number of phrases by using blockbuilding methods and conventional speech therapies. MIXED RECEPTIVE AND EXPRESSIVE DEFICITS Children with both receptive and expressive language impairment may have impaired ability in sound discrimination, deficits in auditory processing, or poor memory for sound sequences. Children with mixed receptive–expressive disturbance exhibit impaired skills in the expression and reception (understanding and comprehension) of spoken language. The expressive difficulties in these children may be similar to those of children with only expressive language disturbance, which is characterized by limited vocabulary, use of simplistic sentences, and short sentence usage. Children with receptive language difficulties may be experiencing additional deficits in basic auditory processing skills, such as discriminating between sounds, rapid sound changes, association of sounds and symbols, and the memory of sound sequences. These deficits may lead to a whole host of communication barriers for a child, including a lack of understanding of questions or directives from others, or inability to follow the conversations of peers or family members. Recognition of mixed expressive–receptive language disturbance may be delayed because of early misattribution of their communication by teachers and parents as a behavioral problem rather than a deficit in understanding. The essential features of mixed receptive–expressive language disturbance are shown on scores on standardized tests; both receptive (comprehension) and expressive language development scores fall substantially below those obtained from standardized measures of nonverbal intellectual capacity. Language difficulties must be sufficiently severe to impair academic achievement or daily social communication. Epidemiology Mixed receptive-expressive language deficits occur less frequently than expressive deficits; however, epidemiologic data are scant regarding specific prevalence rates. Mixed receptive–expressive language disturbance is believed to occur in about 5 percent of preschoolers and to persist in approximately 3 percent of school-age children. It is known to be less common than expressive language disturbance. Mixed receptive– expressive language disorder is believed to be at least twice as prevalent in boys as in girls. Comorbidity Children with mixed receptive–expressive deficits are at high risk for additional speech and language disorders, learning disorders, and additional psychiatric disorders. About half of children with these deficits also have pronunciation difficulties leading to speech sound disorder, and about half also have reading disorder. These rates are significantly higher than the comorbidity found in children with only expressive language problems. ADHD is present in at least one third of children with mixed receptive–expressive language disturbances. Etiology Language disorders most likely have multiple determinants, including genetic factors, developmental brain abnormalities, environmental influences, neurodevelopmental immaturity, and auditory processing features in the brain. As with expressive language disturbance alone, evidence is found of familial aggregation of mixed receptive– expressive language deficits. Genetic contribution to this disorder is implicated by twin studies, but no mode of genetic transmission has been proved. Some studies of children with various speech and language disorders have also shown cognitive deficits, particularly slower processing of tasks involving naming objects, as well as fine motor tasks. Slower myelinization of neural pathways has been hypothesized to account for the slow processing found in children with developmental language disorders. Several studies suggest an underlying impairment of auditory discrimination, because most children with the disorder are more responsive to environmental sounds than to speech sounds. Diagnosis Children with mixed receptive–expressive language deficits develop language more slowly than their peers and have trouble understanding conversations that peers can follow. In mixed receptive–expressive language disorder, receptive dysfunction coexists with expressive dysfunction. Therefore, standardized tests for both receptive and expressive language abilities must be given to anyone suspected of having language disorder with mixed receptive–expressive disturbance. A markedly below-expected level of comprehension of verbal or sign language with intact age-appropriate nonverbal intellectual capacity, confirmation of language difficulties by standardized receptive language tests, and the absence of autism spectrum disorder, confirm the diagnosis of mixed receptive–expressive language deficits; however, in DSM-5, these deficits are included in the diagnosis of language disorder. Clinical Features The essential clinical feature of this language disturbance is significant impairment in both language comprehension and language expression. In the mixed type, expressive impairments are similar to those of expressive language disturbance, but can be more severe. The clinical features of the receptive component of the disorder typically appear before the age of 4 years. Severe forms are apparent by the age of 2 years; mild forms may not become evident until age 7 (second grade) or older, when language becomes complex. Children with language disorder characterized by mixed receptive–expressive disturbance show markedly delayed and below-normal ability to comprehend (decode) verbal or sign language, although they have age-appropriate nonverbal intellectual capacity. In most cases of receptive dysfunction, verbal or sign expression (encoding) of language is also impaired. The clinical features of mixed receptive–expressive language disturbance in children between the ages of 18 and 24 months result from a child’s failure to utter a single phoneme spontaneously or to mimic another person’s words. Many children with mixed receptive–expressive language deficits have auditory sensory difficulties and compromised ability to process visual symbols, such as explaining the meaning of a picture. They have deficits in integrating both auditory and visual symbols—for example, recognizing the basic common attributes of a toy truck and a toy passenger car. Whereas at 18 months, a child with expressive language deficits only comprehends simple commands and can point to familiar household objects when told to do so, a child of the same age with mixed receptive–expressive language disturbance typically cannot either point to common objects or obey simple commands. A child with mixed receptive–expressive language deficits may appears to be deaf. He or she responds normally to sounds from the environment, but not to spoken language. If the child later starts to speak, the speech contains numerous articulation errors, such as omissions, distortions, and substitutions of phonemes. Language acquisition is much slower for children with mixed receptive–expressive language disturbance than for other children of the same age. Children with mixed receptive–expressive language disturbance have difficulty recalling early visual and auditory memories and recognizing and reproducing symbols in proper sequence. Some children with mixed receptive–expressive language deficits have a partial hearing defect for true tones, an increased threshold of auditory arousal, and an inability to localize sound sources. Seizure disorders and reading disorder are more common among the relatives of children with mixed receptive–expressive problems than in the general population. Pathology and Laboratory Examination An audiogram is indicated for all children thought to have mixed receptive–expressive language disturbance to rule out or confirm the presence of deafness or auditory deficits. A history of the child and family and observation of the child in various settings help to clarify the diagnosis. Jenna was a pleasant 2-year-old, who did not yet use any spoken words, and did not respond to simple commands without gestures. She made her needs known with vocalizations and simple gestures (e.g., showing or pointing) such as those typically used by younger children. She seemed to understand the names for only a few familiar people and objects (e.g., mommy, daddy, cat, bottle, and cookie). Compared with other children her age, she had a small comprehension vocabulary and showed limited understanding of simple verbal directions (e.g., “Get your doll.” “Close your eyes.”). Nonetheless, her hearing was normal, and her motor and play skills were developing as expected for her age. She showed interest in her environment and in the activities of the other children at her day care. Lena was a shy, reserved 5-year-old who grew up in a bilingual home. Lena’s parents and older siblings spoke English and Cantonese proficiently. Her grandparents, who lived in the same home, spoke only Cantonese. Lena began to understand and speak both languages much later than her older siblings had. Throughout her preschool years, Lena continued to develop slowly in comprehension and production. At the start of kindergarten, Lena understood fewer English words for objects, actions, and relations than her classmates did. Lena was unable to follow complex classroom instructions, particularly those that involved words for concepts of time (e.g., tomorrow, before, or day) and space (e.g., behind, next to, or under). It was also hard for Lena to match one of several pictures to a syntactically complex sentence that she had heard (e.g., “It was not the train she was waiting for.” “Because he had already completed his work, he was not kept after school.”). Lena played with other children but only rarely tried to speak with them, which led to her being ostracized by her classmates. Lena’s attempts at conversations usually broke down, because she misinterpreted what others said or could not express her own thoughts clearly. Consequently, her classmates generally ignored her, preferring instead to play with more verbally competent peers. Lena’s infrequent interactions further limited her opportunities to learn and to practice her already weak language skills. Lena also showed limited receptive and expressive skills in Cantonese, as revealed by an assessment conducted with the assistance of a Cantonese interpreter. Nonetheless, her nonverbal cognitive and motor skills were within the normal range for her age. Lena was quite proficient in solving spatial and numerical, problems, provided they were presented on paper and were not word problems. Mark received a diagnosis of Language Disorder, based on mixed receptive– expressive deficits when he was a preschooler. By 7 years of age, he had also received the comorbid diagnoses of reading disorder and ADHD. This combination of language, reading, and attention problems made it virtually impossible for Mark to succeed in school, although he was able to engage his peers during free play. His comprehension and attention difficulties limited his ability to understand and to learn important information, or to follow classroom instructions or discussions. Mark fell further and further behind his classmates. He was also disadvantaged because he could read only a few familiar words. This meant that he was neither motivated nor able to learner academic information outside of the classroom by reading. Mark received tutoring and speech and language interventions, and despite some improvements, he continued to lag behind his classmates academically. Despite his academic problems, however, Mark made friends during sports activities in which he excelled, and continued to show nonverbal intellectual skills within the average range. Differential Diagnosis Children with language disorder characterized by mixed receptive–expressive deficits have a deficit in language comprehension as well as in language production. The receptive deficit may be overlooked at first, because the expressive language deficit may be more obvious. In expressive language disturbance alone, comprehension of spoken language (decoding) remains within age norms. Children with speech sound disorder and child-onset fluency disorder (stuttering) have normal expressive and receptive language competence, despite the speech impairments. Most children with mixed receptive–expressive language disturbance have a history of variable and inconsistent responses to sounds; they respond more often to environmental sounds than to speech sounds (Table 31.4a-2). Intellectual disability, selective mutism, acquired aphasia, and autism spectrum disorder should also be ruled out. Table 31.4a-2 Differential Diagnosis of Language Disorder Course and Prognosis The overall prognosis for language disorder with mixed receptive–expressive disturbance is less favorable than that for expressive language disturbance alone. When the mixed disorder is identified in a young child, it is usually severe, and the short-term prognosis is poor. Language develops at a rapid rate in early childhood, and young children with the disorder may appear to be falling behind. In view of the likelihood of comorbid learning disorders and other mental disorders, the prognosis is guarded. Young children with severe mixed receptive-expressive language deficits are likely to have learning disorders in the future. In children with mild versions, mixed disorder may not be identified for several years, and the disruption in everyday life may be less overwhelming than that in severe forms of the disorder. Over the long run, some children with mixed receptive–expressive language disturbance achieve close to normal language functions. The prognosis for children who have mixed receptive–expressive language disturbances varies widely and depends on the nature and severity of the damage. Treatment A comprehensive speech and language evaluation is recommended for children with mixed receptive–expressive language disturbance, given the complexities of having both deficits. Some controversy exists as to whether remediation of receptive deficits before expressive language provides more efficacy overall. A review of the literature indicates that it is not more beneficial to address receptive deficits before expressive, and in fact, in some cases, remediation of expressive language may reduce or eliminate the need for receptive language remediation. Thus, current recommendations are either to address 06 - 31.4b Speech Sound Disorder 31.4b Speech Sound Disorder both simultaneously, or to provide interventions for the expressive component first, and then address the receptive language. Preschoolers with mixed receptive–expressive language problems optimally receive interventions designed to promote social communication and literacy as well as oral language. For children at the kindergarten level, optimal intervention includes direct teaching of key pre-reading skills as well as social skills training. An important early goal of interventions for young children with mixed receptive–expressive language disturbance is the achievement of rudimentary reading skills, in that these skills are protective against the academic and psychosocial ramifications of falling behind early on in reading. Some language therapists favor a low-stimuli setting, in which children are given individual linguistic instruction. Others recommend that speech and language instruction be integrated into a varied setting with several children who are taught several language structures simultaneously. Often, a child with receptive and expressive language deficits will benefit from a small, specialeducational setting that allows more individualized learning. Psychotherapy may be helpful for children with mixed language disorder who have associated emotional and behavioral problems. Particular attention should be paid to evaluating the child’s self-image and social skills. Family counseling in which parents and children can develop more effective, less frustrating means of communicating may be beneficial. 31.4b Speech Sound Disorder Children with speech sound disorder have difficulty pronouncing speech sounds correctly due to omissions of sounds, distortions of sounds, or atypical pronunciation. Formerly called phonological disorder, typical speech disturbances in speech sound disorder include omitting the last sounds of the word (e.g., saying mou for mouse or drin for drink), or substituting one sound for another (saying bwu instead of blue or tup for cup). Distortions in sounds can occur when children allow too much air to escape from the side of their mouths while saying sounds like sh or producing sounds like s or z with their tongue protruded. Speech sound errors can also occur in patterns because a child has an interrupted airflow instead of a steady airflow preventing their words to be pronounced (e.g., pat for pass or bacuum for vacuum). Children with a speech sound disorder can be mistaken for younger children because of their difficulties in producing speech sounds correctly. The diagnosis of a speech sound disorder is made by comparing the skills of a given child with the expected skill level of others of the same age. The disorder results in errors in whole words because of incorrect pronunciation of consonants, substitution of one sound for another, omission of entire phonemes, and, in some cases, dysarthria (slurred speech because of incoordination of speech muscles) or dyspraxia (difficulty planning and executing speech). Speech sound development is believed to be based on both linguistic and motor development that must be integrated to produce sounds. Speech sound disturbances such as dysarthria and dyspraxia are not diagnosed as speech sound disorder if they are known to have a neurological basis, according to DSM5. Thus, speech sound abnormalities accounted for by cerebral palsy, cleft palate, deafness or hearing loss, traumatic brain injury, or neurological conditions are not diagnosed as speech sound disorder. Articulation difficulties not associated with a neurological condition are the most common components of speech sound disorder in children. Articulation deficits are characterized by poor articulation, sound substitution, and speech sound omission, and give the impression of “baby talk.” Typically, these deficits are not caused by anatomical, structural, physiological, auditory, or neurological abnormalities. They vary from mild to severe and result in speech that ranges from completely intelligible to unintelligible. EPIDEMIOLOGY Epidemiologic studies suggest that the prevalence of speech sound disorder is at least 3 percent in preschoolers, 2 percent in children 6 to 7 years of age, and 0.5 percent in 17year-old adolescents. Approximately 7 to 8 percent of 5-year-old children in one large community sample had speech sound production problems of developmental, structural, or neurological origins. Another study found that up to 7.5 percent of children between the ages of 7 and 11 years had speech sound disorders. Of those, 2.5 percent had speech delay (deletion and substitution errors past the age of 4 years) and 5 percent had residual articulation errors beyond the age of 8 years. Speech sound disorders occur much more frequently than disorders with known structural or neurological origin. Speech sound disorder is approximately two to three times more common in boys than in girls. It is also more common among first-degree relatives of patients with the disorder than in the general population. Although speech sound mistakes are quite common in children younger than 3 years of age, these mistakes are usually selfcorrected by age 7 years. Misarticulating after the age of 7 years is likely to represent a speech sound disorder. The prevalence of speech sound disorders reportedly falls to 0.5 percent by mid to late adolescence. COMORBIDITY More than half of children with speech sound disorder have some difficulty with language. Disorders most commonly present with speech sound disorders are language disorder, reading disorder, and developmental coordination disorder. Enuresis may also accompany the disorder. A delay in reaching speech milestones (e.g., first word and first sentence) has been reported in some children with speech sound, but most children with the disorder begin speaking at the appropriate age. Children with both speech sound and language disorders are at greatest risk for attentional problems and specific learning disorders. Children with speech sound disorder in the absence of language disorder have lower risk of comorbid psychiatric disorders and behavioral problems. ETIOLOGY Contributing factors leading to speech disturbance may include perinatal problems, genetic factors, and auditory processing problems. Given the high rates of spontaneous remission in very young children, a maturational delay in the developmental brain process underlying speech has been postulated in some cases. The likelihood of neuronal cause is supported by the observation that children with speech sound disorder are also more likely to manifest “soft neurological signs” as well as language disorder and a higher-than-expected rate of reading disorder. Genetic factors are implicated by data from twin studies that show concordance rates for monozygotic twins that are higher than chance. Articulation disorders caused by structural or mechanical problems are rare. Articulation problems that are not diagnosed as speech sound disorder may be caused by neurological impairment and can be divided into dysarthria and apraxia or dyspraxia. Dysarthria results from an impairment in the neural mechanisms regulating the muscular control of speech. This can occur in congenital conditions, such as cerebral palsy, muscular dystrophy, or head injury, or because of infectious processes. Apraxia or dyspraxia is characterized by difficulty in the execution of speech, even when no obvious paralysis or weakness of the muscles used in speech exists. Environmental factors may play a role in speech sound disorder, but constitutional factors seem to make the most significant contribution. The high proportion of speech sound disorder in certain families implies a genetic component in the development of this disorder. Developmental coordination disorder and coordination in the mouth such as in chewing and blowing the nose may be associated. DIAGNOSIS The essential feature of speech sound disorder is a child’s delay or failure to produce developmentally expected speech sounds, especially consonants, resulting in sound omissions, substitutions, and distortions of phonemes. A rough guideline for clinical assessment of children’s articulation is that normal 3-year-olds correctly articulate m, n, ng, b, p, h, t, k, q, and d; normal 4-year-olds correctly articulate f, y, ch, sh, and z; and normal 5-year-olds correctly articulate th, s, and r. Speech sound disorder cannot be accounted for by structural or neurological abnormalities, and typically, it is accompanied by normal language development. CLINICAL FEATURES Children with speech sound disorder are delayed in, or incapable of, producing accurate speech sounds that are expected for their age, intelligence, and dialect. The sounds are often substitutions—for example, the use of t instead of k—and omissions, such as leaving off the final consonants of words. Speech sound disorder can be recognized in early childhood. In severe cases, the disorder is first recognized at between 2 and 3 years of age. In less severe cases, the disorder may not be apparent until the age of 6 years. A child’s articulation is judged disordered when it is significantly behind that of most children at the same age level, intellectual level, and educational level. In very mild cases, a single speech sound (i.e., phoneme) may be affected. When a single phoneme is affected, it is usually one that is acquired late in normal language acquisition. The speech sounds most frequently misarticulated are also those acquired late in the developmental sequence, including r, sh, th, f, z, l, and ch. In severe cases and in young children, sounds such as b, m, t, d, n, and h may be mispronounced. One or many speech sounds may be affected, but vowel sounds are not among them. Children with speech sound disorder cannot articulate certain phonemes correctly and may distort, substitute, or even omit the affected phonemes. With omissions, the phonemes are absent entirely—for example, bu for blue, ca for car, or whaa? For what’s that? With substitutions, difficult phonemes are replaced with incorrect ones—for example, wabbit for rabbit, fum for thumb, or whath dat? For what’s that? With distortions, the correct phoneme is approximated but is articulated incorrectly. Rarely, additions (usually of the vowel uh) occur—for example, puhretty for pretty, what’s uh that uh? For what’s that? Omissions are thought to be the most serious type of misarticulating, with substitutions the next most serious, and distortions the least serious type. Omissions, which are most frequent in the speech of young children, usually occur at the ends of words or in clusters of consonants (ka for car, scisso for scissors). Distortions, which are found mainly in the speech of older children, result in a sound that is not part of the speaker’s dialect. Distortions may be the last type of misarticulating remaining in the speech of children whose articulation problems have mostly remitted. The most common types of distortions are the lateral slip—in which a child pronounces s sounds with the airstream going across the tongue, producing a whistling effect—and the palatal or lisp —in which the s sound, formed with the tongue too close to the palate, produces a ssh sound effect. The misarticulating of children with speech sound disorder is often inconsistent and random. A phoneme may be pronounced correctly one time and incorrectly another time. Misarticulating is most common at the ends of words, in long and syntactically complex sentences, and during rapid speech. Omissions, distortions, and substitutions also occur normally in the speech of young children learning to talk. But, whereas young, normally speaking children soon replace their misarticulating, children with speech sound disorder do not. Even as children with articulation problems grow and finally acquire the correct phoneme, they may use it only in newly acquired words and may not correct the words learned earlier that they have been mispronouncing for some time. Most children eventually outgrow speech sound disorder, usually by the third grade. After the fourth grade, however, spontaneous recovery is unlikely, and so it is important to try to remediate the disorder before the development of complications. Often, beginning kindergarten or school precipitates the improvement when recovery from speech sound disorder is spontaneous. Speech therapy is clearly indicated for children who have not shown spontaneous improvement by the third or fourth grade. Speech therapy should be initiated at an early age for children whose articulation is significantly unintelligible and who are clearly troubled by their inability to speak clearly. Children with speech sound disorder may have various concomitant social, emotional, and behavioral problems, particularly when comorbid expressive language problems are present. Children with chronic expressive language deficits and severe articulation impairment are the ones most likely to suffer from psychiatric problems. Martin was a talkative, likeable 3-year-old with virtually unintelligible speech, despite excellent receptive language skills and normal hearing. Martin’s level of expressive language development was difficult to quantify due to his very poor pronunciation. The rhythm and melody of his speech, however, suggested that he was trying to produce multiword utterances, as would be expected at his age. Martin produced only a few vowels (/ee/, /ah/, and /oo/), some early developing consonants (/m/, /n/, /d/, /t/, /p/, /b/, /h/, and /w/), and limited syllables. This reduced sound repertoire made many of his spoken words indistinguishable from one another (e.g., he said bahbah for bottle, baby, and bubble, and he used nee for knee, need, and Anita [his sister]). Moreover, he consistently omitted consonant sounds at the end of words and in consonant cluster sequences (e.g., /tr-/, /st-/, /-nt/, and /-mp/). Understandably, on occasion Martin reacted with frustration and tantrums to his difficulties in making his needs understood. Brad was a pleasant, cooperative 5-year-old, who was recognized as early as preschool to have articulation problems, and these persisted into kindergarten. His language comprehension skills, and hearing were within normal limits. He showed some mild expressive language problems, however, in the use of certain grammatical features (e.g., pronouns, auxiliary verbs, and past-tense word endings) and in the formulation of complex sentences. He correctly produced all vowel sounds and most of the early developing consonants, but he was inconsistent in his attempts to produce later-developing consonants (e.g., /r/, /l/, /s/, /z/, /sh/, /th/, and /ch/). Sometimes, he omitted them; sometimes, he substituted other sounds for them (e.g., /w/ for /r/ or /f/ for /th/); occasionally, he even produced them correctly. Brad had particular problems in correctly producing consonant cluster sequences and multisyllabic words. Cluster sequences had omitted or incorrect sounds (e.g., blue might be produced as bue or bwue, and hearts might be said as hots or hars). Multisyllabic words had syllables omitted (e.g., efant for elephant and getti for spaghetti) and sounds mispronounced or even transposed (e.g., aminal for animal and lemon for melon). Strangers were unable to understand approximately 80 percent of Brad’s speech. Brad often spoke more slowly and clearly than usual, however, when he was asked to repeat something, as he often was. Jane was a hyperactive 8-year-old, with a history of significant speech delay. During her preschool and early school years, she had overcome many of her earlier speech errors. A few late-developing sounds (/r/, /l/, and /th/), however, continued to pose a challenge for her. Jane often substituted /f/ or /d/ for /th/ and produced /w/ for /r/ and /l/. Overall, her speech was easily understood, despite these minor errors. Nonetheless, she became somewhat aggressive with her peers because of the teasing she received from her classmates about her speech. DIFFERENTIAL DIAGNOSIS The differential diagnosis of speech sound disorder includes a careful determination of symptoms, severity, and possible medical conditions that might be producing the symptoms. First, the clinician must determine that the misarticulating is sufficiently severe to be considered impairing, rather than a normative developmental process of learning to speak. Second, the clinician must determine that no physical abnormalities account for the articulation errors and must rule out neurological disorders that may cause dysarthria, hearing impairment, mental retardation, and pervasive developmental disorders. Third, the clinician must obtain an evaluation of receptive and expressive language to determine that the speech difficulty is not solely attributable to the above mentioned disorders. Neurological, oral structural, and audiometric examinations may be necessary to rule out physical factors that cause certain types of articulation abnormalities. Children with dysarthria, a disorder caused by structural or neurological abnormalities, differ from children with speech sound disorder in that dysarthria is less likely to remit spontaneously and may be more difficult to remediate. Drooling, slow, or uncoordinated motor behavior; abnormal chewing or swallowing; and awkward or slow protrusion and retraction of the tongue indicate dysarthria. A slow rate of speech also indicates dysarthria (Table 31.4b-1). Table 31.4b-1 Differential Diagnosis of Speech Sound Disorder COURSE AND PROGNOSIS Spontaneous remission of symptoms is common in children whose misarticulating involves only a few phonemes. Children who persist in exhibiting articulation problems after the age of 5 years may be experiencing a myriad of other speech and language impairments, so that a comprehensive evaluation may be indicated at that time. Children older than age 5 with articulation problems are at higher risk for auditory perceptual problems. Spontaneous recovery is rare after the age of 8 years. Some debate exists regarding the relationship between articulation problems and reading disorder, or dyslexia. A recent study comparing children with phonological problems only, with children who had dyslexia only, and those with both phonological difficulties and dyslexia concluded that children with both disorders have somewhat distinct profiles and are comorbid disorders rather than one mixed disorder. TREATMENT Two main approaches have been used successfully to improve speech sound difficulties. The first one, the phonological approach, is usually chosen for children with extensive patterns of multiple speech sound errors that may include final consonant deletion, or consonant cluster reduction. Exercises in this approach to treatment focus on guided practice of specific sounds, such as final consonants, and when that skill is mastered, practice is extended to use in meaningful words and sentences. The other approach, the traditional approach is utilized for children who produce substitution or distortion errors in just a few sounds. In this approach, the child practices the production of the problem sound while the clinician provides immediate feedback and cues concerning the correct placement of the tongue and mouth for improved articulation. Children who have errors in articulation because of abnormal swallowing resulting in tongue thrust and lisps are treated with exercises that improve swallowing patterns and, in turn, improve speech. Speech therapy is typically provided by a speech-language pathologist, yet parents can be taught to provide adjunctive help by practicing techniques used in the treatment. Early intervention can be helpful, because for many children with mild articulation difficulties, even several months of intervention may be helpful in early elementary school. In general, when a child’s articulation and intelligibility is noticeably different than peers by 8 years of age, speech deficits often lead to problems with peers, learning, and self-image, especially when the disorder is so severe that many consonants are misarticulated, and when errors involve omissions and substitutions of phonemes, rather than distortions. Children with persistent articulation problems are likely to be teased or ostracized by peers and may become isolated and demoralized. Therefore, it is important to give support to children with phonological disorders and, whenever possible, to support prosocial activities and social interactions with peers. Parental counseling and monitoring of child–peer relationships and school behavior can help minimize social impairment in children with speech sound and language disorder. 07 - 31.4c Child Onset Fluency Disorder (Stutterin 31.4c Child-Onset Fluency Disorder (Stuttering) 31.4c Child-Onset Fluency Disorder (Stuttering) Child-onset fluency disorder (stuttering) usually begins during the first years of life and is characterized by disruptions in the normal flow of speech by involuntary speech motor events. Stuttering can include a variety of specific disruptions of fluency, including sound or syllable repetitions, sound prolongations, dysrhythmic phonations, and complete blocking or unusual pauses between sounds and syllables of words. In severe cases, the stuttering may be accompanied by accessory or secondary attempts to compensate such as respiratory, abnormal voice phonations, or tongue clicks. Associated behaviors, such as eye blinks, facial grimacing, head jerks, and abnormal body movements, may be observed before or during the disrupted speech. Early intervention is important because children who receive early intervention have been found to be more than 7 times more likely to have full resolution of their stuttering. In severe and some untreated cases, stuttering can become an entrenched pattern that is more challenging to remediate later in life and is associated with significant psychological and social distress. When stuttering becomes chronic, persisting into adulthood, the rates of concurrent social anxiety disorder are reported to be between 40 and 60 percent. EPIDEMIOLOGY An epidemiologic survey of 3- to 17-year-olds derived from the United States National Health Interview Surveys reports that the prevalence of stuttering is approximately 1.6 percent. Stuttering tends to be most common in young children and has often resolved spontaneously by the time the child is older. The typical age of onset is 2 to 7 years of age, with 90 percent of children exhibiting symptoms by age 7 years. Approximately 65 to 80 percent of young children who stutter are likely to have a spontaneous remission over time. According to the DSM-5, the rate dips to 0.8 percent by adolescence. Stuttering affects about three to four males for every one female. The disorder is significantly more common among family members of affected children than in the general population. Reports suggest that for male persons who stutter, 20 percent of their male children and 10 percent of their female children will also stutter. COMORBIDITY Very young children who stutter typically show some delay in the development of language and articulation without additional disorders of speech and language. Preschoolers and school-age children who stutter exhibit an increased incidence of social anxiety, school refusal, and other anxiety symptoms. Older children who stutter also do not necessarily have comorbid speech and language disorders, but often manifest anxiety symptoms and disorders. When stuttering persists into adolescence, social isolation occurs at higher rates than in the general adolescent population. Stuttering is also associated with a variety of abnormal motor movements, upper body tics, and facial grimaces. Other disorders that coexist with stuttering include phonological disorder, expressive language disorder, mixed receptive–expressive language disorder, and ADHD. ETIOLOGY Converging evidence indicates that cause of stuttering is multifactorial, including genetic, neurophysiological, and psychological factors that predispose a child to have poor speech fluency. Although research evidence does not indicate that anxiety or conflicts cause stuttering or that persons who stutter have more psychiatric disturbances than those with other forms of speech and language disorders, stuttering can be exacerbated by certain stressful situations. Other theories about the cause of stuttering include organic models and learning models. Organic models include those that focus on incomplete lateralization or abnormal cerebral dominance. Several studies using EEG found that stuttering males had right hemispheric alpha suppression across stimulus words and tasks; nonstutterers had left hemispheric suppression. Some studies of stutterers have noted an overrepresentation of left-handedness and ambidexterity. Twin studies and striking gender differences in stuttering indicate that stuttering has some genetic basis. Learning theories about the cause of stuttering include the semantogenic theory, in which stuttering is basically a learned response to normative early childhood disfluencies. Another learning model focuses on classic conditioning, in which the stuttering becomes conditioned to environmental factors. In the cybernetic model, speech is viewed as a process that depends on appropriate feedback for regulation; stuttering is hypothesized to occur because of a breakdown in the feedback loop. The observations that stuttering is reduced by white noise and that delayed auditory feedback produces stuttering in normal speakers lend support to the feedback theory. The motor functioning of some children who stutter appears to be delayed or slightly abnormal. The observation of difficulties in speech planning exhibited by some children who stutter suggests that higher-level cognitive dysfunction may contribute to stuttering. Although children who stutter do not routinely exhibit other speech and language disorders, family members of these children often exhibit an increased incidence of a variety of speech and language disorders. Stuttering is most likely to be caused by a set of interacting variables that include both genetic and environmental factors. DIAGNOSIS The diagnosis of childhood-onset fluency disorder (stuttering) is not difficult when the clinical features are apparent and well developed and each of the following four phases (described in the next section) are readily recognized. Diagnostic difficulties can arise when evaluating for stuttering in young children, because some preschool children experience transient dysfluency. It may not be clear whether the nonfluent pattern is part of normal speech and language development or whether it represents the initial stage in the development of stuttering. If incipient stuttering is suspected, referral to a speech pathologist is indicated. CLINICAL FEATURES Stuttering usually appears between the ages of 18 months and 9 years, with two sharp peaks of onset between the ages of 2 to 3.5 years and 5 to 7 years. Some, but not all, stutterers have other speech and language problems, such as phonological disorder and expressive language disorder. Stuttering does not begin suddenly; it typically develops over weeks or months with a repetition of initial consonants, whole words that are usually the first words of a phrase, or long words. As the disorder progresses, the repetitions become more frequent, with consistent stuttering on the most important words or phrases. Even after it develops, stuttering may be absent during oral readings, singing, and talking to pets or inanimate objects. Four gradually evolving phases in the development of stuttering have been identified: Phase 1 occurs during the preschool period. Initially, the difficulty tends to be episodic and appears for weeks or months between long interludes of normal speech. A high percentage of recovery from these periods of stuttering occurs. During this phase, children stutter most often when excited or upset, when they seem to have a great deal to say, and under other conditions of communicative pressure. Phase 2 usually occurs in the elementary school years. The disorder is chronic, with few if any intervals of normal speech. Affected children become aware of their speech difficulties and regard themselves as stutterers. In phase 2, the stuttering occurs mainly with the major parts of speech—nouns, verbs, adjectives, and adverbs. Phase 3 usually appears after the age of 8 years and up to adulthood, most often in late childhood and early adolescence. During phase 3, stuttering comes and goes largely in response to specific situations, such as reciting in class, speaking to strangers, making purchases in stores, and using the telephone. Some words and sounds are regarded as more difficult than others. Phase 4 typically appears in late adolescence and adulthood. Stutterers show a vivid, fearful anticipation of stuttering. They fear words, sounds, and situations. Word substitutions and circumlocutions are common. Stutterers avoid situations requiring speech and show other evidence of fear and embarrassment. Stutterers may have associated clinical features: vivid, fearful anticipation of stuttering, with avoidance of particular words, sounds, or situations in which stuttering is anticipated; and eye blinks, tics, and tremors of the lips or jaw. Frustration, anxiety, and depression are common among those with chronic stuttering. DIFFERENTIAL DIAGNOSIS Normal speech dysfluency in preschool years is difficult to differentiate from incipient stuttering. In stuttering occurs more nonfluencies, part-word repetitions, sound prolongations, and disruptions in voice airflow through the vocal track. Children who stutter appear to be tense and uncomfortable with their speech pattern, in contrast to young children who are nonfluent in their speech but seem to be at ease. Spastic dysphonia is a stuttering-like speech disorder distinguished from stuttering by the presence of an abnormal breathing pattern. Cluttering is a speech disorder characterized by erratic and dysrhythmic speech patterns of rapid and jerky spurts of words and phrases. In cluttering, those affected are usually unaware of the disturbance, whereas, after the initial phase of the disorder, stutterers are aware of their speech difficulties. Cluttering is often an associated feature of expressive language disturbance. COURSE AND PROGNOSIS The course of stuttering is often long term, with periods of partial remission lasting for weeks or months and exacerbations occurring most frequently when a child is under pressure to communicate. In children with mild cases, 50 to 80 percent recover spontaneously. School-age children who stutter chronically may have impaired peer relationships as a result of teasing and social rejection. These children may face academic difficulties, especially if they persistently avoid speaking in class. Stuttering is associated with anxiety disorders in chronic cases, and approximately half of individuals with persistent stuttering have social anxiety disorder. TREATMENT Evidence-based treatments for stuttering are emerging in the literature. One such treatment is the Lidcombe Program, which is based on an operant conditioning model in which parents use praise for periods of time in which the child does not stutter, and intervene when the child does stutter to request the child to self-correct the stuttered word. This treatment program is largely administered at home by parents, under the supervision of a speech and language therapist. A second treatment program being investigated in clinical trials is a family-based, parent-child interaction therapy that identifies stressors possibly associated with increased stuttering and aims to diminish these stressors. A third treatment currently under investigation in clinical trials is based on the knowledge that speaking each syllable in time to a particular rhythm has led to diminished stuttering in adults. This treatment program appears to be promising when administered early on, to preschoolers. Distinct forms of interventions have historically been used in the treatment of stuttering. The first approach, direct speech therapy, targets modification of the stuttering response to fluent-sounding speech by systematic steps and rules of speech mechanics that the person can practice. The other form of therapy for stuttering targets diminishing tension and anxiety during speech. These treatments may utilize breathing exercises and relaxation techniques, to help children slow the rate of speaking and modulate speech volume. Relaxation techniques are based on the premise that it is nearly impossible to be relaxed and stutter in the usual manner at the same time. Current interventions for stuttering use individualized combinations of behavioral 08 - 31.4d Social (Pragmatic) Communication Disord 31.4d Social (Pragmatic) Communication Disorder distraction, relaxation techniques, and directed speech modification. Stutterers who have poor self-image, comorbid anxiety disorders or depressive disorders are likely to require additional treatments with cognitive-behavioral therapy (CBT) and/or pharmacologic agents such as one of the selective serotonin reuptake inhibitor (SSRI) antidepressants. An approach to stuttering proposed by the Speech Foundation of America is labeled self-therapy, based on the premise that stuttering is not a symptom, but a behavior that can be modified. Stutterers are told that they can learn to control their difficulty partly by modifying their feelings about stuttering and attitudes toward it and partly by modifying the deviant behaviors associated with their stuttering blocks. The approach includes desensitizing; reducing the emotional reaction to, and fears of, stuttering; and substituting positive action to control the moment of stuttering. 31.4d Social (Pragmatic) Communication Disorder Social (pragmatic) communication disorder is a newly added diagnosis to DSM-5 characterized by persistent deficits in using verbal and nonverbal communication for social purposes in the absence of restricted and repetitive interests and behaviors. Deficits may be exhibited by difficulty in understanding and following social rules of language, gesture, and social context. This may limit a child’s ability to communicate effectively with peers, in academic settings, and in family activities. To successfully achieve social and pragmatic communication, a child or adolescent would be expected to integrate gestures, language, and social context of a given interaction to correctly infer its meaning. Thus, the child or adolescent would be able to understand another speaker’s “intention” of the communication with verbal and nonverbal cues as well as through an understanding of the environmental and social context of the interaction. One of the reasons that social (pragmatic) communication disorder was introduced into the DSM-5 was to include those children with social communication impairment who do not exhibit restrictive and repetitive interests and behaviors, and therefore do not fulfill the criteria for autism spectrum disorders. Pragmatic communication encompasses the ability to infer meaning in a given communication by not only understanding the words used, but also integrating the phrases into their prior understanding of the social environment. Social (pragmatic) communication disorder is a new disorder; however, the concept of children with social communication deficits without repetitive and restrictive interests and behaviors has been identified for many years, and is often associated with delayed language acquisition and language disorder. EPIDEMIOLOGY It is difficult to estimate the prevalence of social (pragmatic) communication disorder. Nevertheless, a body of literature has documented a profile of children who present with these persistent difficulties in pragmatic language, who do not meet criteria for autism spectrum disorder. COMORBIDITY Social (pragmatic) communication disorder is commonly associated with language disorder, consisting of diminished vocabulary for expected age, deficits in receptive skills, as well as impaired ability to use expressive language. Attentiondeficit/hyperactivity disorder (ADHD) is often concurrent with social (pragmatic) communication disorder. Specific learning disorders with impairments in reading and writing are also commonly comorbid disorders with social (pragmatic) communication disorder. Although some symptoms of social anxiety disorder may overlap with social (pragmatic) communication disorder, the full disorder of social anxiety disorder may emerge comorbidly with social (pragmatic) communication disorder. ETIOLOGY A family history of communication disorders, autism spectrum disorder, or specific learning disorder all appear to increase the risk for social (pragmatic) communication disorder. This suggests that genetic influences are contributing factors in the development of this disorder. The etiology of social (pragmatic) communication disorder, however, is likely to be multifactorial, and given its frequent comorbidity with both language disorder and ADHD, developmental and environmental influences are likely to also play a role. DIAGNOSIS The diagnosis of social (pragmatic) communication disorder can be difficult to distinguish from mild variants of autism spectrum disorder in which repetitive and restricted interests and behaviors are minimal. There have been largely discrepant data regarding how many children previously diagnosed with autism would be excluded from the DSM-5 criteria, which now focus on only two symptom domains: social communication deficits and restricted repetitive interests and behaviors. In one study, only 60.6 percent of children who had previously met the criteria for autistic spectrum disorder in the previous edition of the DSM met DSM-5 criteria for autistic spectrum disorder. However, in another study, up to 91 percent patients with of autism continued to meet the same DSM-5 criteria. The essential features of social (pragmatic) communication disorder are persistently impaired social pragmatic communication resulting in limited effective communication, compromised social relationships, and difficulties with academic or occupational achievement. CLINICAL FEATURES Social (pragmatic) communication disorder is characterized by impaired ability to effectively use verbal and nonverbal communication for social purposes and occurs in the absence of restricted and repetitive interests and behaviors. According to the DSM-5, all of the following features must be present in order to meet diagnostic criteria: (1) Deficits in using appropriate communication such as greeting, or sharing information in a social situation or context. (2) Impaired ability to modulate the tone, level, or vocabulary used in social communication to match the listener and the situation, such as inability to simplify communication when speaking to a young child. (3) Impaired ability in following the rules for conversations such as taking turns or rephrasing a statement for clarification and failure to recognize and respond socially appropriately to verbal and nonverbal feedback. (4) Difficulty understanding things that are not explicitly stated, impaired ability to make inferences, understand humor, or interpret socially ambiguous stimuli. Although the preceding deficits begin in the early developmental period, the diagnosis is rarely made in a child younger than 4 years of age. In milder cases, the difficulties may not become apparent until adolescence when the demands for language and social understanding are increased. The deficits in social communication lead to impairment in function in social situations, in developing relationships, and in family and academic settings. DIFFERENTIAL DIAGNOSIS The primary diagnostic consideration in social (pragmatic) communication disorder is autism spectrum disorder. The two disorders are most easily distinguished when the prominence of restricted and repetitive interests and behaviors characteristic of autistic spectrum disorder is present. However, in many cases of autism, the restrictive interests and repetitive behaviors manifest more prominently in the early developmental period and are not obvious in older childhood. However, even when these features are not observable, if they are obtained by history, social (pragmatic) communication disorder is not diagnosed, rather autism is the diagnosis. Social (pragmatic) communication disorder is considered only when the restricted interests and repetitive behaviors have never been present. ADHD may overlap with social (pragmatic) communication disorder in social communication disturbance; however, the core features of ADHD are not likely to be confused with autism spectrum disorder. In some cases, however, the two disorders may coexist. Another childhood disorder with socially impairing symptoms that may overlap with social (pragmatic) communication disorder is social anxiety disorder. In social anxiety disorder, however, social communication skills are present, but not manifested in feared social situations. In social (pragmatic) communication disorder, appropriate social communication skills are not present in any setting. Both social anxiety disorder and social (pragmatic) communication disorder may occur comorbidly, however, and children with social (pragmatic) communication disorder may be at higher risk for social anxiety disorder. Finally, intellectual disability may be confused with social (pragmatic) communication disorder, in that social communication skills may be deficits in children with intellectual disability. A diagnosis of social (pragmatic) communication disorder is made only when social communication skills are clearly more severe than the intellectual disability. 09 - 31.4e Unspecified Communication Disorder 31.4e Unspecified Communication Disorder COURSE AND PROGNOSIS The course and outcome of social (pragmatic) communication disorder is highly variable and dependent on both the severity of the disorder and potential interventions administered. By age 5 years, most children demonstrate enough speech and language to be able to discern the presence of deficits in social communication. However, in the milder forms of the disorder, social communication deficits may not be identified until adolescence, when language and social interactions are sufficiently complex that deficits stand out. Many children have significant improvement over time; however, even so, some early pragmatic deficits may cause lasting impairment in social relationships and in academic progress. There is a newly growing body of investigations on therapeutic interventions that may affect future outcome and prognosis of social (pragmatic) communication disorder. TREATMENT There are few data to date to inform an evidence-based treatment for social (pragmatic) communication disorder, or to fully distinguish it from other disorders with overlapping symptoms such as autism spectrum disorder, ADHD, and social anxiety disorder. A randomized controlled trial of a social communication intervention directed specifically at children with social (pragmatic) communication disorder aimed at three areas of communication: (1) social understanding and social interaction; (2) verbal and nonverbal pragmatic skills, including conversation; and (3) language processing, involving making inferences, and learning new words. Although the primary outcome measure in this study did not show significant differences for the intervention group versus the “treatment as usual” group, there were several ratings by parents and teachers that demonstrate potential improvements in social communication skills after a 20-session intensive intervention for social (pragmatic) communication disorder. It is clear that continued investigation is necessary to both validate the preceding results and to promote evidence-based treatments for children with social (pragmatic) communication disorder. 31.4e Unspecified Communication Disorder Disorders that do not meet the diagnostic criteria for any specific communication disorder fall into the category of unspecified communication disorder. An example is voice disorder, in which the patient has an abnormality in pitch, loudness, quality, tone, or resonance. To be coded as a disorder, the voice abnormality must be sufficiently severe to impair academic achievement or social communication. Operationally, speech production can be broken down into five interacting subsystems, including respiration (airflow from the lungs), phonation (sound generation in the larynx), resonance (shaping of the sound quality in the pharynx and nasal cavity), articulation (modulation of the sound stream into consonant and vowel sounds with the tongue, jaw, and lips), and suprasegmentalia (speech rhythm, loudness, and intonation). these systems work together to convey information, and voice quality conveys information about the speaker’s emotional, psychological, and physical status. Thus, voice abnormalities can cover a broad area of communication as well as indicate many different types of abnormalities. Cluttering is not listed as a disorder in the DSM-5, but it is an associated speech abnormality in which the disturbed rate and rhythm of speech impair intelligibility. Speech is erratic and dysrhythmic and consists of rapid, jerky spurts that are inconsistent with normal phrasing patterns. The disorder usually occurs in children between 2 and 8 years of age; in two thirds of cases, the patient recovers spontaneously by early adolescence. Cluttering is associated with learning disorders and other communication disorders. REFERENCES Adams C, Lockton E, Freed J, Gaile J, Earl G, McBean K, Nash J, Green J, Vail A, Law J. The Social Communication Intervention Project: A randomized controlled trial of the effectiveness of speech and language therapy for school-age children who have pragmatic and social communication problems with or without autism spectrum disorder. Int J Lang Commun Disord. 2012;47:233–244. Blumgart E, Tran Y, Craig A. Social anxiety in adults who stutter. 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Practitioner Review: Social (pragmatic) communication disorder conceptualization, evidence and clinical implications. J Child Psychol and Psychiatry., 2014;55(3)204–216. Onslow M, O’Brien S. Management of childhood stuttering. J Paediatr Child Health. 2013;49:E112–E115. Packman A, Onslow M. Searching for the cause of stuttering. Lancet. 2002;360:655–656. Petursdottir AI, Carr JE. A review of the recommendations for sequencing receptive and expressive language instruction. J Applied Behavior Analysis. 2011;44:859–876. Ramus F, Marshall DR, Rosen S, van der Lely HK. Phonological deficits in specific language impairment and developmental dyslexia: Towards a multidimensional model. Brain. 2012;136:630–645. Reilly S, Wake M, Ukoumunne OC, Bavin E, Prior M, Cini E, Conway L, Eadie P, Bretherton L. Predicting language outcomes at 4 years of age: Findings from Early Language in Victoria study. Pediatrics. 2010;126:e1530–e1537. Reisinger LM, Cornish KM, Fombonne E. 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A phase II trial of the Westmead Program: Syllable- 10 - 31.5 Autism Spectrum Disorder 31.5 Autism Spectrum Disorder timed speech treatment for preschool children who stutter. Int J Speech Lang Pathol. 2011;13:500–509. Verhoeven L, van Balkom H, eds. Classification of Developmental Language Disorders. Theoretical Issues and Clinical Implications. Mahwah, NJ: Erlbaum; 2004. Wake M, Levickis P, Tobin S, Zens N, Law J, Gold L, Ukoumunne OC, Goldfield S, Le Ha ND, Skeat J, Reilly S. Improving outcomes of preschool language delay in the community: Protocol for the Language for Learning randomized controlled trial. BMC Pediatrics. 2012;12:96–107. Wake M, Tobin S, Girolametto L, Ukomunne OC, Gold L, Levickis P, Sheehan J, Goldfeld S, Reilly S. Outcomes of population based language promotion for slow to talk toddlers at ages 2 and 3 years: Let’s Learn Language cluster randomised clinical trial. BMJ. 2011;343–355. Yaruss JS, Coleman Ce, Quesal RW. Stuttering in school-age children: A comprehensive approach to treatment. Lang Speech Hear Serv Sch. 2012;43:536–548. 31.5 Autism Spectrum Disorder Autism spectrum disorder, previously known as the pervasive developmental disorders, is a phenotypically heterogeneous group of neurodevelopmental syndromes, with polygenic heritability, characterized by a wide range of impairments in social communication and restricted and repetitive behaviors. Prior to the development of the Fifth Edition of the American Psychiatric Association’s Diagnostic and Statistical Manual of Mental Disorders (DSM-5), autism spectrum disorder was conceptualized as five discrete disorders, including: autistic disorder, Asperger’s disorder, childhood disintegrative disorder, Rett syndrome, and pervasive developmental disorder not otherwise specified. Autistic disorder was characterized by impairments in three domains: social communication, restricted and repetitive behaviors, and aberrant language development and usage. A less extensive form of autism spectrum disorder, Asperger’s disorder, did not include language impairment as a diagnostic criterion. Recent clinical consensus has shifted the conceptualization of autism spectrum disorder toward a continuum model in which heterogeneity of symptoms is recognized as inherent in the disorder, and core diagnostic impairments are collapsed into two domains: deficits in social communication, and restricted and repetitive behaviors. Aberrant language development and usage is no longer considered a core feature of autism spectrum disorder. This diagnostic change, is based, in part, on recent studies in siblings with diagnoses of autistic disorder, suggesting that symptom domains may be transmitted separately, and that aberrant language development and usage is not a defining feature, but an associated feature in some individuals with autism spectrum disorder. Autism spectrum disorder is typically evident during the second year of life, and in severe cases, a lack of developmentally appropriate interest in social interactions may be noted even in the first year. Some studies suggest that a decline in social interaction may ensue between the first and second years of life. However, in milder cases, core impairments in autism spectrum disorder may not be identified for several more years. Although language impairment is not a core diagnostic criterion in autism spectrum disorder, clinicians and parents share concerns about a child who by 12 to 18 months has not developed any language, and delayed language accompanied by diminished social behavior are frequently the heralding symptoms in autism spectrum disorder. In up to 25 percent of cases of autism spectrum disorder, some language develops and is subsequently lost. Autism spectrum disorder in children with normal intellectual function and mild impairment in language function may not be identified until middle childhood when both academic and social demands are increased. Children with autism spectrum disorder often exhibit idiosyncratic intense interest in a narrow range of activities, resist change, and typically, do not respond to their social environment in accordance with their peers. According to the DSM-5, diagnostic criteria for autism spectrum disorder include deficits in social communication and restricted interests, which present in the early developmental period, however, when subtle, may not be identified until several years later. approximately one third of children meeting the current DSM-5 diagnosis of autism spectrum disorder, exhibit intellectual disability (ID). Of interest, is that according to DSM-IV-TR, Rett syndrome or disorder appeared to occur exclusively in females and is characterized by normal development for at least 6 months, followed by stereotyped hand movements, a loss of purposeful motions, diminishing social engagement, poor coordination, and decreasing language use. In the formerly labeled childhood disintegrative disorder, development progresses normally for approximately 2 years, after which the child shows a loss of previously acquired skills in two or more of the following areas: language use, social responsiveness, play, motor skills, and bladder or bowel control. The former Asperger’s disorder is characterized by impairment in social relatedness and repetitive and stereotyped patterns of behavior without a delay or marked aberrant in language development and usage. In Asperger’s disorder, cognitive abilities and major adaptive skills are age appropriate, although social communication is impaired. A survey of children undertaken with the former autism spectrum disorders, revealed that the average age of diagnosis was 3.1 years for children with autistic disorder, 3.9 years for children diagnosed with pervasive developmental disorder not otherwise specified, and 7.2 years for those youth with Asperger’s disorder. Children with autism spectrum disorder who exhibited severe language deficits received an autism spectrum disorder diagnosis, on average, a year earlier than children without impairment in language. Children with autism spectrum disorder who exhibited repetitive behaviors such as hand-flapping, toe-walking, and odd play were also identified with autism spectrum disorder disorders at a younger age than those who did not exhibit such behaviors. The current DSM-5 autism spectrum disorder criteria provide specifiers for severity of the main domains of impairment and also specifiers for the presence or absence of language impairment and intellectual impairment. HISTORY OF AUTISTIC DISORDER “Early infantile autism” was described by Leo Kanner in 1943; however, even as early as 1867, the psychiatrist Henry Maudsley had observed a group of very young children with severe mental disorders characterized by marked deviation, delay, and distortion in development. In that era, most serious developmental disturbance in young children was believed to fall within the category of psychoses. Kanner’s classic paper “Autistic Disturbances of Affective Contact,” coined the term infantile autism and provided a clear, comprehensive account of the early childhood syndrome. Kanner described children who exhibited extreme “autistic aloneness”; failure to assume an anticipatory posture; delayed or deviant language development with echolalia and often with pronominal reversal (using you for I); monotonous repetitions of noises or verbal utterances; excellent rote memory; limited range of spontaneous activities, stereotypies, and mannerisms; and anxiously obsessive desire for the maintenance of sameness and dread of change. Socially, Kanner’s sample was described as having poor eye contact; awkward relationships; and a preference for pictures and inanimate objects. Kanner suspected that the syndrome was more frequent than it seemed, and suggested that some children with infantile autism may have been misclassified as “mentally retarded” or schizophrenic. Before 1980, children with symptoms of autism spectrum disorder were generally diagnosed with childhood schizophrenia. Over time, it became evident that autism spectrum disorder and schizophrenia were two distinct psychiatric entities. In some cases, however, a child with autism spectrum disorder may develop comorbid schizophrenic disorder later in childhood. EPIDEMIOLOGY Prevalence Autism spectrum disorders have been increasingly diagnosed over the last two decades, with the current prevalence estimated at approximately 1 percent in the United States. Autistic disorder, based on DSM-IV-TR criteria, is believed to occur at a rate of about 8 cases per 10,000 children (0.08 percent). By definition, the onset of autism spectrum disorder is in the early developmental period; however, some cases are not recognized until the child is much older. Because of this delay between onset and diagnosis, the prevalence rates increase with age in young children. Sex Distribution Autism spectrum disorder is diagnosed four times more often in boys than in girls. In clinical samples, girls with autism spectrum disorder more often exhibit intellectual disability than boys. One potential explanation for this is that girls with autism spectrum disorder without intellectual disability may be less likely to be identified, referred clinically, and diagnosed. ETIOLOGY AND PATHOGENESIS Genetic Factors Family and twin studies suggest that autism spectrum disorder has a significant heritable contribution; however, it does not appear to be fully penetrant. Although up to 15 percent of cases of autism spectrum disorder appear to be associated with a known genetic mutation, in most cases, its expression is dependent on multiple genes. Family studies have demonstrated increased rates of autism spectrum disorder in siblings of an index child, as high as 50 percent in some families with two or more children with autism spectrum disorder. Siblings of a child with autism spectrum disorder are also at increased risk for a variety of developmental impairments in communication and social skills, even when they do not meet criteria for autism spectrum disorder. The concordance rate of autistic disorder in two large twin studies was 36 percent in monozygotic pairs versus 0 percent in dizygotic pairs in one study and about 96 percent in monozygotic pairs versus about 27 percent in dizygotic pairs in the second study. High rates of cognitive impairments, in the nonautistic twin in monozygotic twins with perinatal complications, suggest that contributions of perinatal environmental factors interact with genetic vulnerability differentially in autism spectrum disorder. The heterogeneity in expression of symptoms in families with autism spectrum disorder suggests that there are multiple patterns of genetic transmission. Studies indicate that both an increase and decrease in certain genetic patterns may be risk factors for autism spectrum disorder. In addition to specific genetic factors, gender plays a strong role in the expression of autism spectrum disorder. Genetic studies have identified two biological systems that are influenced in autism spectrum disorder: the consistent finding of elevated platelet serotonin (5-HT), and the mTOR, that is, mammalian target of rapamycin–linked synaptic plasticity mechanisms, which appear to be disrupted in autism spectrum disorder. These will be discussed further in the next section. A number of known genetically caused syndromes include autism spectrum disorder as part of a broader phenotype. The most common of these inherited disorders is fragile X syndrome, an X-linked recessive disorder that is present in 2 to 3 percent of individuals with autism spectrum disorder. Fragile X syndrome exhibits a nucleotide repeat in the 5’ untranslated region of the FMNR1 gene, resulting in symptoms of autism spectrum disorder. Children with fragile X syndrome characteristically exhibit intellectual disability, gross and fine motor impairments, an unusual facies, macroorchidism, and significantly diminished expressive language ability. Tuberous sclerosis, another genetic disorder characterized by multiple benign tumors, inherited by autosomal dominant transmission, is found with greater frequency among children with autism spectrum disorder. Up to 2 percent of children with autism spectrum disorder also have tuberous sclerosis. Researchers who screened the DNA of more than 150 pairs of siblings with autism spectrum disorder found evidence of two regions on chromosomes 2 and 7 containing genes that may contribute to autism spectrum disorder. Additional genes hypothesized to be involved in autism spectrum disorder were found on chromosomes 16 and 17. Biomarkers in Autism Spectrum Disorder Autism spectrum disorder is associated with several biomarkers, potentially resulting from interactions of genes and environmental factors, which then influence neuronal function, dendrite development, and contribute to altered neuronal information processing. Several biomarkers of abnormal signaling in the 5-HT system, the mTORlinked synaptic plasticity mechanisms, and alterations of the γ-aminobutyric acid (GABA) inhibitory system. The first biomarker identified in autism spectrum disorder was elevated serotonin in whole blood, almost exclusively in the platelets. Platelets acquire 5-HT through the process of SERT (serotonin transporter), known to be hereditary, as they pass through the intestinal circulation. The genes that mediate SERT (SLC64A), and the 5-HT receptor 5-HT 2A gene (HTR2A) are known to be more heritable than autism spectrum disorder, and encode the same protein in the platelets and in the brain. Because 5-HT is known to be involved in brain development, it is possible that the changes in 5-HT regulation may lead to alterations in neuronal migration and growth in the brain. Both structural and functional neuroimaging studies have suggested specific biomarkers associated with autism spectrum disorder. Several studies found increased total brain volume in children younger than 4 years of age with autism spectrum disorder, whose neonatal head circumferences were within normal limits or slightly below. By about age 5 years, however, 15 to 20 percent of children with autism spectrum disorder developed macrocephaly. Additional studies found confirmatory data in samples of infants who were later diagnosed with autism spectrum disorder, who exhibited normal head circumferences at birth; by 4 years, 90 percent had larger brain volumes than controls, with 37 percent of the autism spectrum disorder group meeting criteria for macrocephaly. In contrast, structural magnetic resonance imaging (sMRI) studies of children with autism spectrum disorder ranging from 5 to 16 years did not find mean values of total brain volume increased. One study followed the size of the amygdala in youth with autism spectrum disorder in the first few years of life, and similarly, found an increased size in the first few years of life, followed by a decrease in size over time. The size of the striatum has also been found in several studies to be enlarged in young children with autism spectrum disorder, with a positive correlation of striatal size with frequency of repetitive behaviors. The dynamic process of the atypical and changing total brain volume observed in children with autism spectrum disorder lends support for the overarching hypothesis that there are sensitive periods or “critical periods” within the brain’s plasticity that may be disrupted in ways that may contribute to the emergence of autism spectrum disorder. Functional MRI (fMRI) studies have focused on identifying biomarkers, that is, the functional brain correlates of various observed core symptoms in autism spectrum disorder. fMRI studies of children, adolescents, and adults with autism spectrum disorder have employed tasks including face perception, neutral face tasks, “theory of mind” deficits, language and communication impairments, working memory and repetitive behaviors. fMRI studies have provided evidence that individuals with autism spectrum disorder have a tendency to scan faces differently than controls, in that they focus more on the mouth region of the face rather than on the eye region and rather than scan the entire face multiple times, individuals with autism spectrum disorder focus more on individual features of the face. In response to socially relevant stimuli, researchers have come to the conclusion that individuals with autism spectrum disorder have greater amygdala hyperarousal. In terms of “theory of mind,” that is, the ability to attribute emotional states to others, and to oneself, fMRI studies find differences in activation in brain regions such as the right temporal lobe and other areas of the brain known to become activated in controls during tasks involving theory of mind. This difference has been hypothesized by some researchers to represent dysfunction of the mirror neuron system (MNS). Atypical patterns of frontal lobe activation have been found in multiple studies of autism spectrum disorder during face processing tasks, suggesting that this area of the brain may be critical in social perception and emotional reasoning. Decreased activation in individuals with autism spectrum disorder in the left frontal regions of the brain during memory and language-based tasks led researchers to hypothesize that individuals with autism spectrum disorder utilized more visual strategies during language processing than controls did. Both sMRI and fMRI research has contributed to demonstrating brain correlates of core impairments observed in individuals with autism spectrum disorder. Immunological Factors Several reports have suggested that immunological incompatibility (i.e., maternal antibodies directed at the fetus) may contribute to autistic disorder. The lymphocytes of some autistic children react with maternal antibodies, which raises the possibility that embryonic neural tissues may be damaged during gestation. These reports usually reflect single cases rather than controlled studies, and this hypothesis is still under investigation. Prenatal and Perinatal Factors A higher-than-expected incidence of prenatal and perinatal complications seems to occur in infants who are later diagnosed with autism spectrum disorder. The most significant prenatal factors associated with autism spectrum disorder in the offspring are advanced maternal and paternal age at birth, maternal gestational bleeding, gestational diabetes, and first-born baby. Perinatal risk factors for autism spectrum disorder include umbilical cord complications, birth trauma, fetal distress, small for gestational age, low birth weight, low 5-minute Apgar score, congenital malformation, ABO blood group system or Rh factor incompatibility and hyperbilirubinemia. Many of the obstetrical complications that are associated with risk for autism spectrum disorder are also risk factors for hypoxia, which may be an underlying risk factor itself. There is not sufficient evidence to implicate any one single perinatal or prenatal factor in autism spectrum disorder etiology, and a genetic predisposition to autism spectrum disorder may be interacting with perinatal factors. Comorbid Neurological Disorders Electroencephalography (EEG) abnormalities and seizure disorders occur with greater than expected frequency in individuals with autism spectrum disorder. Four percent to 32 percent of individuals with autism spectrum disorder have grand mal seizures at some time, and about 20 to 25 percent show ventricular enlargement on computed tomography (CT) scans. Various EEG abnormalities are found in 10 to 83 percent of children with the previously defined autistic disorder, and although no EEG finding is specific to autistic disorder, there is some indication of failed cerebral lateralization. The current consensus is that autism spectrum disorder is a set of behavioral syndromes caused by a multitude of factors acting on the central nervous system. Psychosocial Theories Studies comparing parents of children with autism spectrum disorder with parents of normal children have shown no significant differences in child-rearing skills. Kanner’s early speculations that parental emotional factors might be implicated as contributing to the development of autism spectrum disorder have been clearly refuted. DIAGNOSIS AND CLINICAL FEATURES The DSM-5 diagnostic criteria for autism spectrum disorder are shown in Table 31.5-1. Table 31.5-1 DSM-5 Diagnostic Criteria for Autism Spectrum Disorder Core Symptoms of Autism Spectrum Disorder Persistent Deficits In Social Communication and Interaction. Children with autism spectrum disorder characteristically do not conform to the expected level of reciprocal social skills and spontaneous nonverbal social interactions. Infants with autism spectrum disorder may not develop a social smile, and as older babies may lack the anticipatory posture for being picked up by a caretaker. Less frequent and poor eye contact is common during childhood and adolescence compared to other children. The social development of children with autism spectrum disorder is characterized by atypical, but not absent, attachment behavior. Children with autism spectrum disorder may not explicitly acknowledge or differentiate the most important persons in their lives—parents, siblings, and teachers—and on the other hand, may not react as strongly to being left with a stranger compared to others their age. Children with autism spectrum disorder often feel and display extreme anxiety when their usual routine is disrupted. By the time children with autism spectrum disorder reach school age, their social skills may have increased, and social withdrawal may be less obvious, particularly in higher-functioning children. An observable deficit, however, often remains in spontaneous play with peers and in subtle social abilities that promote developing friendships. The social behavior of children with autism spectrum disorder is often awkward and may be inappropriate. In older school-aged children, social impairments may be manifested in a lack of conventional back and forth conversation, fewer shared interests, and fewer body and facial gestures during conversations. Cognitively, children with autism spectrum disorder are frequently more skilled in visual-spatial tasks than in tasks requiring skill in verbal reasoning. One observation of the cognitive style of children with autism spectrum disorder is an impaired ability to infer the feelings or emotional state of others around them. That is, individuals with autism spectrum disorder have difficulty with making attributions about the motivation or intentions of others (also termed “theory of mind”) and thus have difficulty developing empathy. The lack of a “theory of mind” produces difficulties interpreting the social behavior of others and leads to a lack of social reciprocation. Individuals with autism spectrum disorder generally desire friendships, and higher functioning children may be aware that their lack of spontaneity and poor skills in responding to the emotions and feelings of their peers are major obstacles in developing friendships. Children with autism spectrum disorder are often avoided or shunned by peers who expect them to conform to their mainstream activities, and experience their behavior as awkward and alienating. Adolescents and adults with autism spectrum disorder often desire romantic relationships, and for some, their increase in social competence and skills over time enables them to develop long-term relationships. Restricted, Repetitive Patterns of Behavior, Interests, and Activities. From the first years of life, in a child with autism spectrum disorder, developmentally expected exploratory play is restricted and muted. Toys and objects may not be used typically, instead, are often manipulated in a ritualistic manner, with fewer symbolic features. Children with autism spectrum disorder generally do not show the level of imitative play or abstract pantomime that other children of their age exhibit spontaneously. The activities and play of children with autism spectrum disorder may appear more rigid, repetitive, and monotonous than their peers. Ritualistic and compulsive behaviors are common in early and middle childhood. Children with autism spectrum disorder often seem to enjoy spinning, banging, and watching water flowing. Frank compulsive behaviors are not uncommon among children with autism spectrum disorder, such as lining up objects, and not infrequently a child with autism spectrum disorder may exhibit a strong attachment to a particular inanimate object. Children with autism spectrum disorder who are severely intellectually disabled have increased rates of self-stimulatory and self-injurious behaviors. Stereotypies, mannerisms, and grimacing emerge most frequently when a child with autism spectrum disorder is a less- structured situation. Children with autism spectrum disorder often find transitions and changes intimidating. Moving to a new house, rearranging furniture in a room, or even a change such as eating a meal before a bath when the reverse was the routine, may evoke panic, fear, or temper tantrums in a child with autism spectrum disorder. Associated Physical Characteristics. At first glance, children with autism spectrum disorder do not show any physical signs indicating the disorder. Children with autism spectrum disorder, overall, do exhibit higher rates of minor physical anomalies, such as ear malformations, and others that may reflect abnormalities in fetal development of those organs along with parts of the brain. A greater than expected number of children with autism spectrum disorder do not show early handedness and lateralization, and remain ambidextrous at an age when cerebral dominance is established in most children. Children with autism spectrum disorder have been observed to have a higher incidence of abnormal dermatoglyphics (e.g., fingerprints) than those in the general population. This finding may suggest a disturbance in neuroectodermal development. Associated Behavioral Symptoms that May Occur in Autism Spectrum Disorder Disturbances In Language Development and Usage. Deficits in language development and difficulty using language to communicate ideas are not among the core criteria for diagnosing autism spectrum disorder; however, they occur in a subset of those individuals with autism spectrum disorder. Some children with autism spectrum disorder are not simply reluctant to speak, and their speech abnormalities do not result from lack of motivation. Language deviance, as much as language delay, is characteristic of more severe subtypes of autism spectrum disorder. Children with severe autism spectrum disorder have significant difficulty putting meaningful sentences together, even when they have large vocabularies. When children with autism spectrum disorder whose language was delayed do learn to converse fluently, their conversations may impart information without typical prosody or inflection. In the first year of life, a typical pattern of babbling may be minimal or absent. Some children with autism spectrum disorder vocalize noises—clicks, screeches, or nonsense syllables—in a stereotyped fashion, without a seeming intent of communication. Unlike most young children who generally have better receptive language skills than expressive ones, children with autism spectrum disorder may express more than they understand. Words and even entire sentences may drop in and out of a child’s vocabulary. It is not atypical for a child with autism spectrum disorder to use a word once and then not use it again for a week, a month, or years. Children with autism spectrum disorder may exhibit speech that contains echolalia, both immediate and delayed, or stereotyped phrases that seem out of context. These language patterns are frequently associated with pronoun reversals. A child with autistic disorder might say, “You want the toy” when she means that she wants it. Difficulties in articulation are also common. Many children with autistic disorder use peculiar voice quality and rhythm. About 50 percent of autistic children never develop useful speech. Some of the brightest children show a particular fascination with letters and numbers. Children with autism spectrum disorder sometimes excel in certain tasks or have special abilities; for example, a child may learn to read fluently at preschool age (hyperlexia), often astonishingly well. Very young children with autism spectrum disorder who can read many words, however, have little comprehension of the words read. Intellectual Disability. About 30 percent of children with autism spectrum disorder function in the intellectually disabled range of intellectual function. Of those, about 30 percent of children function in the mild to moderate range, and about 45 to 50 percent are severely to profoundly intellectually disabled. The intelligent quotient (IQ) scores of autism spectrum disorder children with intellectual impairments tend to reflect most severe problems with verbal sequencing and abstraction skills, with relative strengths in visuospatial or rote memory skills. This finding suggests the importance of defects in language-related functions. Irritability. Broadly defined, irritability includes aggression, self-injurious behaviors, and severe temper tantrums. These phenomena are commonly encountered in children and adolescents with autism spectrum disorder. Severe temper tantrums may be difficult to subdue, and self-injurious behaviors are often problematic to control. These symptoms are often produced by everyday situations in which these youth are expected to transition from one activity to another, sit in a classroom setting, or remain still when they desire to run around. In children with autism spectrum disorder who are lower functioning and have intellectual deficits, aggression may emerge unexpectedly without an obvious trigger or purpose, and self-injurious behaviors such as head banging, skin picking, and biting oneself may also be noted. Instability of Mood and Affect. Some children with autism spectrum disorder exhibit sudden mood changes, with bursts of laughing or crying without an obvious reason. It is difficult to learn more about these episodes if the child cannot express the thoughts related to the affect. Response to Sensory Stimuli. Children with autism spectrum disorder have been observed to overrespond to some stimuli and underrespond to other sensory stimuli (e.g., to sound and pain). It is not uncommon for a child with autism spectrum disorder to appear deaf, at times showing little response to a normal speaking voice; on the other hand, the same child may show intent interest in the sound of a wristwatch. Some children have a heightened pain threshold or an altered response to pain. Indeed, some children with autism spectrum disorder do not respond to an injury by crying or seeking comfort. Some youth with autism spectrum disorder perseverate on a sensory experience; for example, they frequently hum a tune or sing a song or commercial jingle before saying words or using speech. Some particularly enjoy vestibular stimulation— spinning, swinging, and up-and-down movements. Hyperactivity and Inattention. Hyperactivity and inattention are both common behaviors in young children with autism spectrum disorder. Lower than average activity level is less frequent; when present, it often alternates with hyperactivity. Short attention span, poor ability to focus on a task, may also interfere with daily functioning Precocious Skills. Some individuals with autism spectrum disorder have precocious or splinter skills of great proficiency, such as prodigious rote memories or calculating abilities, usually beyond the capabilities of their normal peers. Other potential precocious abilities in some children with autism spectrum disorder include hyperlexia, an early ability to read well (even though they cannot understand what they read), memorizing and reciting, and musical abilities (singing or playing tunes or recognizing musical pieces). Insomnia. Insomnia is a frequent sleep problem among children and adolescents with autism spectrum disorder, estimated to occur in 44 to 83 percent of school-aged children. Both behavioral and pharmacologic interventions have been applied as interventions. Behavioral interventions include modification of parental behavior before and at bedtime, and providing routines that remove reinforcers for remaining awake. Medication interventions have included melatonin, which appears to be a promising agent in doses ranging from 1 mg fast-release to 4 mg controlled-release in the few controlled studies for insomnia in youth with autism spectrum disorder. Minor Infections and Gastrointestinal Symptoms. Young children with autism spectrum disorder have been reported to have a higher-than-expected incidence of upper respiratory infections and other minor infections. Gastrointestinal symptoms commonly found among children with autism spectrum disorder include excessive burping, constipation, and loose bowel movements. Also seen is an increased incidence of febrile seizures in children with autism spectrum disorder. Some children do not show temperature elevations with minor infectious illnesses and may not show the typical malaise of ill children. In other children, behavior problems and relatedness seem to improve noticeably during a minor illness, and in some, such changes are a clue to physical illness. Assessment Tools A standardized instrument that can be very helpful in eliciting comprehensive information regarding autism spectrum disorder is the Autism Diagnostic Observation Schedule-Generic (ADOS-G). Brett was the first of two children born to middle-class parents both in their early 40s after difficult pregnancy, with an induced labor at 36 weeks due to fetal distress. As an infant, Brett was undemanding and relatively placid; he did not have colic, and motor development proceeded appropriately, but language development was delayed. Brett’s parents first became concerned about his development when he was 18 months of age and still not speaking; however, upon questioning, they noted that, in comparison to other toddlers in his play group, Brett had seemed less uninterested in social interaction and the social games with toddlers and adults. Stranger anxiety became marked at 18 months, much later compared to the other toddlers in his day care program. Brett would become extremely upset if his usual day care worker was not present and would tantrum until his mother took him home. Brett’s pediatrician initially reassured his parents that he was a “late talker”; however, when Brett was 24 months old he was referred for developmental evaluation. At 24 months, motor skills were age appropriate. His language and social development, however, was severely delayed, and he was noted to be resistant to changes in routine and unusually sensitive to aspects of the inanimate environment. Brett’s play skills were quite limited, and he played with toys in repetitive and idiosyncratic ways. His younger sister, now 12 months, was beginning to say a few words, and the family history was negative for language and developmental disorders. A comprehensive medical evaluation revealed a normal EEG and CT scan; genetic screening and chromosome analysis were normal as well. Brett was diagnosed with autism spectrum disorder, and he was enrolled in a special education program in which he gradually began to speak. His speech was extremely literal and characterized by a monotonic voice quality and an occasional pronoun reversal. Brett often spoke and was able to make his needs known; however, his language was odd and the other toddlers did not play with him. Brett pursued mainly solo activities and remained quite isolated. By age 5 years, Brett was quite attached to his mother and often became separation anxious and upset when she went out, exhibiting severe tantrums. Brett also had developed a number of self-stimulatory behaviors in which he engaged, such as waving his fingers in front of his eyes. His extreme sensitivity to change continued over the next few years. Intelligence testing revealed a full-scale IQ in the average range with relative weakness in the verbal subtests compared to the performance subtests. In the 4th grade, Brett began to have serious behavioral problems at school and at home. Brett was unable to complete his class work, would wander around the classroom, and would begin to tantrum when the teacher insisted that he sit in his seat. He would sometimes begin screaming so loudly that he had to be asked to leave the classroom. He would then become upset and throw all of his books off his desk in a rage, sometimes inadvertently hitting other students. It took him up to 2 hours to calm down. At home, Brett would fly into a tantrum if anyone touched his things, and he would become stubborn and belligerent when asked to do anything that he was not expecting. Brett’s tantrum behavior continued into middle school, and by the 8th grade, when he was 13 years old, these behaviors became so severe that the school warned his parents that he was becoming unmanageable. Brett was evaluated by a child and adolescent psychiatrist who recommended a social skills group for him and prescribed risperidone, starting with 0.5 mg p.o. b.i.d. and titrating up to 1.5 mg p.o. bid. At that dose, Brett’s tantrums were less frequent and less severe. Brett seemed calmer in general, and did not become physically out of control during tantrums. Brett continued in middle school in a combination of special education classes and regular classes. Brett’s social skills group was helpful in terms of teaching him how to approach peers in ways that would lead to less rejection. Brett had made some acquaintances, and by the time he started high school, he had acquired two friends who would come to his home and play video games with him. Brett knew that he was different than the other students, but he had trouble articulating what was different about him. Brett continued in high school with a combination of special and regular education and had plans to attend a community college and live at home for the first year. (Adapted from a case by Fred Volkmar, M.D.) DIFFERENTIAL DIAGNOSIS Disorders to consider in the differential diagnosis of autism spectrum disorder include social (pragmatic) communication disorder, the newly described DSM-5 communication disorder; schizophrenia with childhood onset; congenital deafness or severe hearing disorder; and psychosocial deprivation. It is also difficult to make the diagnosis of autism spectrum disorder because of its potentially overlapping symptoms with childhood schizophrenia, intellectual disability syndromes with behavioral symptoms, and language disorders. In view of the many concurrent problems often encountered in autism spectrum disorder, Michael Rutter and Lionel Hersov suggested a stepwise approach to the differential diagnosis. Social (Pragmatic) communication disorder This disorder is characterized by difficulty in conforming to typical storytelling, understanding the rules of social communication through language, exemplified by a lack of conventional greeting others, taking turns in a conversation, and responding to verbal and nonverbal cues of a listener. Other forms of language impairment may accompany social communication disorder such as delay in learning language or expressive and receptive difficulties. Social communication disorder is found with greater frequency in relatives of individuals with autism spectrum disorder, which increases the difficulty in discriminating this disorder from autism spectrum disorder. Although relationships may be negatively affected by social communication disorder, this disorder does not include restricted or repetitive behaviors and interests, as autism spectrum disorder does. Childhood Onset Schizophrenia Schizophrenia is rare in children younger than 12 years and almost nonexistent before the age of 5 years. Characterized by hallucinations or delusions, childhood onset schizophrenia has a lower incidence of seizures and intellectual disability and poor social skills. Table 31.5-2 compares autism spectrum disorder and schizophrenia with childhood onset. Table 31.5-2 Autism Spectrum Disorder versus Childhood Onset Schizophrenia Intellectual Disability with Behavioral Symptoms Children with intellectual disability may exhibit behavioral symptoms that overlap with some autism spectrum disorder features. The main differentiating features between autism spectrum disorder and intellectual disability are that children with intellectual disability syndromes generally display global impairments in both verbal and nonverbal areas, whereas children with autism spectrum disorder are relatively weak in social interactions compared to other areas of performance. Children with intellectual disability generally relate verbally and socially to adults and peers in accordance with their mental age, and they exhibit a relatively even profile of limitations. Language Disorder Some children with language disorders also have autism spectrum disorder features, which may present a diagnostic challenge. Table 31.5-3 summarizes the major differences between autism spectrum disorder and language disorders. Table 31.5-3 Autism Spectrum Disorder versus Language Disorder Congenital Deafness or Hearing Impairment Because children with autism spectrum disorder may appear mute or lack language development, congenital deafness and hearing impairment must be considered and ruled out. Differentiating factors include the following: infants with autism spectrum disorder may babble only infrequently, whereas deaf infants often have a history of relatively normal babbling that then gradually tapers off and may stop at 6 months to 1 year of age. Deaf children generally respond only to loud sounds, whereas children with autism spectrum disorder may ignore loud or normal sounds and respond to soft or low sounds. Most importantly, audiogram or auditory-evoked potentials indicate significant hearing loss in deaf children. Deaf children usually seek out nonverbal social communication with regularity and seek social interactions with peers and family members more consistently than children with autism spectrum disorder. Psychosocial Deprivation Severe neglect, maltreatment, and lack of parental care can lead children to appear apathetic, withdrawn, and alienated. Language and motor skills may be delayed. Children with these signs generally improve when placed in a favorable and enriched psychosocial environment, but such improvement is not the case with children with autism spectrum disorder. COURSE AND PROGNOSIS Autism spectrum disorder is typically a lifelong, albeit heterogeneous, disorder with a highly variable severity and prognosis. Children with autism spectrum disorder and IQs above 70 with average adaptive skills, who develop communicative language by ages 5 to 7 years, have the best prognoses. A longitudinal study comparing symptoms in children with high-IQ autism spectrum disorder at the age of 5 years, with their symptoms at age 13 through young adulthood, found that a small proportion no longer met criteria for autism spectrum disorder. Most of these youth demonstrated positive changes in communication and social domains over time. Early intensive behavioral interventions have been found to provide a profound positive impact on many children with autism spectrum disorder, and in some cases lead to recovery and function in the average range. The autism spectrum disorder symptom areas that do not seem to improve substantively over time with early behavioral interventions are related to ritualistic and repetitive behaviors. However, currently, evidence-based behavioral interventions specifically targeting repetitive behaviors may ameliorate them. The prognosis of a given child with autism spectrum disorder is generally improved if the home environment is supportive. TREATMENT The goals of treatment for children with autism spectrum disorder are to target core behaviors to improve social interactions, communication, broaden strategies to integrate into schools, develop meaningful peer relationships, and increase long-term skills in independent living. Psychosocial treatment interventions aim to help children with autism spectrum disorder to develop skills in social conventions, increase socially acceptable and prosocial behavior with peers, and to decrease odd behavioral symptoms. In many cases, language and academic remediation are also required. In addition, treatment goals generally include reduction of irritable and disruptive behaviors that may emerge in school and at home and may exacerbate during transitions. Children with intellectual disability require developmentally appropriate behavioral interventions to reinforce socially acceptable behaviors and encourage selfcare skills. In addition, parents of children with autism spectrum disorder often benefit from psychoeducation, support, and counseling in order to optimize their relationships and effectiveness with their children. Comprehensive treatment for autism spectrum disorder including intensive behavioral programs, parent training and participation, and academic/educational interventions have provided the most promising results. Components of these comprehensive treatments include expanding social skills, communication, and language, often through practicing imitation, joint attention, social reciprocity, and play in a directed but child-centered manner. Five randomized controlled trials (RCTs) of early intensive comprehensive behavioral interventions targeting core features of autism spectrum disorder in children ranging in age from 2 years to 5 years of age have shown increases in language acquisition, social interactions, and educational achievement at the end of the study period compared to control groups. The study periods ranged from 12 weeks to several years, and the settings were at home, in clinic, or at school. The comprehensive treatment models or adapted versions of them were used either alone or in combinations in these RCTs as described below. Psychosocial Interventions Early Intensive Behavioral and Developmental Interventions UCLA/Lovaas-based Model. This intensive and manualized intervention primarily utilizes techniques derived from applied behavior analysis, which is administered on a one-to-one basis for many hours per week. A therapist and a child will work on practicing specific social skills, language usage, and other target play skills, with reinforcement and rewards provided for accomplishments and mastery of skills. Early Start Denver Model (ESDM) Interventions are administered in naturalistic settings such as in day care, at home, and during play with other children. Parents are typically taught to be co-therapists and provide the training at home while educational settings also provide the interventions. The focus of the interventions is on developing basic play skills and relationship skills, and applied behavior analysis techniques are integrated into the interventions. This approach is focused on training for very young children and is applied within the context of the child’s daily routine. 3. Parent Training Approaches This includes Pivotal Response Training, in which parents are taught to facilitate social and communication development within the home and during activities by targeting gateway or pivotal social behaviors for mastery by the child with the expectation that once these central social skills were mastered, a natural generalizing of social behaviors would follow. Extensive parent and family components are integrated into this type of intervention. Other parent training approaches focus on language acquisition, and for parents, may be administered at a lower intensity such as weekly; however, once parents are trained, the interventions occur throughout the day with the child. Another example of a parent training approach is the Hanen More Than Words Program. Social Skills Approaches Social Skills Training. Typically provided by therapeutic leaders to children of various ages in a group setting with peers; children are given guided practice in initiating social conversation, greetings, initiating games, and joint attention. Emotion identification and regulation are often included in practice with recognizing and learning how to label emotions in given social situations, learning to attribute appropriate emotional reactions in others, and social problem-solving techniques. The goals are that with practice in the group setting, the child will be able to use the techniques in less-structured settings and internalize strategies to interact positively with peers. Behavioral Interventions (BIs) and Cognitive-Behavioral Therapy (CBT) for Repetitive Behaviors and Associated Symptoms Behavioral Therapy. Applied behavioral analysis has been found to be somewhat effective in reducing some repetitive behaviors in children and adolescents with autism spectrum disorder. Early intervention is recommended for repetitive behaviors that are self-injurious; behavioral interventions may need to be combined with pharmacologic treatments to adequately manage the symptoms. Cognitive-Behavioral Therapy. There is a significant evidence base from RCTs for the efficacy of CBT for symptoms of anxiety, depression, and obsessive-compulsive disorders in children. There are fewer controlled trials of this treatment in children with autism spectrum disorder, although there are at least two published studies in which CBT was used to treat repetitive behavior in individuals with autism spectrum disorder. Interventions for comorbid symptoms in autism spectrum disorder Neurofeedback. This modality has been administered in an attempt to influence symptoms of attention-deficit/hyperactivity disorder (ADHD), anxiety, and increased social interaction by providing computer games or other games in which the desired behavior is reinforced, while the child wears electrodes that monitor electrical activity in the brain. The aim is to influence brainwave activity to prolong or produce electrical activity present during the desired behaviors. This modality is still under investigation in the treatment of symptoms in autism spectrum disorder. 2. Management of insomnia in autism spectrum disorder. Insomnia is a prevalent concern among children and adolescents with autism spectrum disorder, and both behavioral and pharmacologic interventions may be administered to improve this condition. The most common behavioral intervention for insomnia in autism spectrum disorder is based on changing the parents behavior first toward the child at bedtime and throughout the night, such that there is a removal of reinforcement and attention for being awake, leading to a gradual extinction of the “staying awake” behavior. Several studies using massage therapy before bedtime in children with autism spectrum disorder between the ages of 2 years and 13 years provided an improvement in falling asleep and a sense of relaxation. Educational interventions for children with autism spectrum disorder Treatment and Education of Autistic and Communication-related Handicapped children (TEACCH). Originally developed at the University of North Carolina at Chapel Hill in the 1970s, TEACCH involves structured teaching based on the notion that children with autism spectrum disorder have difficulty with perception, and so this teaching method incorporates many visual supports and a picture schedule to aid in teaching academic subjects as well as socially appropriate responses. The physical environment is arranged to support visual learning, and the day is structured to promote autonomy and social relatedness. Broad-based approaches. These educational plans include a blend of teaching strategies that use behavioral analysis and also focus on language remediation. Behavioral reinforcement is provided for socially acceptable behaviors while academic subjects are being taught. TEACCH may also be incorporated into a broader special educational program for autism spectrum disorder. Computer-based approaches and virtual reality. Computer-based approaches and virtual reality teaching are centered on using computer-based programs, games, and interactive programs to teach language acquisition and reading skills. This provides the child with a sense of mastery and delivers a behaviorally based instruction in a modality that is appealing for the child. The Let’s Face It! program is a computerized game that helps to teach children with autism spectrum disorder to recognize faces. It consists of seven interactive computer games that target changes in facial expression, attention to the eye region of the face, holistic face recognition, and identifying emotional expression. A randomized controlled trial of use of this program with children with autism spectrum disorder provided evidence that after 20 hours of face training with Let’s Face It!, compared to the control group, the trained children demonstrated improvement in their ability to focus on the eye region of a face and improved their analytic and holistic face-processing skills. Several studies using virtual reality environments to teach children with autism spectrum disorder social skills and interaction have provided evidence of their value. In one study, a virtual café for children with autism spectrum disorder allowed the children to practice ordering and paying for drinks and food by navigation with the use of a computer mouse. Psychopharmacological Interventions Psychopharmacological interventions in autism spectrum disorder are mainly directed at ameliorating impairing associated behavioral symptoms rather than core features of autism spectrum disorder. Target symptoms include irritability, broadly including aggression, temper tantrums and self-injurious behaviors, hyperactivity, impulsivity, and inattention. Irritability. Two second-generation antipsychotics, risperidone and aripiprazole, have been approved by the Food and Drug Administration (FDA) in the United States for treatment of irritability in individuals with autism spectrum disorder. Risperidone, a high-potency antipsychotic with combined dopamine (D2) and serotonin (5-HT2) receptor antagonist properties, has been shown to subdue aggressive or self-injurious behaviors in children with and without autism spectrum disorder. Starting with the National Institutes of Health supported Research Units on Pediatric Psychopharmacology randomized controlled trial of risperidone for treating irritability in autism spectrum disorder in 2002, there have been seven randomized controlled trials, three reanalysis studies, and two add-on studies, which have converged to confirm risperidone as an efficacious pharmacological treatment for irritability in children and adolescents with autism spectrum disorder in doses ranging from 0.5 mg to 1.5 mg. Some of the preschoolers in this study were also receiving intensive behavioral treatments. Risperidone is considered the first-line of medication treatment for children and adolescents with autism spectrum disorder who exhibit severe irritability. Despite its efficacy, risperidone’s main side effects of weight gain and increased appetite; metabolic side effects such as hyperglycemia, prolactin elevation, and dyslipidemia; along with other common adverse effects such as fatigue, drowsiness, dizziness, and drooling have limited its use in some individuals. Risperidone should be used with caution in individuals with underlying cardiac abnormalities or hypotension, since risperidone may contribute to orthostatic hypotension. In further continuation studies of risperidone in the treatment of irritability in autism spectrum disorder, persistent efficacy and tolerability were found over a 6-month period, with a rapid return of symptoms in good responders when the risperidone was discontinued. Other drugs studied in the treatment of irritability in autism spectrum disorder include aripiprazole and olanzapine. Two large studies utilizing aripiprazole in the treatment of tantrums, aggression, and self-injury in children and adolescents with autism spectrum disorder found that aripiprazole was both efficacious and safe. Doses ranged from 5 mg to 15 mg per day. Main side effects included sedation, dizziness, insomnia, akathisia, nausea, and vomiting. Although weight gain was not as pronounced as with risperidone, it was still considered a moderate adverse event, with approximately 1.3 to 1.5 kg gained during an 8-week study period. The weight gain was similar at the lower and higher doses. Olanzapine, which specifically blocks 5-HT2A and D2 receptors and also blocks muscarinic receptors, has been studied in children and adolescents with autism spectrum disorder for the treatment of irritability with a trend toward a positive response; however, significant weight gain of approximately 3.5 kg occurred. The main side effect was sedation. Hyperactivity, Impulsivity, and Inattention. Several randomized placebocontrolled trials of methylphenidate have been conducted for the treatment of hyperactivity, impulsivity, and inattention in children and adolescents with autism spectrum disorder. The Research Units of Pediatric Psychopharmacology found methylphenidate to be at least moderately efficacious at doses of 0.25 to 0.5 mg/kg for youth with autism spectrum disorder and ADHD symptoms. Efficacy of methylphenidate in this population was less effective than in children with ADHD without autism spectrum disorder, and children with autism spectrum disorder developed more frequent side effects, including increased irritability, compared to ADHD children. A study of methylphenidate in the treatment of hyperactivity and inattention in preschoolers with autism spectrum disorder found the stimulant safe and relatively efficacious; half of the preschoolers developed side effects including increased stereotypies, gastrointestinal upset, sleep problems, and emotional lability. Among nonstimulants, one double-blind placebo-controlled study of hyperactivity, impulsivity, and inattention using atomoxetine in children with autism spectrum disorder found that it was significantly more effective than placebo. Side effects included sedation, irritability, constipation, and nausea. Clonidine, an α-agonist has also been studied in children with autism spectrum disorder for the treatment of hyperactivity with mixed results. Guanfacine was also found to be of use in some cases. Repetitive and Stereotypic Behavior. These core symptoms of autism spectrum disorder have been studied using selective serotonin reuptake inhibitor (SSRI) antidepressants, second-generation antipsychotics (SGAs), and mood-stabilizing agents such as valproate. One study with fluoxetine found the medication group only slightly better and not significantly better than the placebo group regarding the target symptoms, and another trial with escitalopram found no difference between groups. Risperidone, however, was found to be effective in targeting irritability, and restrictive and repetitive behaviors were improved. One recent study using valproate in a 12-week trial with 55 children with a mean age of 9½ years with autism spectrum disorder, found that those who were considered responders with respect to irritability were also found to be spending less time engaging in repetitive behaviors. Agents Administered For Behavioral Impairment In Autism Spectrum Disorder Based On Open Trials. Quetiapine is an antipsychotic with more potent 5-H2 than D2 receptor blocking properties. Although only open-label trials have been done with this agent, it is sometimes tried when risperidone and olanzapine are not efficacious or well tolerated. It has been used in clinical practice at doses ranging from 50 to 200 mg per day. Adverse effects include drowsiness, tachycardia, agitation, and weight gain. Clozapine has a heterocyclic chemical structure that is related to certain conventional antipsychotics, such as loxapine (Loxitane), although clozapine carries a lower risk of extrapyramidal symptoms. It is not generally used in the treatment of aggression and self-injurious behavior unless those behaviors coexist with psychotic symptoms. The most serious adverse effect is agranulocytosis, which necessitates monitoring white blood cell count weekly during clozapine use. Its use is generally limited to treatment-resistant psychotic patients. Ziprasidone has receptor-blocking properties at the 5-HT2A and D2 receptor sites and carries little risk of extrapyramidal and antihistaminic effects. No guidelines exist for its use in autistic children with aggressive and self-injurious behaviors, but it has been used clinically to treat the latter behaviors in children who are treatment resistant. In studies of its use in adults with schizophrenia, dose ranges of 40 to 160 mg were found to be effective. Adverse effects include sedation, dizziness, and lightheadedness. An electrocardiography (ECG) recording is generally obtained before use of this medication. Lithium (Eskalith) has been shown to be efficacious in children with aggression without autism spectrum disorder, and it is used clinically in the treatment of aggressive or self-injurious behaviors when antipsychotic medications are not effective. Agents Used For Behavioral Impairment In Autism Spectrum Disorder Without Evidence Of Efficacy. A double-blind study investigated the efficacy of amantadine (Symmetrel), which blocks N-methyl-d-aspartate (NMDA) receptors, in the treatment of behavioral disturbance, such as irritability, aggression, and hyperactivity, in children with autism. Some researchers have suggested that abnormalities of the glutamatergic system may contribute to the emergence of autism spectrum disorders. High glutamate levels have been found in children with the formerly labeled Rett syndrome. In the amantadine study, 47 percent of children on amantadine were rated “improved” by their parents, and 37 percent of children on placebo were rated “improved” by parents in irritability and hyperactivity, although this difference was not statistically significant. Investigators rated the children on amantadine “significantly improved” with respect to hyperactivity. A double-blind, placebo-controlled study of the efficacy of the anticonvulsant lamotrigine (Lamictal) on hyperactivity in children with autism showed high rates of placebo improvement in ratings of hyperactivity, which were similar to response on the medication. Clomipramine (Anafranil) has been used in autism spectrum disorder, without RCTs to provide evidence of positive results. Fenfluramine (Pondimin), which reduces blood serotonin levels, has also been used unsuccessfully in the treatment of autism. Improvement does not seem to be associated with a reduction in blood serotonin level. Naltrexone (ReVia), an opioid receptor antagonist, has been investigated without much success, based on the notion that blocking endogenous opioids would reduce autistic symptoms. Tetrahydrobiopterin, a coenzyme that enhances the action of enzymes was used in a double-blind placebo-controlled crossover study of 12 children with autistic disorder and low concentrations of spinal tetrahydrobiopterin. The children received a daily dose of 3 mg tetrahydrobiopterin per kilogram during a 6-month period alternating with placebo. Results indicated small, nonsignificant changes in the total scores on the Childhood Autism Rating Scale after 3- and 6-month treatment. Post hoc analysis of the three core symptoms of autism—social interaction, communication, and stereotyped behaviors— revealed a significant improvement in social interaction score after 6 months of active treatment. A positive correlation was noted between social response and IQ. These results suggest that there is a possible effect of tetrahydrobiopterin on the social functioning of children with autism. A recent case report suggested that low-dose venlafaxine (Effexor) was efficacious in three adolescents and young adults with autistic disorder with self-injurious behavior and hyperactivity. Dose of venlafaxine used was 18.75 mg per day, and efficacy was reported to be sustained over a 6-month period. Complementary and Alternative Medicine (CAM) Approaches to Autism Spectrum Disorder Complementary and alternative medicine (CAM) is a group of nontraditional treatments that are generally used in conjunction with conventional treatments. Safe interventions that have been applied to target both core and associated behavioral features of autism spectrum disorder with unknown efficacy include the following: music therapy, to promote communication and expression; and yoga, to promote attention and decrease activity level. A biologically based practice that is deemed safe and shown to be efficacious is melatonin, which is effective in reducing sleep-onset latency in children. Other biological practices that are recognized as safe but with unknown efficacy include vitamin C, multivitamins, essential fatty acids, and the amino acids carnosine and carnitine. Secretin has been shown to be ineffective in RCTs in the treatment of autism spectrum disorder. DISORDERS INCLUDED IN AUTISTIC SPECTRUM DISORDER The autistic spectrum covers a range of behaviors which, prior to the change in the DSM5 were listed separately, and which are now no longer diagnosed as separate entities in the diagnostic manual. Nevertheless, the descriptive value of these entities remains important, and it may be some time before the disorders described herein disappear from the psychiatric lexicon. In addition, they remain in use in Europe and around the world as useful diagnostic entities and are still coded as separate disorders in ICD-10 as discussed. International Classification of Diseases, Tenth Edition (ICD-10) The classification system used in International Classification of Diseases, Tenth Revision (ICD-10) is not congruent with the revisions made in DSM-5 about autistic disorders. ICD-10 still includes separate designations for Rett syndrome, Childhood Disintegrative Disorder, Asperger’s Disorder, and Pervasive Development Disorder Not Otherwise Specified (Table 31.5-4). The authors of Synopsis believe these subtypes to be clinically useful, and each is described below. The reader should be aware, however, that according to DSM-5, each is subsumed under the rubric of Autism Spectrum Disorder and should so be diagnosed. Table 31.5-4 ICD-10 Diagnostic Criteria for Pervasive Developmental Disorders Rett Syndrome In 1965, Andreas Rett, an Australian physician, identified a syndrome in 22 girls who appeared to have developed normally for at least 6 months followed by devastating developmental deterioration. Rett syndrome is a progressive condition that has its onset after some months of what appears to be normal development. Head circumference is normal at birth and developmental milestones are unremarkable in early life. Between 5 and 48 months of age, generally between 6 months and 1 year, head growth begins to decelerate. Available data indicate a prevalence of 6 to 7 cases of Rett syndrome per 100,000 girls. Originally, it was believed that Rett syndrome occurred only in females, but males with the disorder or syndromes that are very close to this disorder have now been described. Rett syndrome is not fully included within autism spectrum disorder, and if present along with autism spectrum disorder it should be diagnosed as an associated disorder. Etiology. The cause of Rett syndrome is unknown, although the progressive deteriorating course after an initial normal period is compatible with a metabolic disorder. In some patients with Rett syndrome, the presence of hyperammonemia has led to postulation that an enzyme metabolizing ammonia is deficient, but hyperammonemia has not been found in most patients with Rett syndrome. It is likely that Rett syndrome has a genetic basis. It has been seen primarily in girls, and case reports so far indicate complete concordance in monozygotic twins. Diagnosis and Clinical Features. During the first 5 months after birth, infants have age-appropriate motor skills, normal head circumference, and normal growth. Social interactions show the expected reciprocal quality. At 6 months to 2 years of age, however, these children develop progressive encephalopathy with a number of characteristic features. The signs often include the loss of purposeful hand movements, which are replaced by stereotypic motions, such as hand-wringing; the loss of previously acquired speech; psychomotor retardation; and ataxia. Other stereotypical hand movements may occur, such as licking or biting the fingers and tapping or slapping. The head circumference growth decelerates and produces microcephaly. All language skills are lost, and both receptive and expressive communicative and social skills seem to plateau at developmental levels between 6 months and 1 year. Poor muscle coordination and an apraxic gait with an unsteady and stiff quality develop. Associated features include seizures in up to 75 percent of affected children and disorganized EEG findings with some epileptiform discharges in almost all young children with Rett syndrome, even in the absence of clinical seizures. An additional associated feature is irregular respiration, with episodes of hyperventilation, apnea, and breath holding. The disorganized breathing occurs in most patients while they are awake; during sleep, the breathing usually normalizes. Many patients with Rett syndrome also have scoliosis. As the disorder progresses, muscle tone seems to change from an initial hypotonic condition to spasticity to rigidity. Although children with Rett syndrome may live for well over a decade after the onset of the disorder, after 10 years, many patients are wheelchair-bound, with muscle wasting, rigidity, and virtually no language ability. Long-term receptive and expressive communication and socialization abilities remain at a developmental level of less than 1 year. Dana was born as a full-term and healthy baby term after an uncomplicated pregnancy. An amniocentesis had been obtained because of advanced maternal age of 40 years, and findings were normal. At birth, Dana received good Apgar scores and her weight, height, and head circumference were all near the 50th percentile. Her development during the first months of life was unremarkable. At approximately 8 months of age, her development seemed to wane, and her interest in the environment, including the social environment, declined. Dana’s developmental milestones failed to progress, and she became markedly delayed; she was just starting to walk at her second birthday and had no spoken language. Evaluation at that time revealed that head growth had decelerated. Self-stimulatory behaviors emerged, and in addition, marked cognitive and communicative delays were noted on formal testing. Dana began to lose purposeful hand movements and developed unusual stereotypical hand-washing behaviors. By age 6, her EEG was abnormal and abnormal hand movements were prominent. Subsequently, Dana developed truncal ataxia and breath-holding spells, and motor skills further deteriorated. (Adapted from Fred Volkmar, M.D.) Differential Diagnosis. Rett syndrome shares some features with autism spectrum disorder; however, the two disorders have some predictable differences. In Rett syndrome, there is deterioration of developmental milestones, head circumference, and overall growth, whereas in autism spectrum disorder, aberrant development is usually present from early on. In Rett syndrome, specific and characteristic hand motor movements are always present; in autism spectrum disorder hand mannerisms may or may not appear. Poor coordination, ataxia, and apraxia are predictably part of Rett syndrome; however, individuals with autism spectrum disorder may have unremarkable gross motor function. In Rett syndrome, verbal abilities are usually lost completely, whereas in autism spectrum disorder language is widely variable from markedly aberrant to relatively mildly impaired. Respiratory irregularity is characteristic of Rett syndrome, and seizures often appear early. In autistic disorder, no respiratory disorganization is seen, and seizures do not develop in most patients; when seizures do develop, they are more likely in adolescence than in childhood. For autism spectrum disorder that is associated with another neurodevelopmental disorder such as Rett syndrome, the latter disorder is diagnosed in association with autism spectrum disorder. Course and Prognosis. Rett syndrome is progressive, and those individuals who live into adolescence and adulthood function at a cognitive and social level equivalent to that in the first year of life. Treatment. Treatment is symptomatic. Physiotherapy has been beneficial for the muscular dysfunction, and anticonvulsant treatment is usually necessary to control the seizures. Behavior therapy, along with medication, may help control self-injurious behaviors, as it does in the treatment of autistic disorder, and it may help regulate the breathing disorganization. Childhood Disintegrative Disorder The previous diagnosis of childhood disintegrative disorder, now included in autism spectrum disorder, is characterized by marked regression in several areas of functioning after at least 2 years of apparently normal development. Childhood disintegrative disorder, also called Heller’s syndrome and disintegrative psychosis, was described in 1908 as a deterioration over several months of intellectual, social, and language function occurring in 3- and 4-year-olds with previously normal function. After the deterioration, the children closely resembled children with autistic disorder. Epidemiology. Epidemiological data have been complicated by the variable diagnostic criteria used, but childhood disintegrative disorder is estimated to be much less common than the formerly diagnosed autistic disorder. The prevalence has been estimated to occur in about 1 in 100,000 boys. The ratio of boys to girls is estimated to be between 4 and 8 boys to 1 girl. Etiology. The cause of childhood disintegrative disorder is unknown, but it has been associated with other neurological conditions, including seizure disorders, tuberous sclerosis, and various metabolic disorders. Diagnosis and Clinical Features. The diagnosis is made based on features that fit a characteristic age of onset, clinical picture, and course. Cases reported have ranged in onset from ages 1 to 9 years, but in most, the onset is between 3 and 4 years. Whereas previously diagnosed as a separate entity, DSM-5 conceives of childhood disintegrative disorder as a subset of autism spectrum disorder. The onset may be insidious over several months or relatively abrupt, with abilities diminishing in days or weeks. In some cases, a child displays restlessness, increased activity level, and anxiety before the loss of function. The core features of the disorder include loss of communication skills, marked regression of reciprocal interactions, and the onset of stereotyped movements and compulsive behavior. Affective symptoms are common, particularly anxiety, as is the regression of self-help skills, such as bowel and bladder control. To receive the diagnosis, a child must exhibit loss of skills in two of the following areas: language, social or adaptive behavior; bowel or bladder control; play; and motor skills. Abnormalities must be present in both of the following categories: reciprocal social communication skills, and restricted and repetitive behavior. The main neurological associated feature is seizure disorder. Ron’s early history was within normal limits. By age 2, he was speaking in sentences, and his development appeared to be proceeding appropriately. At 3½ years of age, he abruptly exhibited a period of marked behavioral regression shortly after the birth of a sibling. Ron lost previously acquired skills in communication and was no longer toilet trained. Ron became more withdrawn and less interested in social interaction, exhibiting various self-stimulatory behaviors repeatedly. Comprehensive medical examination failed to reveal any conditions that might account for this developmental regression. Behaviorally, Ron exhibited features of autism spectrum disorder. At follow-up at age 12, he spoke only an occasional single word and had severe mental retardation. (Adapted from Fred Volkmar, M.D.) Differential Diagnosis. The differential diagnosis of the formerly diagnosed childhood disintegrative disorder includes receptive and expressive language disorder, mental retardation with behavioral problems, and Rett syndrome. Childhood disintegrative disorder is characterized by the loss of previously acquired development. Before the onset of childhood disintegrative disorder (occurring at 2 years or older), language has usually progressed to sentence formation. This skill is strikingly different from the premorbid history of even high-functioning patients with autistic disorder, in whom language generally does not exceed single words or phrases before diagnosis of the disorder. Once the disorder occurs, however, those with childhood disintegrative disorder are more likely to have no language abilities than are high-functioning patients with autistic disorder. In Rett syndrome, the deterioration occurs much earlier than in childhood disintegrative disorder, and the characteristic hand stereotypies of Rett syndrome do not occur in childhood disintegrative disorder. Course and Prognosis. The course of childhood disintegrative disorder is variable, with a plateau reached in most cases, a progressive deteriorating course in rare cases, and some improvement in occasional cases to the point of regaining the ability to speak in sentences. Most patients are left with at least moderate mental retardation. Treatment. Treatment of childhood disintegrative disorder includes the same components available in the treatment of autistic disorder. Asperger’s Disorder The former diagnosis of Asperger’s disorder is characterized by impairment and oddity of social interaction and restricted interest and behavior. Unlike the former autistic disorder, in Asperger’s disorder there are no significant delays in language or cognitive development. In 1944, Hans Asperger, an Austrian physician, described a syndrome that he named “autistic psychopathy.” His original description of the syndrome described individuals with normal intelligence who exhibit a qualitative impairment in reciprocal social interaction and behavioral oddities without delays in language development. Asperger’s disorder occurs in a wide variety of severities, including cases in which very subtle social cues are missed, but overall social interactions are mastered. Etiology. Asperger’s disorder, a version of autism spectrum disorder, has a complex etiology including genetic contribution and potentially environmental and perinatal contributing factors. Diagnosis and Clinical Features. The clinical features include at least two of the following indications of qualitative social impairment: Markedly abnormal nonverbal communicative gestures, the failure to develop peer relationships at the expected level. Restricted interests and patterns of behavior are present, but when they are subtle, they may not be immediately identified or singled out as different from those of other children. According to DSM-IV-TR, individuals with Asperger’s disorder exhibit no language delay, clinically significant cognitive delay, or adaptive impairment. Currently, the clinical phenotype of Asperger’s disorder is subsumed within the DSM-5 diagnosis of autism spectrum disorder. Jared was an only child. Birth, medical, and family histories were unremarkable. His motor development was slightly delayed, but language milestones were within normal limits. His parents became concerned about him at age 4 when he was enrolled in a nursery school and was noted to have difficulties in peer interaction, joining activities, and following the rules that were so pronounced that he could not continue in the program. In grade school, he was enrolled in regular education classes and was noted to have difficulties making friends and playing sports with the other students, and he often played alone and spent time alone at lunch and recess. His greatest difficulties arose in peer interactions—he was viewed as eccentric and did not seem to understand how to interact with peers. At home, he seemed captivated by watching the weather channel on television, which he insisted on watching and pursued with great interest and intensity. On examination at age 13, Jared had markedly restricted and intense interests and exhibited pedantic and odd patterns of communication with a monotonic voice quality. Psychological testing revealed an IQ within the normal range. Formal communication examination revealed ageappropriate skills in receptive and expressive language but marked impairment in pragmatic language skills. (Adapted from Fred Volkmar, M.D.) Differential Diagnosis. The differential diagnosis includes social anxiety disorder, obsessive-compulsive disorder, and schizoid personality disorder. According to the previous DSM-IV-TR, the most obvious characteristics of Asperger’s disorder compared to autistic disorder are the absence of language delay and dysfunction. The lack of language delay and impaired use of language were previous requirements for Asperger’s disorder; however, social and communication deficits are present. Studies comparing children with Asperger’s disorder and autistic disorder found that children with Asperger’s disorder were more likely to seek social interaction, and due to their awareness of their impairment sought more vigorously to make friends. Although in this subgroup within autism spectrum disorder significant delay in language is not a feature, some delay in the acquisition of language, and some impairment in verbal communication has been noted in more than one third of clinical samples. Course and Prognosis. The factors associated with a good prognosis in this subgroup within autism spectrum disorder are a normal IQ and more competencies in social skills. Reports of some adults diagnosed with Asperger’s disorder indicate that their social and communication deficits remain and they continue to relate in an awkward way and appear socially uncomfortable. Treatment. Treatment of individuals who meet the criteria for the previous Asperger’s disorder diagnosis aims to promote social communication and peer relationships. Interventions are initiated with the goal of shaping interactions so that they better match those of peers. Very often children with Asperger’s disorder are highly verbal and have excellent academic achievement. The tendency of children and adolescents with Asperger’s disorder to rely on rigid rules and routines can become a source of difficulty for them and be an area that requires therapeutic intervention. A comfort with routines, however, can be utilized to foster positive habits that may enhance the social life of a child with Asperger’s disorder. Self-sufficiency and problemsolving techniques are often helpful for these individuals in social situations and work settings. Some of the same techniques used for autistic disorder are likely to benefit patients with Asperger’s disorder with severe social impairment. Pervasive Developmental Disorder Not Otherwise Specified Whereas the DSM-IV-TR defines pervasive disorder not otherwise specified as a condition with severe, pervasive impairment in communication skills or the presence of restricted and repetitive activities and associated impairment in social interactions, DSM-5 conceives of this as encompassed within a diagnosis of autism spectrum disorder. Anna was the older of two children. She had been a difficult baby who was not easy to console but her motor and communicative development seemed appropriate. She was socially related and sometimes enjoyed interaction, but she was easily overstimulated. She exhibited some hand flapping behavior, especially when she was excited. Anna’s parents sought evaluation when she was 4 years of age because of problems with getting along with other children. At evaluation Anna was found to have language and cognitive function within the normal range. Anna had difficulty relating to her parents as sources of support and comfort. She displayed behavioral rigidity and a tendency to impose routines on social skills. Anna was placed in a special education kindergarten and did well academically, although problems in peer interactions and unusual affective responses persisted. As an adolescent, Anna describes herself as a “loner,” who often retreats from others and avoids social interaction and tends to be comfortable with solitary activities. (Adapted from Fred Volkmar, M.D.) Treatment. 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Eur Neuropsychopharmacol. 2011;21:600–620. 31.6 Attention Deficit/Hyperactivity Disorder ATTENTION-DEFICIT/HYPERACTIVITY DISORDER Attention-deficit/hyperactivity disorder (ADHD) is a neuropsychiatric condition affecting preschoolers, children, adolescents, and adults around the world, characterized by a pattern of diminished sustained attention, and increased impulsivity or hyperactivity. Based on family history, genotyping, and neuroimaging studies, there is clear evidence to support a biological basis for ADHD. Although multiple regions of the brain and several neurotransmitters have been implicated in the emergence of symptoms, dopamine continues to be a focus of investigation regarding ADHD symptoms. The prefrontal cortex of the brain has been implicated because of its high utilization of dopamine and its reciprocal connections with other brain regions involved in attention, inhibition, decision-making, response inhibition, working memory, and vigilance. ADHD affects up to 5 to 8 percent of school-aged children, with 60 to 85 percent of those diagnosed as children continuing to meet criteria for the disorder in adolescence, and up to 60 percent continuing to be symptomatic into adulthood. Children, adolescents, and adults with ADHD often have significant impairment in academic functioning as well as in social and interpersonal situations. ADHD is frequently associated with comorbid disorders including learning disorders, anxiety disorders, mood disorders, and disruptive behavior disorders. The Fifth Edition of the American Psychiatric Association’s Diagnostic and Statistical Manual of Mental Disorders (DSM5) has made several changes to the diagnostic criteria of ADHD in youth and in adults. Whereas in the past, ADHD symptoms had to be present by age 7 years, in DSM-5, “several inattentive or hyperactive-impulsive symptoms” must be present by age 12 years. Previously, there were two subtypes: Inattentive and Hyperactive/Impulsive type. In DSM-5, however, subtypes have been replaced by the following three specifiers, which essentially denote the same groups: (1) combined presentation, (2) predominantly inattentive presentation, and (3) predominantly hyperactive/impulsive presentation. Additional changes in DSM-5 include permitting a comorbid ADHD and autism spectrum diagnosis to be made. Finally, in DSM-5, for adolescents 17 years and older and for adults, only five symptoms, rather than six symptoms of either inattention or hyperactivity and impulsivity are required. In addition, to reflect the developmental differences in ADHD across the life span, examples of symptoms have been added to the DSM-5 criteria for ADHD. To confirm a diagnosis of ADHD, impairment from inattention and/or hyperactivity and impulsivity must be present in at least two settings and interfere with developmentally appropriate social or academic functioning. For current DSM-5 changes see Table 31.6-1. Table 31.6-1 DSM-5 Diagnostic Criteria for Attention-Deficit/Hyperactivity Disorder ADHD has historically been described in the literature using different terminology. In the early 1900s, impulsive, disinhibited, and hyperactive children—many of whom also had neurological damage due to encephalitis—were grouped under the label hyperactive syndrome. In the 1960s, a heterogeneous group of children with poor coordination, learning disabilities, and emotional lability, but without specific neurological disorders, were described as having “minimal brain damage”; however, over time, it became clear that this was an inappropriate term. Many hypotheses have been suggested to explain ADHD symptoms including theories of abnormal arousal and poor ability to modulate emotions. This theory was initially supported by the observation that stimulant medications increased sustained attention and improved focus. ADHD is one of the most well-researched childhood psychiatric disorders with strong evidence-based treatments. Epidemiology Rates of ADHD have been reported to be 7 to 8 percent in prepubertal elementary school children. Epidemiologic studies suggest that ADHD occurs in about 5 percent of youth including children and adolescents, and about 2.5 percent of adults. The rate of ADHD in parents and siblings of children with ADHD is 2 to 8 times greater than in the general population. ADHD is more prevalent in boys than in girls, with the ratio ranging from 2:1 to as high as 9:1. First-degree biological relatives (e.g., siblings of probands with ADHD) are at high risk for developing ADHD as well as other psychiatric disorders, including disruptive behavior disorders, anxiety disorders, and depressive disorders. Siblings of children with ADHD are also at higher risk than the general population for learning disorders and academic difficulties. The parents of children with ADHD show an increased incidence of substance use disorders. Symptoms of ADHD are often present by age 3 years, but unless they are very severe, the diagnosis is frequently not made until the child is in kindergarten, or elementary school, when teacher information is available comparing the index child peers of the same age. Etiology Data suggest that the etiology of ADHD is largely genetic, with a heritability of approximately 75 percent. ADHD symptoms are the product of complex interactions of neuroanatomical and neurochemical systems evidenced by data from twin and adoption family genetic studies, dopamine transport gene studies, neuroimaging studies, and neurotransmitter data. Most children with ADHD have no evidence of gross structural damage in the central nervous system (CNS). In some cases, contributory factors for ADHD may include prenatal toxic exposures, prematurity, and prenatal mechanical insult to the fetal nervous system. Food additives, colorings, preservatives, and sugar have been proposed as possible contributing causes of hyperactive behavior; however, studies have not confirmed these theories. Neither artificial food coloring nor sugar have been established as causes of ADHD. There is no clear evidence that omega-3 fatty acids are beneficial in the treatment of ADHD. Genetic Factors. Evidence for a significant genetic contribution to ADHD has emerged from family studies, which reveal an increased concordance in monozygotic compared to dizygotic twins, as well as a marked increased risk of 2 to 8 times for siblings as well as parents of an ADHD child, compared to the general population. Clinically, one sibling may have predominantly impulsivity/hyperactivity symptoms and others may have predominantly inattention symptoms. Up to 70 percent of children with ADHD meet criteria for a comorbid psychiatric disorder, including learning disorders, anxiety disorders, mood disorder conduct disorders, and substance use disorders. Several hypotheses of the mode of transmission of ADHD have been proposed, including a sex-linked hypothesis, which would explain the significantly increased rates of ADHD in males. Other theories have focused on a model of interaction of multiple genes that produces the various symptoms of ADHD. Numerous investigations continue to identify specific genes involved in ADHD. Cook and colleagues have found an association of the dopamine transporter gene (DAT1) with ADHD, although data from other research groups have not confirmed that result. Family studies and populationbased studies have found an association between the dopamine 4 receptor seven-repeat allele gene (DRD4) gene and ADHD. Most molecular research on ADHD has focused on genes that influence the metabolism or action of dopamine. Continued investigation is necessary to clarify the complex relationships between multiple interactive genes and the emergence of ADHD. Neurochemical Factors. Many neurotransmitters are postulated to be associated with ADHD symptoms; however, dopamine is a major focus of clinical investigation, and the prefrontal cortex has been implicated based on its role in attention and regulation of impulse control. Animal studies have shown that other brain regions such as locus ceruleus, which consists predominantly of noradrenergic neurons, also play a major role in attention. The noradrenergic system includes the central system (originating in the locus ceruleus) and the peripheral sympathetic system. Dysfunction in peripheral epinephrine, which causes the hormone to accumulate peripherally, may potentially feed back to the central system and “reset” the locus ceruleus to a lower level. In part, hypotheses regarding the neurochemistry of ADHD have arisen from the predictable effect of medications. Simulants, known to be the most effective medications in the treatment of ADHD, affect both dopamine and norepinephrine, leading to neurotransmitter hypotheses that may include dysfunction in both the adrenergic and dopaminergic systems. Stimulants increase catecholamine concentrations by promoting their release and blocking their uptake. Neurophysiological Factors. EEG studies in ADHD children and adolescents over the last several decades have found evidence of increased theta activity, especially in the frontal regions. Further studies of youth with ADHD have provided data showing elevated beta activity in their electroencephalography (EEG) studies. Clarke and colleagues, studying EEG findings in children and adolescents over the last two decades found that those ADHD children with combined type of ADHD were the ones who showed significantly elevated beta activity on EEG, and further studies indicate that these youth also tend to show increased mood lability and temper tantrums. Current investigation of EEG in youth with ADHD have identified behavioral symptom clusters among children with similar EEG profiles. Neuroanatomical Aspects. Researchers have hypothesized networks within the brain for promoting components of attention including focusing, sustaining attention, and shifting attention. They describe neuroanatomical correlations for the superior and temporal cortices with focusing attention; external parietal and corpus striatal regions with motor executive functions; the hippocampus with encoding of memory traces; and the prefrontal cortex with shifting from one stimulus to another. Further hypotheses suggest that the brainstem, which contains the reticular thalamic nuclei function, is involved in sustained attention. A review of magnetic resonance imaging (MRI), positron emission tomography (PET), and single photon emission computerized tomography (SPECT) suggests that populations of children with ADHD show evidence of both decreased volume and decreased activity in prefrontal regions, anterior cingulated, globus pallidus, caudate, thalamus, and cerebellum. PET scans have also shown that female adolescents with ADHD have globally lower glucose metabolism than both control female and male adolescents without ADHD. One theory postulates that the frontal lobes in children with ADHD do not adequately inhibit lower brain structures, an effect leading to disinhibition. Developmental Factors. Higher rates of ADHD are present in children who were born prematurely and whose mothers were observed to have maternal infection during pregnancy. Perinatal insult to the brain during early infancy caused by infection, inflammation, and trauma may, in some cases, be contributing factors in the emergence of ADHD symptoms. Children with ADHD have been observed to exhibit nonfocal (soft) neurological signs at higher rates than those in the general population. Reports in the literature indicate that September is a peak month for births of children with ADHD with and without comorbid learning disorders. The implication is that prenatal exposure to winter infections during the first trimester may contribute to the emergence of ADHD symptoms in some susceptible children. Psychosocial Factors. Severe chronic abuse, maltreatment, and neglect are associated with certain behavioral symptoms that overlap with ADHD including poor attention and poor impulse control. Predisposing factors may include the child’s temperament and genetic–familial factors. Diagnosis The principal signs of inattention, impulsivity, and hyperactivity may be elicited on the basis of a detailed history of a child’s early developmental patterns along with direct observation of the child, especially in situations that require sustained attention. Hyperactivity may be more severe in some situations (e.g., school) and less marked in others (e.g., one-on-one interviews), and may be less obvious in pleasant structured activities (sports). The diagnosis of ADHD requires persistent, impairing symptoms of either hyperactivity/impulsivity or inattention in at least two different settings. For example, most children with ADHD have symptoms in school and at home. The diagnostic criteria for ADHD are outlined in Table 31.6-1. Distinguishing features of ADHD are short attention span and high levels of distractibility for chronological age and developmental level. In school, children with ADHD often exhibit difficulties following instructions and require increased individualized attention from teachers. At home, children with ADHD frequently have difficulty complying with their parents’ directions and may need to be asked multiple times to complete relatively simple tasks. Children with ADHD typically act impulsively, are emotionally labile, explosive, lack focus, and are irritable. Children for whom hyperactivity is a predominant feature are more likely to be referred for treatment earlier than are children whose primarily symptoms are attention deficit. Children with the combined inattentive and hyperactive-impulsive symptoms of ADHD, or predominantly hyperactive-impulsive symptoms of ADHD, are more apt to have a stable diagnosis over time and to exhibit comorbid conduct disorder than those children with inattentive ADHD. Specific learning disorders in the areas of reading, arithmetic, language, and writing occur frequently in association with ADHD. Global developmental assessment must be considered to rule out other sources of inattention. School history and teachers’ reports are critical in evaluating whether a child’s difficulties in learning and school behavior are caused primarily by inattention or compromised understanding of the academic material. In addition to intellectual limitations, poor performance in school may result from maturational problems, social rejection, mood disorders, anxiety, or poor self-esteem due to learning disorders. Assessment of social relationships with siblings, peers, and adults, and engagement in free and structured activities may yield valuable diagnostic clues to the presence of ADHD. The mental status examination in a given child with ADHD who is aware of his or her impairment may reflect a demoralized or depressed mood; however, thought disorder or impaired reality testing is not expected. A child with ADHD may exhibit distractibility and perseveration and signs of visual-perceptual, auditory-perceptual, or languagebased learning disorders. A neurological examination may reveal visual, motor, perceptual, or auditory discriminatory immaturity or impairments without overt signs of visual or auditory disorders. Children with ADHD often have problems with motor coordination and difficulty copying age-appropriate figures, rapid alternating movements, right–left discrimination, ambidexterity, reflex asymmetries, and a variety of subtle nonfocal neurological signs (soft signs). If there are indications of possible absence spells, clinicians should obtain a neurological consultation and an EEG to rule out seizure disorders. A child with an unrecognized temporal lobe seizure focus may have behavior disturbances, which can resemble those of ADHD. Clinical Features ADHD can have its onset in infancy, although it is rarely recognized until a child is at least toddler age. More commonly, infants with ADHD are active in the crib, sleep little, and cry a great deal. In school, children with ADHD may attack a test rapidly, but may answer only the first two questions. They may be unable to wait to be called on in school and may respond before everyone else. At home, they cannot be put off for even a minute. Impulsiveness and an inability to delay gratification are characteristic. Children with ADHD are often susceptible to accidents. The most cited characteristics of children with ADHD, in order of frequency, are hyperactivity, attention deficit (short attention span, distractibility, perseveration, failure to finish tasks, inattention, poor concentration), impulsivity (action before thought, abrupt shifts in activity, lack of organization, jumping up in class), memory and thinking deficits, specific learning disabilities, and speech and hearing deficits. Associated features often include perceptual motor impairment, emotional lability, and developmental coordination disorder. A significant percent of children with ADHD show behavioral symptoms of aggression and defiance. School difficulties, both learning and behavioral, commonly exist with ADHD. Comorbid communication disorders or learning disorders that hamper the acquisition, retention, and display of knowledge complicate the course of ADHD. Justin was a 9-year-old African American adopted boy who was referred for an evaluation by his 4th grade teacher, who informed his adoptive parents that she was unable to manage Justin’s impulsive and aggressive behaviors in the classroom. Justin was attending public school and was in a regular classroom with two resource room periods per day to help him with reading and math. Justin also received speech therapy once a week. Justin had been referred in the past for psychiatric evaluation, but his adoptive parents were opposed to medication so they did not follow through. Justin’s adoptive parents knew very little about his biological family other than that his biological mother was known to be a polydrug abuser and was currently incarcerated. Justin was adopted as an infant and his pediatrician had told his adoptive parents that Justin was entirely healthy at birth. However, ever since kindergarten, Justin’s teachers had complained that Justin did “not seem to listen,” had “poor concentration,” and was unable to stay in his seat. Because Justin was an engaging and cute child, his teachers in kindergarten and first grade made accommodations for him in their classrooms despite their complaints. When Justin entered the 2nd grade, however, it became clear that he was struggling with reading and writing, and an individualized educational program (IEP) evaluation was initiated. Justin was provided with resource room periods for remediation during the school day, but Justin continued to have additional problems getting along with his peers during lunch, and even at recess. Justin was often found arguing or fighting with other children who said that he did not know the rules of their games. Justin became angry when he was criticized by his peers and would often push his classmates. At home, Justin’s adoptive parents were becoming more and more frustrated with Justin because he seemed to take hours to do a few math problems, and was unable to write a paragraph without a lot of help. Justin would become easily annoyed when frustrated with himself and then run around the house in a silly and disruptive manner. Justin was a good-hearted child who seemed to get along best with children who were younger than he was. Justin did not seem to make any close friends among his classmates, and the teachers indicated that Justin’s peers sometimes avoided him because he was too rough during play and he did not follow the rules of their games. Justin had a difficult time waiting his turn and he became easily provoked when he was reprimanded. Consequently, Justin became alienated and often bullied by his classmates. Justin was aware that he was not able to keep up with the classwork, and he told his adoptive parents that he was just “stupid.” Although Justin acted in a rambunctious and impulsive manner, he also appeared sad, and one day after a fight with several peers, he told his adoptive parents that he was going to “kill” himself. At this point, Justin’s parents became worried and decided that Justin’s teacher was right, and they would seek a psychiatric evaluation for Justin. During the initial evaluation with a child and adolescent psychiatrist, Justin was found to be a well-developed, cute, and active child, who appeared distracted and fidgety and somewhat sad. When asked about it, Justin said that he wanted to do “better” in school but that nobody liked him, he was failing his classes, and that he didn’t like doing homework. He denied suicidal thoughts and reported that he had only said that to his parents because he was angry at his peers. Justin admitted that it was very difficult for him to understand his school work and impossible to complete his assignments. During the evaluation, several parent and teacher rating scales were obtained. These included The Child Behavior Checklist, and the SNAP Rating Scale. Justin’s teacher and parents endorsed similar symptoms including poor organization, inability to follow directions, being forgetful in daily activities, impulsivity, with several episodes of running into the street without looking, blurting things out in the classroom without raising his hand, and recurrent fights with peers. Justin was observed to look dejected in school when he was excluded from play activities by peers, and sullen or angry at home when he was asked to read or do homework. Based on the clinical history, the rating scales and teacher’s report, a diagnosis of Attention-Deficit/Hyperactivity Disorder, with the DSM-5 specifier of combined presentation, was made. In addition, Justin was noted to have a mood disorder with depressed mood, which did not qualify for a major depression. A treatment plan was suggested including a behavioral plan allowing Justin to receive rewards for effort on his homework along with a trial of a stimulant medication. After an extensive medical history was obtained and a recent physical examination by his pediatrician did not reveal any systemic illnesses, an EEG was decided upon, mainly because it was not possible to obtain a full medical and cardiac history due to an absence of early medical records, and his adoptive parents did not have access to his birth and neonatal medical records. After obtaining a normal EEG, Justin was started on a trial dose of a short-acting stimulant, methylphenidate (Ritalin) at 10 mg, to determine if he could tolerate a stimulant without any unexpected sensitivities. Justin had no adverse effects and was shortly switched to the long-acting stimulant Concerta, 36 mg which would last between 10 and 12 hours. Justin became more vigilant in class and seemed to be less restless and more focused, and his teacher reported that he was not getting out of his seat as often, although he continued to blurt out in class when he was not called on, and he continued to have difficulty following directions and forgetting things. Because Justin was not experiencing any adverse effects and was still displaying some ADHD symptoms, his Concerta was increased to 54 mg per day. At this dose both his teacher and parents noticed a marked improvement in his ability to sit and finish his classwork and homework. However, he began to have significant problems with insomnia, and was becoming fatigued from not being able to fall asleep until about 2 a.m. on a nightly basis. The child and adolescent psychiatrist and Justin’s parents discussed two options to address the insomnia. One was to add a dose of short-acting clonidine in the evenings to cause a calming effect along with some sedative properties, and the other was to initiate a trial of Daytrana, the methylphenidate transdermal patch, which could be applied to deliver a similar dose of methylphenidate throughout the day, and the patch could be removed at approximately 4 pm or 5 pm to determine which produced the desired effect for the target symptoms for the most optimal amount of time. Because the Daytrana patch may deliver medication for an hour or so after its removal, Justin would need to try several different removal times to find the optimal treatment time. Justin’s family and his child psychiatrist determined that it would be the best next step for Justin to try the Daytrana patch rather than add an additional medication to treat his insomnia. Justin was tried on the Daytrana transdermal 20 mg patch and found that if it was removed by 5 p.m., he was able to fall asleep within 30 to 45 minutes after getting into bed. Despite some mild erythema around the site of the patch, Justin experienced no other side effects and was glad that he did not have to take pills each morning. It was determined by Justin’s parents, teachers, and child and adolescent psychiatrist that Justin’s ADHD symptoms were now under much improved control. Justin began to receive better grades and his self-esteem was noticeably increased. However, Justin still had difficulties with peers and felt that he wasn’t making as many friends as he wanted. Justin’s child psychiatrist suggested that Justin be placed in a weekly social skills group that was led by a psychologist who had experience with group interventions for children with ADHD. This was arranged, and although Justin, at first, did not want to attend, after a few sessions, in which Justin was praised for appropriate interactions with peers within his group, Justin decided that he liked the group, and over time, even invited a few of his peers from the group to his home to play. The combination of the medication and the social skills group resulted in a significant improvement in Justin’s ADHD symptoms as well as in the quality of his relationships with peers and even his family. (Adapted from Greenhill LL, Hechtman LI. Attention-Deficit/Hyperactivity Disorder In: Sadock BJ, Sadock VA, Ruiz P, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Vol. 2. Philadelphia: Lippincott Williams & Wilkins; 2009:3571.) Pathology and Laboratory Examination A child being evaluated for ADHD should receive a comprehensive psychiatric and medical history. Prenatal, perinatal, and toddler information should be included in the history. Complications of mother’s pregnancy should also be obtained. Medical problems that may produce symptoms overlapping with ADHD include petit mal epilepsy, hearing and visual impairments, thyroid abnormalities, and hypoglycemia. A thorough cardiac history should be taken, including an investigation of the lifetime history of syncope, family history of sudden death, and a cardiac examination of the child. Although it is reasonable to obtain an electrocardiography (ECG) study prior to treatment, if any cardiac risk factors are present, a cardiology consultation and examination are warranted. No specific laboratory measures are pathognomonic of ADHD. A continuous performance task, a computerized task in which a child is asked to press a button each time a particular sequence of letters or numbers is flashed on a screen, is not specifically a useful diagnostic tool for ADHD; however, it may be useful in comparing a child’s performance before and after medication treatment, particularly at different doses. Children with poor attention tend to make errors of omission—that is, they fail to press the button when the sequence has flashed. Impulsivity is often manifested by errors of commission, in which an impulsive child cannot resist pushing the button, even when the desired sequence has not yet appeared on the screen. Differential Diagnosis A temperamental constellation of high activity level and short attention span, in the normal range for the child’s age, and without impairment, should be ruled out. Differentiating these temperamental characteristics from the cardinal symptoms of ADHD before the age of 3 years is difficult, mainly because of the overlapping features of a normally immature nervous system and the emerging signs of visual-motorperceptual impairments frequently seen in ADHD. Anxiety in a child needs to be evaluated. Anxiety can accompany ADHD as a symptom or comorbid disorder, and anxiety can manifest with overactivity and easy distractibility. It is not uncommon for a child with ADHD to become demoralized or, in some cases, to develop depressive symptoms in reaction to persistent frustration with academic difficulties and resulting low self-esteem. Mania and ADHD share many core features, such as excessive verbalization, motoric hyperactivity, and high levels of distractibility. In addition, in children with mania, irritability seems to be more common than euphoria. Although mania and ADHD can coexist, children with bipolar I disorder exhibit more waxing and waning of symptoms than those with ADHD. Recent follow-up data for children who met the criteria for ADHD and subsequently developed bipolar disorder suggest that certain clinical features occurring during the course of ADHD predict future mania. Children with ADHD who had developed bipolar I disorder at 4year follow-up had a greater co-occurrence of additional disorders and a greater family history of bipolar disorders and other mood disorders than children without bipolar disorder. Frequently, oppositional defiant disorder, or conduct disorder and ADHD may coexist, and when that occurs, both disorders are diagnosed. Specific learning disorders of various kinds must also be distinguished from ADHD; a child may be unable to read or do mathematics because of a learning disorder, rather than because of inattention. ADHD often coexists with one or more learning problems, including deficits in reading, mathematics or written expression. Course and Prognosis The course of ADHD is variable. Symptoms have been shown to persist into adolescence in 60 to 85 percent of cases, and into adult life in approximately 60 percent of cases. The remaining 40 percent of cases may remit at puberty, or in early adulthood. In some cases, the hyperactivity may disappear, but the decreased attention span and impulsecontrol problems persist. Overactivity is usually the first symptom to remit, and distractibility is the last. ADHD does not usually remit during middle childhood. Persistence is predicted by a family history of the disorder, negative life events, and comorbidity with conduct symptoms, depression, and anxiety disorders. When remission occurs, it is usually between the ages of 12 and 20. Remission can be accompanied by a productive adolescence and adult life, satisfying interpersonal relationships, and few significant sequelae. Most patients with the disorder, however, undergo partial remission and are vulnerable to antisocial behavior, substance use disorders, and mood disorders. Learning problems often continue throughout life. In about 60 percent of cases, some symptoms persist into adulthood. Those who persist with the disorder may show diminished hyperactivity but remain impulsive and accident-prone. Although the educational attainments of people with ADHD as a group are lower than those of people without ADHD, early employment histories do not differ from those of people with similar educations. Children with ADHD whose symptoms persist into adolescence are at higher risk for developing conduct disorder. Children with both ADHD and conduct disorder are also at risk for developing substance use disorders. The development of substance abuse disorders among ADHD youth in adolescence appears to be more related to the presence of conduct disorder rather than to ADHD. Most children with ADHD have some social difficulties. Socially dysfunctional children with ADHD have significantly higher rates of comorbid psychiatric disorders, and experience more problems with behavior in school as well as with peers and family members. Overall, the outcome of ADHD in childhood seems to be related to the degree of persistent comorbid psychopathology, especially conduct disorder, social disability, and chaotic family factors. Optimal outcomes may be promoted by ameliorating children’s social functioning, diminishing aggression, and improving family situations as early as possible. Treatment Pharmacotherapy. Pharmacologic treatment is considered the first line of treatment for ADHD. Central nervous system stimulants are the first choice of agents in that they have been shown to have the greatest efficacy with generally mild tolerable side effects. Stimulants are contraindicated in children, adolescents, and adults with known cardiac risks and abnormalities. In medically healthy youth, however, excellent safety records are documented for short- and sustained-release preparations of methylphenidate (Ritalin, Ritalin-SR, Concerta, Metadate CD, Metadate ER), dextroamphetamine (Dexedrine, Dexedrine spansules, Vyvanse), and dextroamphetamine and amphetamine salt combinations (Adderall, Adderall XR). Newer preparations of methylphenidate, include Methylin, a chewable form of methylphenidate; Daytrana, a methylphenidate patch; and dexmethylphenidate, the denantiomer (Focalin), and its longer acting form Focalin XR. These newer preparations aim to maximize the target effects and minimize the adverse effects in individuals with ADHD who obtain partial response from methylphenidate or whose dose was limited by side effects. Vyvanse (lisdexamfetamine dimesylate) is a pro-drug of dextroamphetamine, which requires intestinal metabolism in order to reach its active form. Vyvanse is approved by the U.S. Food and Drug Administration (FDA) for children 6 years and older. Vyvanse, inactive until it is metabolized, is a less likely agent to have risks of abuse or overdose. It has side effects and efficacy similar to the other forms of amphetamines used in the treatment of ADHD. Current strategies favor once a day sustained-release stimulant preparations for their convenience and diminished rebound side effects. Advantages of the sustained-release preparations for children are that one dose in the morning will sustain the effects all day, and the child is no longer required to interrupt his or her school day, as well as the physiologic advantage that the medication is sustained at an approximately even level in the body throughout the day so that periods of rebound and irritability are avoided. Table 31.6-2 contains comparative information on the above medications. Table 31.6-2 Stimulant Medications in the Treatment of Attention-Deficit/Hyperactivity Disorder (ADHD) Nonstimulant medications approved by the FDA in the treatment of ADHD include atomoxetine (Strattera), a norepinephrine uptake inhibitor. Unlike the stimulants, Strattera carries with it a black box warning for potential increases in suicidal thoughts or behaviors and requires children with ADHD to be monitored for these symptoms, similarly to children who are administered antidepressants. A-agonists including clonidine (Catapres) and guanfacine (Tenex) have also been found to be effective in treating ADHD. The FDA has recently approved the extended-release forms of clonidine (Kapvay) and the extended release form of guanfacine (Intuniv) for the treatment of ADHD in children 6 years and older. Antidepressants, such as bupropion (Wellbutrin, Wellbutrin SR), have been used with variable success in the treatment of ADHD. (Table 31.6-3 contains comparative information on the nonstimulant medications and Table 31.6-4 indicates FDA-approved ages for ADHD medications.) Table 31.6-3 Nonstimulant Medications for Attention-Deficit/Hyperactivity Disorder (ADHD) Table 31.6-4 FDA Approval for ADHD Medications STIMULANT MEDICATIONS. Methylphenidate and amphetamine preparations are dopamine agonists; however, the precise mechanism of the stimulant’s central action remains unknown. Methylphenidate preparations have been shown to be highly effective in up to three fourths of children with ADHD, with relatively few adverse effects. Concerta, the 10- to 12-hour extended-release OROS (osmotic controlled-release extended delivery system) form of methylphenidate, is administered once daily in the morning and is effective during school hours as well as after school during the afternoon and early evening. Both shorter forms of methylphenidate and Concerta have similar common adverse effects including headaches, stomachaches, nausea, and insomnia. Some children experience a rebound effect, in which they become mildly irritable and appear to be slightly hyperactive for a brief period when the medication wears off. In children with a history of motor tics, some observations must be made as, in some cases, methylphenidate can exacerbate the tics, whereas in other children the tics are unaffected or even improved. Because tics wax and wane, it is important to observe their patterns over some time. Another common concern about use of methylphenidate preparations over long periods is potential growth suppression. During periods of use, methylphenidate is associated with slightly decreased rates of growth, and if used over many years continuously without any drug holidays growth suppression of about several centimeters has been noted. When given “drug holidays” on weekends or summers, children tend to eat more and also make up the growth. The methylphenidate products have been shown to improve ADHD children’s scores on tasks of vigilance, such as on math calculation tests, the continuous performance task, and paired associations. Daytrana, a transdermal delivery system designed to release methylphenidate continuously on application of the patch to the skin, has been developed and approved for use in children and adolescents. Advantages of Daytrana include an alternative for children who have difficulties swallowing pills, and that the patch can individualize how many hours per day a given child with ADHD is receiving medication. This is important because a child with ADHD who needs the medication in the late afternoons to do homework but develops insomnia if the medication is still present after dinner, is able to remove the patch at the desired time. Thus, an individualized delivery time may be provided for each child by virtue of how many hours the patch is left on the skin. This is in contrast to oral sustained-release forms of methylphenidate, such as Concerta, in which the release time continues for 12 hours after the pill is swallowed. A double-blind randomized study in children with ADHD who wore the methylphenidate patch for 12 hours at a time, showed efficacy of the patch preparation doses ranging from patches delivering 0.45 mg per hour to 1.8 mg per hour of methylphenidate. The effectiveness of the patch reached a plateau without much further improvement as dose was increased, but intensive behavioral interventions were also being administered. A delay in the onset of the transdermal medication effect was approximately an hour. Side effects were similar to oral preparations of methylphenidate. Approximately half of the children exhibited at least minor erythematous reactions to the patch; however, these side effects are usually well tolerated by children on the patch. Dextroamphetamine and dextroamphetamine/amphetamine salt combinations are usually the second drugs of choice when methylphenidate is not effective. Vyvanse is advantageous because it is inactive until it is metabolized. NONSTIMULANT MEDICATIONS. Atomoxetine HCl (Strattera) is a norepinephrine uptake inhibitor approved by the FDA for the treatment of ADHD in children age 6 years and older. The mechanism of action is not well understood, but it is believed to involve selective inhibition of presynaptic norepinephrine transporter. Atomoxetine is well absorbed by the gastrointestinal tract, and maximal plasma levels are reached in 1 to 2 hours after ingestion. It has been shown to be effective for inattention as well as impulsivity in children and in adults with ADHD. Its half-life is approximately 5 hours and it is usually administered twice daily. Most common side effects include diminished appetite, abdominal discomfort, dizziness, and irritability. In some cases, increases in blood pressure and heart rate have been reported. Atomoxetine is metabolized by the cytochrome P450 (CYP) 2D6 hepatic enzyme system. A small fraction of the population are poor metabolizers of CYP 2D6–metabolized drugs and, for those individuals, plasma concentrations of the drug may rise as much as fivefold for a given dose of medication. Drugs that inhibit CYP 2D6, including fluoxetine, paroxetine, and quinidine, may lead to increased plasma levels of this medication. Despite its short half-life, atomoxetine has been shown in a recent study to be effective in reducing symptoms of ADHD in children during the school day when administered once daily. Another recent study of a combination of atomoxetine alone and combined with fluoxetine in the treatment of 127 children with ADHD and symptoms of anxiety or depression suggested that atomoxetine alone can lead to improvements in mood and anxiety. Children who received combined atomoxetine and fluoxetine experienced greater increases in blood pressure and pulse than those who were treated with atomoxetine only. α-Agonists, short-acting, and the extended-release forms of clonidine hydrochloride (Kapvay) and Guanfacine (Intuniv) are FDA approved for the treatment of ADHD in children and adolescents from 6 to 7 years of age. Kapvay, a centrally acting α-2adrenergic receptor agonist is believed to exert its effect on the prefrontal cortex, although the mechanism of action is unknown. Kapvay is available in 0.1 mg and 0.2 mg tablets, and is generally used twice daily, once in the morning and once at night, to provide a round-the-clock effect. Kapvay is initiated at 0.1 mg at bedtime and can be increased in increments of 0.1 mg at weekly intervals. The maximal dose recommended is 0.2 mg twice daily. Kapvay is not used interchangeably with the short-acting clonidine. Because Kapvay is an antihypertensive agent, it causes a decrease in blood pressure and heart rate. These vital signs must be monitored in patients, especially during initiation and titration of the dose. Common side effects include somnolence, headache, upper abdominal pain, and fatigue. When Kapvay is tapered, it is recommended to taper no more than 0.1 mg every 3 to 7 days. Intuniv, the extended release preparation of guanfacine, is a once-a-day medication for children between 6 and 17 years of age, available in 1 mg, 2 mg, 3 mg, and 4 mg tabs. Intuniv is a tablet that is swallowed whole, and should be taken with water, milk or other liquids; it is not recommended to take Intuniv with a high-fat meal. Intuniv is typically initiated as a 1 mg tab daily and titrated by 1 mg per day at 1-week intervals. The maximum dose approved is 4 mg per day. As a monotherapy, improvement in ADHD symptoms have been found to occur at 0.05 to 0.08 mg/kg once daily. As an adjunctive treatment, optimal doses are reported to range from 0.05 to 0.12 mg/kg/day. Common side effects for Intuniv include somnolence, sedation, fatigue, nausea, hypotension, insomnia, and dizziness. Heart rate and blood pressure must be monitored as in Kapvay. When discontinuing Intuniv, a gradual taper decreasing by 1 mg every 3 to 7 days is recommended. α-Adrenergic agents including the short- and extended-release preparations of guanfacine and clonidine are sometimes preferred treatments in children with ADHD and comorbid tic disorders that have been exacerbated while the patient was taking stimulants. Bupropion has been shown to be somewhat effective for some children and adolescents in the treatment of ADHD. One multisite, double-blind, placebo-controlled study found a positive result regarding the efficacy of bupropion. No further studies have compared bupropion with other stimulants. The risk of seizure development while on this drug is increased when using doses of greater than 400 mg per day. Few data confirm the efficacy of selective serotonin reuptake inhibitors (SSRIs) in the treatment of ADHD, but due to the frequency of comorbid depression and anxiety with ADHD, in cases of comorbidity, the SSRIs are likely to be considered at least in conjunction with a stimulant. Tricyclic drugs are not recommended in the treatment of ADHD due to potential cardiac arrhythmia effects. The reports of sudden death in at least four children with ADHD who were being treated with desipramine (Norpramin, Pertofrane) have made the tricyclic antidepressants an unlikely choice. Antipsychotics are occasionally introduced to treat refractory severely hyperactive children and adolescents who are significantly dysfunctional. Antipsychotics are generally not chosen in the treatment of ADHD due to the risks of tardive dyskinesia, withdrawal dyskinesia, neuroleptic malignant syndrome, and weight gain. Modafinil (Provigil), another type of CNS stimulant, originally developed to reduce daytime sleepiness in patients with narcolepsy, has been tried clinically in the treatment of adults with ADHD. Only one randomized, double-blind, placebo-controlled study of the efficacy and safety of modafinil film-coated tablets in approximately 250 adolescents with ADHD showed that 48 percent of those on active treatment were rated as “much” or “very much” improved compared with 17 percent of patients receiving placebo. The dosage range was from 170 to 425 mg administered once daily, titrated to optimal doses based on efficacy and tolerability. Modafinil failed to receive FDA approval based on a Stevens-Johnson skin rash that occurred in a patient during the trial. The most common side effects included insomnia, headache, and decreased appetite. Venlafaxine has been tried in clinical practice, especially for children and adolescents with combinations of ADHD and depression or anxiety features. No clear empirical evidence supports the use of venlafaxine in the treatment of ADHD. One open-label report of reboxetine, a selective norepinephrine reuptake inhibitor in 31 children and adolescents with ADHD who were resistant to methylphenidate treatment suggested that this agent may have efficacy. In this open trial, reboxetine was initiated and maintained at 4 mg per day. Most common side effects included drowsiness, sedation, and gastrointestinal symptoms. Reboxetine and other new agents in this class await controlled studies to further evaluate their potential efficacy. TREATMENT OF CNS STIMULANT SIDE EFFECTS. CNS stimulants are generally well tolerated, and current consensus is that once a day dosing is preferable for convenience and to minimize rebound side effects. Long-term tolerability of once-daily mixed amphetamine salts has shown mild side effects, most commonly decreased appetite, insomnia, and headache. A variety of strategies have been suggested for children and/or adolescents with ADHD who respond favorably to methylphenidate, but for whom insomnia has become a significant problem. Clinical strategies to manage insomnia include use of diphenhydramine (25 to 75 mg), low dose of trazodone (25 to 50 mg), or the addition of an α-adrenergic agent, such as guanfacine. In some cases, insomnia may attenuate on its own after several months of treatment. Monitoring Pharmacological Treatment STIMULANTS. Stimulant medications have adrenergic effects and cause moderate increases in blood pressure and pulse rate. At baseline, the most recent American Academy of Child and Adolescent Psychiatry (AACAP) practice parameters recommend the following workup before starting use of stimulant medications: physical examination, blood pressure, pulse, weight, and height. It is recommended that children and adolescents being treated with stimulants have their height, weight, blood pressure, and pulse checked on a quarterly basis and have a physical examination annually. Monitoring starts with the initiation of medication. Because school performance is most markedly affected, special attention and effort should be given to establishing and maintaining a close collaborative working relationship with a child’s school personnel. In most patients, stimulants reduce overactivity, distractibility, impulsiveness, explosiveness, and irritability. No evidence indicates that medications directly improve any existing impairments in learning, although, when the attention deficits diminish, children can learn more effectively. In addition, medication can improve self-esteem when children are no longer constantly reprimanded for their behavior. Children treated with medications should be taught the purpose of the medication and given the opportunity to describe any side effects that they may be experiencing. Psychosocial Interventions. Psychosocial interventions for children with ADHD include psychoeducation, academic organization skills remediation, parent training, behavior modification in the classroom and at home, cognitive behavioral therapy (CBT), and social skills training. Social skills groups, behavioral training for parents of children with ADHD, and behavioral interventions at school and at home have been studied alone and in combination with medication management for ADHD. Evaluation and treatment of coexisting learning disorders or additional psychiatric disorders is important. When children are helped to structure their environment, their anxiety diminishes. It is beneficial for parents and teachers to work together to develop a concrete set of expectations for the child and a system of rewards for the child when the expectations are met. A common goal of therapy is to help parents of children with ADHD recognize and promote the notion that, although the child may not “voluntarily” exhibit symptoms of ADHD, he or she is still capable of being responsible for meeting reasonable expectations. Parents should also be helped to recognize that, despite their child’s difficulties, every child faces the normal tasks of maturation, including significant building of self-esteem when he or she develops a sense of mastery. Therefore, children with ADHD do not benefit from being exempted from the requirements, expectations, and planning applicable to other children. Parental training is an integral part of the psychotherapeutic interventions for ADHD. Most parental training is based on helping parents develop usable behavioral interventions with positive reinforcement that target both social and academic behaviors. Group therapy aimed at both refining social skills and increasing self-esteem and a sense of success may be very useful for children with ADHD who have great difficulty functioning in group settings, especially in school. A recent year-long group therapy intervention in a clinical setting for boys with the disorder described the goals as helping the boys improve skills in game playing and feeling a sense of mastery with peers. The boys were first asked to do a task that was fun, in pairs, and then were gradually asked to do projects in a group. They were directed in following instructions, waiting, and paying attention, and were praised for successful cooperation. MULTIMODAL TREATMENT STUDY OF CHILDREN WITH ADHD (MTA STUDY) The National Institute of Mental Health (NIMH)–supported Multimodal Treatment Study of Children with ADHD (The MTA Cooperative Group, 1999) was a 14-month–long randomized clinical trial involving six clinical sites comparing four treatment strategies. More than 500 children diagnosed with DSM-IV ADHD, combined type, were randomly assigned to (1) systematic medication management utilizing an initial placebocontrolled titration and t.i.d. dosing 7 days per week and monthly 30-minute clinic visits, (2) behavior therapy consisting of 27 sessions of group parent training, eight individual parent sessions, an 8-week summer treatment program, 12 weeks of classroom administered behavior therapy with a half-time aide, and 10 teacher consultation sessions, (3) a combination of medication and behavior therapy, or (4) usual community care. All groups showed improvement over baseline; however, a combination of medication management and behavior therapy led to greater reduction in symptoms in children with ADHD alone or ADHD and Oppositional Defiant Disorder than behavior therapy alone or community care. The combination treatment had significantly better outcomes for those children with ADHD and anxiety and/or mood disorders compared to behavioral treatment and community care. Combined treatment but not medication management was superior for improvement in oppositional and aggressive symptoms, anxiety and mood symptoms, teacher rated social skills, parent– child relationships, and reading achievement. Furthermore, mean dose of medication per day was less in the combination group than in the medication-only management group. Results A follow-up of the MTA sample at 6 and 8 years revealed that the clinical presentation of the disorder, including severity of ADHD, comorbid conduct disturbance, and intellect were stronger predictors of later functioning than the type of treatment received in childhood during the 14-month study period. Although treatment-related improvements for the children who participated in the MTA study are maintained as long as treatment continues, the differential treatment efficacy appeared to be lost at approximately the 3- year mark. Overall, the evidence suggests that medication and psychosocial interventions for the combined type of ADHD in childhood provides the broadest benefit in functioning for this population. This is especially pertinent in view of the comorbidity of learning disorders, anxiety, mood disorders, and other disruptive behavior disorders that occur in children with ADHD. UNSPECIFIED ATTENTION-DEFICIT/HYPERACTIVITY DISORDER The DSM-5 includes Unspecified ADHD as a category for disturbances of inattention or hyperactivity that cause impairment, but do not meet the full criteria for ADHD. ADULT MANIFESTATIONS OF ADHD ADHD was historically believed to be a childhood condition resulting in delayed development of impulse control that would be generally outgrown by adolescence. In the last few decades many more adults with ADHD have been identified, diagnosed, and successfully treated. Longitudinal follow-up has shown that up to 60 percent of children with ADHD have persistent impairment from symptoms into adulthood. Genetic studies, brain imaging, and neurocognitive and pharmacological studies in adults with ADHD have replicated findings demonstrated in children with ADHD. Increased public awareness and treatment studies within the last decade have led to widespread acceptance of the need for diagnosis and treatment of adults with ADHD. Epidemiology Among adults, evidence suggests an approximate 4 percent prevalence of ADHD in the population. ADHD in adulthood is generally diagnosed by self-report, given the lack of school information and observer information available; therefore, it is more difficult to make an accurate diagnosis. Etiology Currently, ADHD is believed to be largely transmitted genetically, and increasing evidence supports this hypothesis, including the genetic studies, twin studies, and family studies outlined in the child and adolescent ADHD section. Brain imaging studies have obtained data suggesting that adults with ADHD exhibit decreased prefrontal glucose metabolism on PET compared with adults without ADHD. It is unclear whether these data reflect the presence of the disorder or a secondary effect of having ADHD over a period of time. Further studies using SPECT have revealed increased dopamine transporter (DAT) binding densities in the striatum of the brain in samples of adults with ADHD. This finding may be understood within the context of treatment for ADHD, in that standard stimulant treatment for ADHD, such as methylphenidate, acts to block DAT activity, possibly leading to a normalization of the striatal brain region in individuals with ADHD. Diagnosis and Clinical Features The clinical phenomenology of ADHD features inattention and manifestations of impulsivity prevailing as the core of this disorder. A leading figure in the development of criteria for adult manifestations of ADHD is Paul Wender, from the University of Utah, who began his work on adult ADHD in the 1970s. Wender developed criteria that could be applied to adults (Table 31.6-5). They included a retrospective diagnosis of ADHD in childhood, and evidence of current impairment from ADHD symptoms in adulthood. Furthermore, evidence exists of several additional symptoms that are typical of adult behavior as opposed to childhood behaviors. Table 31.6-5 Utah Criteria for Adult Attention-Deficit/Hyperactivity Disorder (ADHD) In adults, residual signs of the disorder include impulsivity and attention deficit (e.g., difficulty in organizing and completing work, inability to concentrate, increased distractibility, and sudden decision-making without thought of the consequences). Many people with the disorder have a secondary depressive disorder associated with low selfesteem related to their impaired performance and which affects both occupational and social functioning. Brett was a 26-year-old man convinced by his new wife to seek an evaluation for his distractibility, forgetfulness, and “not listening” after a minor traffic accident. After consulting his mother, Brett reported that in grade school, he was often “in trouble” for talking out of turn, and his mother recalled teachers’ reports that Brett often made careless mistakes on tests, forgot his assignments, and had great difficulty sitting still. Although as a young child he was considered gifted intellectually, when he got to the third grade his grades were only average, and he seemed more interested in getting his work done quickly than correctly. Brett was talkative and loud and enjoyed sports, although he was not particularly talented at them. Nevertheless, Brett had acquaintances and superficial friends because he was likeable, funny, and even entertaining. Brett had no idea what he wanted to do when he grew up, and during his senior year in high school, he neglected to finish any of his college applications on time, and ended up attending a community college part-time. During the two years after high school, Brett held down a series of jobs only briefly, including a construction job, a waiter position in a restaurant, and a Fed-Ex driver, and then decided that he wanted to become an actor. Brett went on a series of auditions, but found that he would become distracted and did poorly remembering his lines and even spaced out during readings. Despite that, he was chosen for one commercial. Brett reported that he had never had problems with abuse of drugs or alcohol, and he occasionally drank beer socially. During his evaluation with a child and adolescent psychiatrist, Brett disclosed that his greatest difficulties were with tasks that seemed boring to him. He had difficulty maintaining his attention, was easily distracted, felt restless most of the time, and became frustrated when he was expected to sit still for long periods of time. Brett endorsed 6 inattentive and 5 hyperactive/impulsive symptoms on a DSM ADHD Checklist of current symptoms. Brett met the diagnostic criteria for Adult ADHD, combined type, with a probable onset in childhood. Brett’s medical history was negative for all major illnesses, and neither he nor his parents had a history of cardiac abnormalities. He took no prescribed medications. After discussing the situation with his psychiatrist and his wife, Brett decided that he would like to try a stimulant medication. A trial of a once-a-day extended-release formulation of a stimulant medication was selected: Adderall XR 10 mg. At his first follow-up visit, a week later, Brett reported that he felt a slight effect from this medication but it was not enough to improve his functioning, so Brett and his psychiatrist agreed that he would increase his dose to 20 mg per day. At his next follow-up appointment, Brett reported that he had noticed significant improvement in his ability to focus, concentrate, and remember his lines in auditions. In fact, he had just received a small part in an upcoming movie. Brett and his wife were both thrilled with the results, and Brett continued to return monthly for follow up visits. (Adapted from McGough J. Adult manifestations of attention-deficit/hyperactivity disorder. In: Sadock BJ, Sadock VA, Ruiz P, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Philadelphia: Lippincott Williams & Wilkins; 2009:3577.) Differential Diagnosis A diagnosis of ADHD is likely when symptoms of inattention and impulsivity are described by adults as a life-long problem, not as episodic events. The overlap of ADHD and hypomania, bipolar II disorder, and cyclothymia is controversial and difficult to sort out retrospectively. Clear-cut histories of discrete episodes of hypomania and mania, with or without periods of depression, are suggestive of a mood disorder rather than a clinical picture of ADHD; however, ADHD may have predated the emergence of a mood disorder in some individuals. In such a case, ADHD and bipolar disorder may be diagnosed comorbidly. Adults with an early history of chronic school difficulties related to paying attention, activity level, and impulsive behavior are generally diagnosed with ADHD, even when a mood disorder occurs later in life. Anxiety disorders can coexist with ADHD, and are less difficult than hypomania to distinguish from it. Course and Prognosis The prevalence of ADHD diminishes over time, although at least half of children and adolescents may have the disorder into adulthood. Many children initially diagnosed with ADHD, combined type, exhibit fewer impulsive-hyperactive symptoms as they get older and, by the time they are adults, will meet criteria for ADHD, inattentive type. As with children, adults with ADHD demonstrate higher rates of learning disorders, anxiety disorders, mood disorders, and substance use disorder compared with the general population. Treatment Treatment of ADHD in adults targets pharmacotherapy, mainly long-acting stimulants, similar to that used with children and adolescents with ADHD. In adults, only the longacting stimulants are FDA approved in the treatment of ADHD. Signs of a positive response are an increased attention span, decreased impulsiveness, and improved mood. Psychopharmacological therapy may be needed indefinitely. Clinicians should use standard ways to monitor drug response and patient compliance. REFERENCES Antshel KM, Hargrave TM, Simonescu M, Kaul P, Hendricks K, Faraone SV. Advances in understanding and treating ADHD. BMC Medicine. 2011;9:7. Clarke AR, Barry RJ, Dupuy FE, Heckel LD, McCarthy R, Selikowitz M, Johnstone SJ. Behavioural differences between EEG-defined subgroups of children with Attention-Deficit/Hyperactivity Disorder. Clin Neurophysiol. 2011;122:1333– 1341. Cortese S, Kelly C, Chabernaud C, Proal E, Di Martino A, Milham MP, Castellanos FX. Toward systems neuroscience of ADHD: A meta-analysis of 55 fMRI studies. Am J Psychiatry. 2012;169:1038–1055. Elbe D, MacBride A, Reddy D. Focus on lisdexamfetamine: A review of its use in child and adolescent psychiatry. J Can Acad Child Adolesc Psychiatry. 2010;19:303–314. Greenhill, LL, Hechtman, L. Attention-deficit disorders. In: Sadock BJ, Sadock VA, & Ruiz P, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Vol. 2. Philadelphia. Lippincott Williams & Wilkins; 2009:3560. Hammerness PG, Perrin JM, Shelley-Abrahamson R, Wilens TE. Cardiovascular risk of stimulant treatment in pediatric attention-deficit/hyperactivity disorder: Update and clinical recommendations. J Am Acad Child Adolesc Psych. 2011;50:978–990. Hechtman L. Comorbidity and neuroimaging in attention-deficit hyperactivity disorder. Can J Psychiatry. 2009;54:649– 650. Kratochvil CJ, Lake M, Pliszka SR, Walkup JT. Pharmacologic management of treatment-induced insomnia in ADHD. J Am 12 - 31.7 Specific Learning Disorder 31.7 Specific Learning Disorder Acad Child Adolesc Psychiatry. 2005;44:499. McGough J. Adult manifestations of attention-deficit/hyperactivity disorder. In: Sadock BJ, Sadock VA, & Ruiz P, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Vol. 2. Philadelphia: Lippincott Williams & Wilkins; 2009:3572. Molina BSG, Hinshaw SP, Swanson JM, Arnold LE, Vitiello B, Jenson PS, Epstien JN, Hoza BM, Hechtman L, Abikoff HB, Elliot GR, Greenhill LL, Newcorn JH, Wells KC, Wigal T, Gibbons RD, Hur K, Houck PR, & The MTA Cooperative Group. The MTA at 8 years: Prospective follow-up of children treated for combined type ADHD in a multisite study. J Am Acad Child Adolesc Psychiatry. 2009;48:484–500. MTA Cooperative Group. A 14-month randomized clinical trial of treatment strategies for attention-deficit/hyperactivity disorder. Multimodal treatment study of children with ADHD. Arch Gen Psychiatry. 1999;56:1073–1086. Pelham WE, Manos MJ, Ezzell CE, Tresco KE, Gnagy EM, Hoffman MT, Onyango AN, Fabiano GA, Lopez-Williams A, Wymbs BT, Caserta D, Chronis AM, Burrows-Maclean L, Morse G. A dose-ranging study of a methylphenidate transdermal system in children with ADHD. J Am Acad Child Adolesc Psychiatry. 2005;44:522. Ratner A, Laor N, Bronstein Y, Weizman A, Toren P. Six-week open-label reboxetine treatment in children and adolescents with attention-deficit/hyperactivity disorder. J Am Acad Child Adolesc Psychiatry. 2005;44:428. Sassi RB. In this issue/Abstract thinking: From pixels to voxels: Television, brain, and behavior. J Am Acad Child Adolesc Psychiatry. 2013;52:665–666. Stevens LJ, Kuczek T, Burgess JR, Hurt E, Arnold LE. Dietary sensitivities and ADHD symptoms: Thirty-five years of research. Clin Pediatr. 2011;50:279–293. Tresco KE, Lefler EK, Power TJ. Psychosocial interventions to improve the school performance of students with attentiondeficit/hyperactivity disorder. Mind Brain. 2011;1:69–74. Weiss M, Tannock R, Kratochvil C, Dunn D, Velez-Borras J, Thomason C, Tamura R, Kelsey D, Stevens L, Allen AJ. A randomized, placebo-controlled study of once-daily atomoxetine in the school setting in children with ADHD. J Am Acad Child Adolesc Psychiatry. 2005;44:647. 31.7 Specific Learning Disorder Specific learning disorder in youth is a neurodevelopmental disorder produced by the interactions of heritable and environmental factors that influence the brain’s ability to efficiently perceive or process verbal and nonverbal information. It is characterized by persistent difficulty learning academic skills in reading, written expression, or mathematics, beginning in early childhood, that is inconsistent with the overall intellectual ability of a child. Children with specific learning disorder often find it difficult to keep up with their peers in certain academic subjects, whereas they may excel in others. Academic skills that may be compromised in specific learning disorder include reading single words and sentences fluently, written expression and spelling, and calculation and solving mathematical problems. Specific learning disorder results in underachievement that is unexpected based on the child’s potential as well as the opportunity to have learned more. Specific learning disorder in reading, spelling, and mathematics appears to aggregate in families. There is an increased risk of four to eight times in first-degree relatives for reading deficits, and about five to ten times for mathematics deficits, compared to the general population. Specific learning disorder occurs two to three times more often in males than in females. Learning problems in a child or adolescent identified in this manner can establish eligibility for academic services through the public school system. The American Psychiatric Association’s Fifth Edition of the Diagnostic and Statistical Manual of Mental Disorders (DSM5), combines the DSM-IV diagnoses of reading disorder, mathematics disorder, and disorder of written expression and learning disorder not otherwise specified into a single diagnosis: Specific learning disorder. Learning deficits in reading, written expression, and mathematics in the DSM-5 are designated using specifiers. DSM-5 notes that the term dyslexia is an equivalent term describing a pattern of learning difficulties, including deficits in accurate or fluent word recognition, poor decoding, and poor spelling skills. Dyscalculia is noted to be an alternative term referring to a pattern of deficits related to learning arithmetic facts, processing numerical information, and performing accurate calculations. Specific learning disorder of all types affects approximately 10 percent of youth. This represents approximately half of all public school children who receive special education services in the United States. In 1975, Public Law 94–142 (the Education for All Handicapped Children Act now known as the Individual with Disabilities Education Act [IDEA]) mandated all states to provide free and appropriate educational services to all children. Since that time, the number of children identified with learning disorders has increased, and a variety of definitions of learning disabilities have arisen. To meet the criteria for specific learning disorder, a child’s achievement must be significantly lower than expected in one or more of the following: reading skills, comprehension, spelling, written expression, calculation, mathematical reasoning, and/or the learning problems interfere with academic achievement or activities of daily living. It is common for specific learning disorder to include more than one area of skills deficits. Children with specific learning disorder in the area of reading can be identified by poor word recognition, slow reading rate, and impaired comprehension compared with most children of the same age. Current data suggest that most children with reading difficulties have deficits in speech sound processing skills, regardless of their intelligence quotient (IQ), and in DSM-5, there is no longer a diagnostic criterion for specific learning disorder comparing the specific deficit to overall IQ. Current consensus is that children with reading impairment have trouble with word recognition and “sounding out” words because they cannot efficiently process and use phonemes (the smaller bits of words that are associated with particular sounds). A recent epidemiologic study found four profiles including (1) weak reading, (2) weak language, (3) weak math, or (4) combined weak math and reading, accounting for 70 percent of children with specific learning impairments. Low scores in short-term memory for speech sounds characterized the profile with weak language, whereas, low speech sound awareness was associated with the weak reading group, but not the weak language group. Finally, in another recent study it was found that the weak math group did not show speech sound deficits, Severe specific learning disorder may make it agonizing for a child to succeed in school, often leading to demoralization, low self-esteem, chronic frustration, and compromised peer relationships. Specific learning disorder is associated with an increased risk of comorbid disorders, including attention-deficit/hyperactivity disorder (ADHD), communication disorders, conduct disorders, and depressive disorders. Adolescents with specific learning disorder are at least 1.5 times more likely to drop out of school, approximating rates of 40 percent. Adults with specific learning disorder are at increased risk for difficulties in employment and social adjustment. Specific learning disorder often extends to skills deficits in multiple areas such as reading, writing, and mathematics. Moderate to high heritability is believed to contribute to specific learning disorder, and furthermore, it appears that many cognitive traits are polygenic. In addition, there is pleiotropy, that is, the same genes may affect skills necessary for diverse learning tasks. Factors such as perinatal injury and specific neurological conditions may contribute to the development of specific learning disorder. Conditions such as lead poisoning, fetal alcohol syndrome, and in utero drug exposure are also associated with increased rates of specific learning disorder. SPECIFIC LEARNING DISORDER WITH IMPAIRMENT IN READING Reading impairment is present in up to 75 percent of children and adolescents with specific learning disorder. Students who have learning problems in other academic areas most commonly experience difficulties with reading as well. Reading impairment is characterized by difficulty in recognizing words, slow and inaccurate reading, poor comprehension, and difficulties with spelling. Reading impairment is often comorbid with other disorders in children, particularly, ADHD. The term developmental alexia was historically used to define a developmental deficit in the recognition of printed symbols. This was simplified by adopting the term dyslexia in the 1960s. Dyslexia was used extensively for many years to describe a reading disability syndrome that often included speech and language deficits and right–left confusion. Reading impairment is frequently accompanied by disabilities in other academic skills, and the term dyslexia remains as an alternate term for a pattern of reading and spelling difficulties. Epidemiology An estimated 4 to 8 percent of youth in the United States have been identified with dyslexia, encompassing a variety of reading, spelling, and comprehension deficits. Three to four times as many boys as girls are reported to have reading impairments in clinically referred samples. In epidemiological samples, however, rates of reading impairments are much closer among boys and girls. Boys with reading impairment are referred for psychiatric evaluation more often than girls due to comorbid ADHD and disruptive behavior problems. No clear gender differential is seen among adults who report reading difficulties. Comorbidity Children with reading difficulties are at high risk for additional learning deficits including mathematics and written expression. The DSM-5 Language disorder, also known as specific language impairment, has traditionally been viewed as distinct from dyslexia and dyscalculia. Children with language disorder have poor word knowledge, limited abilities to form accurate sentence structure, and impairments in the ability to put words together to produce clear explanations. Children with language disorder may have delayed development of language acquisition, and difficulties with grammar and syntactical knowledge. Specific learning disorder in the areas of reading and mathematics frequently occur comorbidly with language disorder. In one study, it was found that among dyslexic samples, 19 percent to 63 percent also have language impairment. Conversely, reading impairment has been found in 12.5 percent to 85 percent of individuals with language disorder. In twin studies, reading impairments were found to be significantly higher in those children with specific learning impairment and in family members of children with the disorder. There are also high rates of comorbidity between reading impairment and mathematics impairment; in some studies the comorbidity has been reported to be up to 60 percent. It appears that children with both reading and math impairment may perform more poorly in mathematics; however, the reading skills of the comorbid children were no different from children who had only reading disorder and not math disorder. Comorbid psychiatric disorders are also frequent, such as ADHD, Oppositional Defiant Disorder, Conduct Disorders, and Depressive Disorders, especially in adolescents. Data suggest that up to 25 percent of children with reading impairment may have comorbid ADHD. Conversely, it is estimated that between 15 and 30 percent of children diagnosed with ADHD have specific learning disorder. Family studies suggest that ADHD and reading impairment may share some degree of heritability. That is, some genetic factors contribute to both reading impairment and attentional syndromes. Youth with reading impairments have higher than average rates of depression on self-report measures and experience higher levels of anxiety symptoms than children without specific learning disorder. Furthermore, children with reading impairment are at increased risk for poor peer relationships and exhibit less skill in responding to subtle social cues. Etiology Data from cognitive, neuroimaging, and genetic studies suggest that reading impairment is a neurobiological disorder with a significant genetic contribution. It reflects a deficiency in processing sounds of speech sounds, and thus, spoken language. Children who struggle with reading most likely also have a deficit in speech sound processing skills. Children with this deficit cannot effectively identify the parts of words that denote specific sounds, leading to difficulty in recognizing and “sounding out” words. Youth with reading impairment are slower than peers in naming letters and numbers. The core deficits for children with reading impairment include poor processing of speech sounds and deficits in comprehension, spelling, and sounding out words. Because reading impairment typically includes a language deficit, the left brain has been hypothesized to be the anatomical site of this dysfunction. Several studies using magnetic resonance imaging (MRI) studies have suggested that the planum temporale in the left brain shows less asymmetry than the same site in the right brain in children with both language disorders and specific learning disorder. Positron emission tomographic (PET) studies have led some researchers to conclude that left temporal blood flow patterns during language tasks differ between children with and without learning disorders. Cell analysis studies suggest that in reading impaired individuals, the visual magnocellular system (which normally contains large cells) contains more disorganized and smaller cell bodies than expected. Studies indicate that 35 to 40 percent of firstdegree relatives of children with reading deficits also have reading disability. Several studies have suggested that phonological awareness (i.e., the ability to decode sounds and sound out words) is linked to chromosome 6. Furthermore, the ability to identify single words has been linked to chromosome 15. Impairment in reading and spelling has now been linked to susceptibility loci on multiple chromosomes, including chromosomes 1, 2, 3, 6, 15, and 18. Although a recent research study identified a locus on chromosome 18 as a strong influence on single word reading and phoneme awareness, generalist genes have also been implicated as responsible for learning disorders. Many genes believed to be associated with specific learning disorder, may also influence normal variation in learning abilities. In addition, genes that affect abilities in reading, for example, are hypothesized to also affect written expression and potentially mathematics skills. Several historical hypotheses about the origin of reading deficits are now known to be untrue. The first myth is that reading impairments are caused by visual–motor problems, or what has been termed scotopic sensitivity syndrome. There is no evidence that children with reading impairment have visual problems or difficulties with their visual–motor system. The second false theory is that allergies can cause, or contribute to, reading disability. Finally, unsubstantiated theories have implicated the cerebellar–vestibular system as the source of reading disabilities. Research in cognitive neuroscience and neuropsychology supports the hypothesis that encoding processes and working memory, rather than attention or long-term memory, are areas of weakness for children with reading impairment. One study found an association between dyslexia and birth in the months of May, June, and July, suggesting that prenatal exposure to a maternal infectious illness, such as influenza, in the winter months may contribute to reading disabilities. Complications during pregnancy and prenatal and perinatal difficulties are common in the histories of children with reading disabilities. Extremely low birth weight and severely premature children are at higher risk for specific learning disorder. Children born very preterm have been noted to be at increased risk of minor motor, behavioral, and specific learning disorder. An increased incidence of reading impairment occurs in intellectually average children with cerebral palsy and epilepsy. Children with postnatal brain lesions in the left occipital lobe, resulting in right visual-field blindness, as well as youth with lesions in the splenium of the corpus callosum that blocks transmission of visual information from the intact right hemisphere to the language areas of the left hemisphere experience reading impairments. Children malnourished for long periods during early childhood are at increased risk of compromised performance cognition, including reading. Diagnosis Reading impairment is diagnosed when a child’s reading achievement is significantly below that expected of a child of the same age (Table 31.7-1). Characteristic diagnostic features include difficulty recalling, evoking, and sequencing printed letters and words; processing sophisticated grammatical constructions; and making inferences. School failure and ensuing poor self-esteem can exacerbate the problems as a child becomes more consumed with a sense of failure and spends less time focusing on academic work. Students with reading impairment are entitled to an educational evaluation through the school district to determine eligibility for special education services. Special education classification, however, is not uniform across states or regions, and students with identical reading difficulties may be eligible for services in one region, but ineligible in another. Table 31.7-1 DSM-5 Diagnostic Criteria for Specific Learning Disorder Clinical Features Children with reading disabilities are usually identified by the age of 7 years (second grade). Reading difficulty may be apparent among students in classrooms where reading skills are expected as early as the first grade. Children can sometimes compensate for reading disorder in the early elementary grades by the use of memory and inference, particularly in children with high intelligence. In such instances, the disorder may not be apparent until age 9 (fourth grade) or later. Children with reading impairment make many errors in their oral reading. The errors are characterized by omissions, additions, and distortions of words. Such children have difficulty in distinguishing between printed letter characters and sizes, especially those that differ only in spatial orientation and length of line. The problems in managing printed or written language can pertain to individual letters, sentences, and even a page. The child’s reading speed is slow, often with minimal comprehension. Most children with reading disability have an age-appropriate ability to copy from a written or printed text, but nearly all spell poorly. Associated problems include language difficulties: discrimination and difficulty in sequencing words properly. A child with reading disorders may start a word either in the middle or at the end of a printed or written sentence. Most children with reading disorder dislike and avoid reading and writing. Their anxiety is heightened when they are confronted with demands that involve printed language. Many children with specific learning disorder who do not receive remedial education have a sense of shame and humiliation because of their continuing failure and subsequent frustration. These feelings grow more intense with time. Older children tend to be angry and depressed and exhibit poor self-esteem. Jackson, a 10-year-old boy, was referred for evaluation of failing to complete inclass assignments and homework, and failing tests in reading, spelling, and arithmetic. For the past 2 years (grades 5 and 6), he had been attending a special education class every morning in the local community school, based on an assessment from the second grade. A subsequent psychoeducational assessment by a clinical psychologist confirmed reading problems. Jackson was eligible for a full-day special education class, whereupon he started attending a program with eight other students ranging from 6 to 12 years of age. Clinical interview with his parents revealed a normal pregnancy and neonatal period, and a history of language delay. In preschool and kindergarten, Jackson was reported to have had difficulty with rhyming games and showed a lack of interest in books and preferred to play with construction toys. In the first grade, Jackson had more difficulty learning to read than other boys in his class and continued to have problems pronouncing multisyllabic words (e.g., he said “aminals” for “animals” and “sblanation” for “explanation”). Family history was positive for reading deficits and ADHD. Jackson’s father disclosed a history of his own reading problems, and Jackson’s older brother, 15 years of age, had ADHD, which was well controlled with stimulant medication. Jackson’s parents were concerned about his poor focus in school, and wondered whether he had ADHD. In the clinical interview with Jackson, he rarely made eye contact, mumbled a lot, and struggled to find the right words (e.g., manifested many false starts, hesitations, and nonspecific terms, such as “the thing that you draw . . . um . . . pencil—no . . . um . . . lines with”). He admitted to disliking school, adding “Reading is boring and stupid—I’d rather be skateboarding.” Jackson complained about how much reading he was given—even in math—and commented, “Reading takes so much time. By the time I figure out a word, I can’t remember what I just read and so have to read the stuff again.” Psychoeducational assessment included the Wechsler Intelligence Scale for ChildrenIV, Clinical Evaluation of Language Fundamentals-IV (CELF-IV), the Wechsler Individual Achievement Test-II, and self-ratings of anxiety, depression, and selfesteem. Results indicated low-average verbal and above average performance IQ, poor word attack and word identification skills (below 12th percentile), poor comprehension (below 9th percentile), poor spelling (below 6th percentile), weak comprehension of oral language (below 16th percentile), elevated but subthreshold scores on the Children’s Depression Inventory, and low self-esteem. Although Jackson manifested symptoms of inattention, restlessness, and oppositional behavior (particularly at school), he did not meet criteria for ADHD. Jackson met DSM-5 criteria for specific learning disorder, with deficits in reading and written expression. Recommendations included continuation in special education plus attendance at a summer camp specializing in children with reading disorder, as well as ongoing monitoring of self-esteem and depressive traits. At 1-year follow-up, Jackson and his parents reported striking improvements in his reading, overall school performance, mood, and self-esteem. Both Jackson and his family felt that the specialized instruction provided during the summer camp was very helpful. The program had provided one-on-one focused and explicit instruction for 1 hour a day for a total of 70 hours. Jackson explained that he had been taught “like a game plan” to read, and challenged the clinician to give him a “really tough long word to read.” He demonstrated strategies that he had learned to read the word “unconditionally” and also explained what it meant. To boost his fluency in reading and comprehension, he was provided with assignments to read along with audiotaped versions of books, use of graphic organizers to facilitate reading comprehension, and continued participation in the summer camp reading program. (Adapted from Rosemary Tannock, Ph.D.) Pathology and Laboratory Examination No specific physical signs or laboratory measures are helpful in the diagnosis of reading deficits. Psychoeducational testing, however, is critical in determining these deficits. The diagnostic battery generally includes a standardized spelling test, written composition, processing and using oral language, design copying, and judgment of the adequacy of pencil use. The reading subtests of the Woodcock-Johnson Psycho-Educational BatteryRevised, and the Peabody Individual Achievement Test-Revised are useful in identifying reading disability. A screening projective battery may include human-figure drawings, picture-story tests, and sentence completion. The evaluation should also include systematic observation of behavioral variables. Course and Prognosis Children with reading disability may gain knowledge of printed language during their first 2 years in grade school, without remedial assistance. By the end of the first grade, many children with reading problems, in fact, have learned how to read a few words; however, by the third grade, keeping up with classmates is exceedingly difficult without remedial educational intervention. When remediation is instituted early, in milder cases, it may not be necessary after the first or second grade. In severe cases and depending on the pattern of deficits and strengths, remediation may be continued into the middle and high school years. Differential Diagnosis Reading deficits are often accompanied by comorbid disorders, such as language disorder, disability in written expression, and ADHD. Data indicate that children with reading disability consistently present difficulties with linguistic skills, whereas children with ADHD only, do not. Children with reading disability, without ADHD, however, may have some overlapping deficits in cognitive inhibition, for example, they perform impulsively on continuous performance tasks. Deficits in expressive language and speech discrimination along with reading disorder may lead to a comorbid diagnosis of language disorder. Reading impairment must be differentiated from intellectual disability syndromes in which reading, along with most other skills, are below the achievement expected for a child’s chronological age. Intellectual testing helps to differentiate global deficits from more specific reading difficulties. Poor reading skills resulting from inadequate schooling can be detected by comparing a given child’s achievement with classmates on reading performance on standardized reading tests. Hearing and visual impairments should be ruled out with screening tests. Treatment Remediation strategies for children with reading impairments focus on direct instruction that leads a child’s attention to the connections between speech sounds and spelling. Effective remediation programs begin by teaching the child to make accurate associations between letters and sounds. This approach is based on the theory that the core deficits in reading impairments are related to difficulty recognizing and remembering the associations between letters and sounds. After individual letter-sound associations have been mastered, remediation can target larger components of reading such as syllables and words. The exact focus of any reading program can be determined only after accurate assessment of a child’s specific deficits and weaknesses. Positive coping strategies include small, structured reading groups that offer individual attention and make it easier for a child to ask for help. Children and adolescents with reading difficulties are entitled to an individual education program (IEP) provided by the public school system. Yet, for high school students with persistent reading disorders and ongoing difficulties with decoding and work identification, IEP services may not be sufficient to remediate their problems. A study of students with reading disorders in 54 schools indicated that, at the high school level, specific goals are not adequately met solely through school remediation. It is likely that high schoolers with persisting reading difficulties may have greater benefit from individualized reading remediation. Reading instruction programs such as the Orton Gillingham and Direct Instructional System for Teaching and Remediation (DISTAR) approaches begin by concentrating on individual letters and sounds, advance to the mastery of simple phonetic units, and then blend these units into words and sentences. Thus, if children are taught to cope with graphemes, they will learn to read. Other reading remediation programs, such as the Merrill program, and the Science Research Associates, Inc. (SRA) Basic Reading Program, begin by introducing whole words first and then teach children how to break them down and recognize the sounds of the syllables and the individual letters in the word. Another approach teaches children with reading disorders to recognize whole words through the use of visual aids and bypasses the sounding-out process. One such program is called the Bridge Reading Program. The Fernald method uses a multisensory approach that combines teaching whole words with a tracing technique so that the child has kinesthetic stimulation while learning to read the words. SPECIFIC LEARNING DISORDER WITH IMPAIRMENT IN MATHEMATICS Children with mathematics difficulties have difficulty learning and remembering numerals, cannot remember basic facts about numbers, and are slow and inaccurate in computation. Poor achievement in four groups of skills have been identified in mathematics disorder: linguistic skills (those related to understanding mathematical terms and converting written problems into mathematical symbols), perceptual skills (the ability to recognize and understand symbols and order clusters of numbers), mathematical skills (basic addition, subtraction, multiplication, division, and following sequencing of basic operations), and attentional skills (copying figures correctly and observing operational symbols correctly). A variety of terms over the years, including dyscalculia, congenital arithmetic disorder, acalculia, Gerstmann syndrome, and developmental arithmetic disorder have been used to denote the difficulties present in mathematics disorder. Core deficits in dyscalculia are in processing numbers, and good language abilities are skills needed for accurate counting, calculating, and understanding mathematical principles. Mathematics deficits can, however, occur in isolation or in conjunction with language and reading impairments. According to the DSM-5, the diagnosis of specific learning disorder with impairment in mathematics consists of deficits in arithmetic counting and calculations, has difficulty remembering mathematics facts, and may count on fingers instead. Additional deficits include difficulty with mathematic concepts and reasoning, leading to difficulties in applying procedures to solve quantitative problems. These deficits lead to skills that are substantially below what is expected for the child’s chronological age and cause significant interference in academic success, as documented by standardized academic achievement testing. Epidemiology Mathematics disability alone is estimated to occur in about 1 percent of school-age children, that is about one of every five children with specific learning disorder. Epidemiological studies have indicated that up to 6 percent of school-age children have some difficulty with mathematics, and prevalence estimates of 3.5 to 6.5 percent have been reported for impairing forms of dyscalculia. Although specific learning disorder overall occurs two to three times more often in males, mathematics deficits may be relatively more frequent in girls than reading deficits. Many studies of learning disorders in children have grouped reading, writing, and mathematics disability together, which makes it more difficult to ascertain the precise prevalence of mathematics disability. Comorbidity Mathematics deficits are commonly found to be comorbid, with deficits in both reading and written expression. Children with mathematics difficulties may also be at higher risk for expressive language problems, and developmental coordination disorder. Etiology Mathematics deficiency, as with other areas of specific learning disorder, has a significant genetic contribution. High rates of comorbidity with reading deficits have been reported in the range of 17 percent up to 60 percent. One theory proposed a neurological deficit in the right cerebral hemisphere, particularly in the occipital lobe areas. These regions are responsible for processing visual–spatial stimuli that, in turn, are responsible for mathematical skills. This theory, however, has received little support in subsequent neuropsychiatric studies. Causes of deficits in mathematics are believed to be multifactorial, including genetic, maturational, cognitive, emotional, educational, and socioeconomic factors. Prematurity and very low birth weight are also a risk factor for specific learning disorder, including mathematics. Compared with reading abilities, arithmetic abilities seem to depend more on the amount and quality of instruction. Diagnosis The diagnosis of specific learning disorder in mathematics is made when a child’s skill in mathematical reasoning, or calculation, remain significantly below what is expected for that child’s age, for a period of at least 6 months, even when remedial interventions have been administered. Many different skills contribute to mathematics proficiency. These include linguistic skills, conceptual skills, and computational skills. Linguistic skills involve being able to understand mathematical terms, understand word problems, and translate them into the proper mathematical process. Conceptual skills involve recognition of mathematical symbols and being able to use mathematical signs correctly. Computational skills include the ability to line up numbers correctly and to follow the “rules” of the mathematical operation. The DSM-5 diagnostic criteria for specific learning disorder with impairment in mathematics are provided in Table 31.7-1. Clinical Features Common features of mathematics deficit include difficulty learning number names, remembering the signs for addition and subtraction, learning multiplication tables, translating word problems into computations, and performing calculations at the expected pace. Most children with mathematics deficits can be detected during the second and third grades in elementary school. A child with poor mathematics abilities typically has problems with concepts, such as counting and adding even one-digit numbers, compared with classmates of the same age. During the first 2 or 3 years of elementary school, a child with poor mathematics skill may just get by in mathematics by relying on rote memory. But soon, as mathematics problems require discrimination and manipulation of spatial and numerical relations, a child with mathematics difficulties is overwhelmed. Some investigators have classified mathematics deficiencies into the following categories: difficulty learning to count meaningfully; difficulty mastering cardinal and ordinal systems; difficulty performing arithmetic operations; and difficulty envisioning clusters of objects as groups. Children with mathematics difficulty have trouble associating auditory and visual symbols, understanding the conservation of quantity, remembering sequences of arithmetic steps, and choosing principles for problem-solving activities. Children with these problems are presumed to have good auditory and verbal abilities; however, in many cases, the mathematics deficits may occur in conjunction with reading, writing, and language problems. In these cases, the other deficiencies may compound the impairment of the poor mathematics skill. Mathematics difficulty, in fact, often coexists with other disorders affecting reading, expressive writing, coordination, and language. Spelling problems, deficits in memory or attention, and emotional or behavioral problems may be present. Young grade-school children may exhibit specific learning problems in reading and writing, and these children should also be evaluated for mathematics deficits. The exact relationship between mathematics deficits and the deficits in language and dyslexia is not clear. Although children with language disorder do not necessarily experience mathematics deficiencies, these conditions often coexist, and both are associated with impairments in decoding and encoding processes. Lena, an 8-year-old girl, was referred for evaluation of impairing problems in attention and academic achievement, which were first noted in kindergarten but were now causing difficulty at home and school. Lena attended a regular third-grade class in a local public school, which she had been attending since midway through kindergarten. Lena’s history included a mild delay in speech acquisition (e.g., first words at approximately 18 months of age and short sentences at approximately 3 years of age), but otherwise she had no major developmental problems until kindergarten, when her teacher had raised concerns about inattentiveness, difficulty following instructions, and her difficulty in mastering basic number concepts (e.g., inaccurate counting of sets of objects). A speech, language, and hearing assessment completed at the end of kindergarten revealed mild language problems that did not warrant specific intervention. School reports from grades 1 and 2 noted ongoing concerns about inattention, poor reading skills, and difficulty mastering simple arithmetic facts, and “making careless mistakes in copying numbers from the board and in doing addition and subtraction.” These problems continued through grade 2, despite some in-school accommodations (e.g., moving Lena’s seat closer to the teacher) and modifications (e.g., providing her with printed sheets of arithmetic problems so she did not need to copy them herself). Lena’s parents reported a 3-year history of losing things, fidgeting at the dinner table, and difficulty concentrating on games and homework, and forgetting to bring notes to and from school. Psychological assessment included the Wechsler Intelligence Scale for Children-III, Clinical Evaluation of Language Fundamentals-IV, Comprehensive Test of Phonological Processing, and the Woodcock-Johnson Psycho-Educational Battery–III. Results indicated average intelligence, with relatively weaker performance on tests of perceptual organization, weak phonological (speech sound) awareness, mild deficits in receptive and expressive language, and reading and arithmetic abilities that were well below grade level. Parent and teacher ratings on a standardized behavior questionnaire (Conners’ Rating Scales-Long Form) were above clinical threshold for ADHD. Lena was given a diagnosis of ADHD, predominantly inattentive type, and specific learning disorder with impairment in reading, based on the history, school achievement, and standardized assessment. She did not meet criteria for communication disorder, and it was speculated that her mathematics problems did not cause impairment like her reading disorder and ADHD did. Recommendations included the following: family psychoeducation clarifying the ADHD and specific learning disorder, remedial interventions for reading, and treatment of her ADHD with a long-acting stimulant agent. At 1-year follow-up, Lena and her parents reported noticeable improvement with inattention, but ongoing problems with reading and more significant deficits in mathematics. Mathematics remediation was added to her weekly schedule. Two years later, when Lena was 11 years of age, her parents called for an “urgent reevaluation” due to a sudden worsening of her difficulties at home and school. Clinical evaluation revealed adequate stimulant treatment response of her ADHD, more marked deficits in reading speed accuracy compared to others her age, and significant deficits in mathematics. Lena’s parents reported that she had started lying about having mathematics homework or refused to do it, was suspended from mathematics class twice in the past 3 months because of oppositional behavior, and had failed sixthgrade mathematics. Lena acknowledged disliking and worrying about math: “whenever the teacher starts asking questions and looks in my direction, my mind just goes blank and I feel sort of shaky—it’s so bad in tests that I have to leave class to get myself together.” At this point, an additional component of anxiety was noted to be contributing to her school impairments. Recommendations were expanded to include increased specific educational remediation for mathematics. At follow-up, Lena reported that the resource teacher had taught her some helpful strategies to address her anxiety about mathematics, as well as ways of classifying word problems and differentiating critical information from irrelevant information. She continued to be a robust responder to long-acting stimulants for her ADHD, and had only minimal difficulties concentrating on homework after school. (Adapted from case material by Rosemary Tannock, Ph.D.) Pathology and Laboratory Examination No physical signs or symptoms indicate mathematics disorder, but educational testing and standardized measurement of intellectual function are necessary to make this diagnosis. The Keymath Diagnostic Arithmetic Test measures several areas of mathematics including knowledge of mathematical content, function, and computation. It is used to assess ability in mathematics of children in grades 1 to 6. Course and Prognosis A child with a specific learning disorder in mathematics can usually be identified by the age of 8 years (third grade). In some children, the disorder is apparent as early as 6 years (first grade); in others, it may not be apparent until age 10 (fifth grade) or later. Too few data are currently available from longitudinal studies to predict clear patterns of developmental and academic progress of children classified as having mathematics disorder in early school grades. On the other hand, children with a moderate mathematics disorder who do not receive intervention may have complications, including continuing academic difficulties, shame, poor self-concept, frustration, and depression. These complications can lead to reluctance to attend school, and demoralization about academic success. Differential Diagnosis Mathematics deficits must be differentiated from global causes of impaired functioning such as intellectual disability. Arithmetic difficulties in intellectual disability are accompanied by similar impairments in overall intellectual functioning. Inadequate schooling can affect a child’s arithmetic performance on a standardized arithmetic test. Conduct disorder or ADHD can occur comorbidly with specific learning disorder in mathematics and, in these cases, both diagnoses should be made. Treatment Mathematics difficulties for children are best remediated with early interventions that lead to improved skills in basic computation. The presence of specific learning disorder in reading along with mathematics difficulties can impede progress; however, children are quite responsive to remediation in early grade school. Children with indications of mathematics disorder as early as in kindergarten require help in understanding which digit in a pair is larger, counting abilities, identification of numbers, and remembering sequences of numbers. Flash cards, workbooks, and computer games can be a viable part of this treatment. One study indicated that mathematics instruction is most helpful when the focus is on problem-solving activities, including word problems, rather than only computation. Project MATH, a multimedia self-instructional or group-instructional in-service training program, has been successful for some children with mathematics disorder. Computer programs can be helpful and can increase compliance with remediation efforts. Social skills deficits can contribute to a child’s hesitation in asking for help, so a child identified with a mathematics disorder may benefit from gaining positive problemsolving skills in the social arena as well as in mathematics. SPECIFIC LEARNING DISORDER WITH IMPAIMRENT IN WRITTEN EXPRESSION Written expression is the most complex skill acquired to convey an understanding of language and to express thoughts and ideas. Writing skills are highly correlated with reading for most children; however, for some youth, reading comprehension may far surpass their ability to express complex thoughts. Written expression in some cases is a sensitive index of more subtle deficits in language usage that typically are not detected by standardized reading and language tests. Deficits in written expression are characterized by writing skills that are significantly below the expected level for a child’s age and education. Such deficits impair the child’s academic performance and writing in everyday life. Components of writing disorder include poor spelling, errors in grammar and punctuation, and poor handwriting. Spelling errors are among the most common difficulties for a child with a writing disorder. Spelling mistakes are most often phonetic errors; that is, an erroneous spelling that sounds like the correct spelling. Examples of common spelling errors are: fone for phone, or beleeve for believe. Historically, dysgraphia (i.e., poor writing skills) was considered to be a form of reading disorder; however, it is now clear that impairment in written expression can occur on its own. Terms once used to describe writing disability include spelling disorder and spelling dyslexia. Writing disabilities are often associated with other forms of specific learning disorders; however, impaired writing ability may be identified later than other forms because it is generally acquired later than verbal language and reading. In contrast with the DSM-5, which includes specific learning disorder in written expression, the 10th Edition of the International Statistical Classification of Diseases and Related Health Problems (ICD-10) includes a separate specific spelling disorder. Epidemiology The prevalence of specific learning disorder with impairment in written expression has been reported to occur in the range of 5 to –15 percent of school-age children. Over time, specific learning disorder remits in many youth, leading to a persistent rate of specific learning disorder of 4 percent in adults. The gender ratio in writing deficits is two to three to one in boys compared with girls. Impaired written expression often occurs along with deficits in reading, but not always. Comorbidity Children with impaired writing ability are significantly more likely to have language disorder and impairments in reading and mathematics compared to the general population of youth. ADHD occurs with greater frequency in children with writing disability than in the general population. Youth with specific learning disorder, including writing disability, are at higher risk for social skills difficulties, and some develop poor self-esteem and depressive symptoms. Etiology Causes of writing disability are believed to be similar to those of reading disorder, that is, underlying deficits in using the components of language related to letter sounds. Genetic factors are a significant factor in the development of writing disability. Writing difficulties often accompany language disorder, leading an affected child to have trouble with understanding grammatical rules, finding words, and expressing ideas clearly. According to one hypothesis, impairment in written expression may result from combined effects of language disorder, and reading disorder. Hereditary predisposition to writing impairment is supported by the finding that most youth with impaired written expression have first-degree relatives with similar difficulties Children with limited attention spans and high levels of distractibility may find writing an arduous task. Diagnosis The DSM-5 diagnosis of specific learning disorder with impairment in written expression is based on a child’s poor ability to use punctuation and grammar accurately in sentences, inability to organize paragraphs, or to clearly articulate ideas in writing. Poor performance on composing written text may also include poor handwriting and impaired ability to spell and to place words sequentially in coherent sentences, compared to others of the same age. In addition to spelling mistakes, youth with impaired written expression make grammatical mistakes, such as using incorrect tenses, forgetting words in sentences, and placing words in the wrong order. Punctuation may be incorrect, and the child may have poor ability to remember which words begin with capital letters. Additional symptoms of impaired written expression include the formation of letters that are not legible, inverted letters, and mixtures of capital and lowercase letters in a given word. Other features of writing disorders include poor organization of written stories, which lack critical elements such as “where,” “when,” and “who” or clear expression of the plot. Clinical Features Youth with impairments in written expression struggle early in grade school with spelling words and expressing their thoughts according to age-appropriate grammatical norms. Their spoken and written sentences contain an unusually large number of grammatical errors and poor paragraph organization. Affected children commonly make simple grammatical errors, even when writing a short sentence. For example, despite constant reminders, affected youth frequently fail to capitalize the first letter of the first word in a sentence, and fail to end the sentence with a period. Typical features of impaired written expression include spelling errors, grammatical errors, punctuation errors, poor paragraph organization, and poor handwriting. In higher grades in school, affected youth’s written sentences become more conspicuously primitive, odd, and inaccurate compared to what is expected of students at their grade level. For youth with impaired written expression, word choices are often erroneous and inappropriate, paragraphs are disorganized and not in proper sequence, and spelling accuracy becomes increasingly difficult as their vocabulary becomes larger and more abstract. Associated features of writing impairments may include reluctance to go to school, refusal to do assigned written homework, and concurrent academic difficulties in other areas. Many children with impaired written expression understandably become frustrated and angry, and harbor feelings of shame and inadequacy regarding poor academic achievement. In some cases, depressive disorders can result from a growing sense of isolation, estrangement, and despair. Young adults with impaired written expression who do not receive remedial intervention continue to have writing skills deficits and a persistent sense of incompetence and inferiority. Brett, an 11-year-old boy, was referred for evaluation of increasing problems in school over a 2-year period, including failure to complete assigned schoolwork and homework, inattention and oppositional behavior, and deteriorating grades and test scores. At the time of assessment, he was enrolled in a regular fifth-grade class in a public school, which he had been attending since grade 1. Clinical interview with parents revealed that Brett had a twin brother (monozygotic) with a history of language problems for which he had received speechlanguage therapy in the preschool years and remedial reading in the primary grades. Brett, however, had not exhibited difficulty in speech or language development, according to parental report and scores on standardized tests of oral language administered in the preschool years. His current and previous school reports indicate that Brett participated well in class discussions and had no difficulty in reading or mathematics; however, his written work was far below grade level. In each of the last 2 years, his teachers had expressed increasing concerns about Brett’s refusal to complete written work, failure to hand in homework, daydreaming and fidgeting in class, and withdrawal from class activities. Brett admitted to an increasing dislike of school and especially writing assignments. He explained, “It’s writing, writing all day long—even in math and science. I know how to do the problems and the experiments, but I hate having to write it all down—my mind just goes blank.” Brett complained “My teacher is always on at me, telling me that I’m lazy and haven’t done enough, and that my writing is atrocious. He tells me, I’ve got a bad attitude—so why would I want to go to school?” Brett and his parents reported that, over the past year, he has been down, increasing frustrated with school, and has refused to do homework. They all agree that Brett has had a few brief episodes of depressed mood. Testing by a clinical psychologist revealed average to high-average scores on the verbal and performance scales of the Wechsler Intelligence Scale for Children-III and average scores on the reading and arithmetic subtests of the Wide Range Achievement Test-3 (WRAT-3). However, scores on the WRAT-3 spelling subtest were below the 9th percentile, which was significantly below expectations for age and ability. Examination of his spelling errors revealed that, although his spelling was typically phonologically accurate (i.e., could plausibly be pronounced to sound like the target word), it was unacceptable in that he used letter sequences that did not resemble English, regardless of pronunciation (e.g., “houses” was written as “howssis,” “phones” was written as “fones,” and “exact” was written as “egszakt”). Moreover, his performance was well below age and grade on standardized tests of written expression (TOWL-3), as well as on a brief (5-minute) informal assessment of expository text generation on a favorite topic (e.g., newspaper article on recent sports event). During the 5-minute writing activity, he was observed to frequently stare out of the window, to shift positions and to chew on his pencil, to get up to sharpen his pencil and to sigh when he did put pencil to paper, and to write slowly and laboriously. At the end of 5 minutes, he had produced three short sentences without any punctuation or capitalization that were barely legible, containing several misspellings and grammatical errors, and that were not linked semantically. By contrast, later in the assessment, he described the sporting event with detail and enthusiasm. A speech-language evaluation revealed average scores on standard tests of oral language (Clinical Evaluation of Language Fundamentals-IV), but he was noted to omit sounds or syllables in a multisyllabic word in a nonword repetition test, which has been found to be sensitive to mild residual language impairments and written language impairments. The clinical team formulated a diagnosis of specific learning disorder with impairment in written expression, based on Brett’s inability to compose written text, poor spelling, and grammatical errors, without problems in reading or mathematics or a history of language impairments. He did not meet full diagnostic criteria for any other DSM-5 disorder, including oppositional defiant disorder, ADHD, or mood disorder. Recommendations included the following: psychoeducation, the need for educational accommodations (e.g., provision of additional time for test taking and written assignments, specific educational intervention to facilitate written expression and to teach note taking, and use of specific computer software to support written composition and spelling), and counseling should his depressed mood continue or worsen. (Adapted from case material from Rosemary Tannock, Ph.D.) Pathology and Laboratory Examination Whereas no physical signs of a writing disorder exist, educational testing is used in making a diagnosis of writing disorder. Diagnosis is based on a child’s writing performance being markedly below expected production for his age, as confirmed by an individually administered standardized expressive writing test. Currently available tests of written language include the Test of Written Language (TOWL), the DEWS, and the Test of Early Written Language (TEWL). Evaluation for impaired vision and hearing is recommended. When impairments in written expression are noted, a child should be administered a standardized intelligence test, such as WISC-R to determine the child’s overall intellectual capacity. Course and Prognosis Specific learning disorder with impairment in writing, reading, and mathematics often coexist, and additional language disorder may be present as well. A child with all of the above disabilities will likely be diagnosed with language disorder first and impaired written expression last. In severe cases, an impaired written expression is apparent by age 7 (second grade); in less severe cases, the disorder may not be apparent until age 10 (fifth grade) or later. Youth with mild and moderate impairment in written expression fare well if they receive timely remedial education early in grade school. Severely impaired written expression requires continual, extensive remedial treatment through the late part of high school and even into college. The prognosis depends on the severity of the disorder, the age or grade when the remedial intervention is started, the length and continuity of treatment, and presence or absence of associated or secondary emotional or behavioral problems. Differential Diagnosis It is important to determine whether disorders such as ADHD or major depression are interfering with a child’s focus and thereby preventing the production of adequate writing in the absence of a specific writing impairment. If true, treatment for the other disorder should improve a child’s writing performance. Commonly comorbid disorders with writing disability are language disorder, mathematics disorder, developmental coordination disorder, disruptive behavior disorders, and ADHD. Treatment Remedial treatment for writing disability includes direct practice in spelling and sentence writing as well as a review of grammatical rules. Intensive and continuous administration of individually tailored, one-on-one expressive and creative writing therapy appears to effect favorable outcome. Teachers in some special schools devote as much as 2 hours a day to such writing instruction. The effectiveness of a writing intervention depends largely on an optimal relationship between the child and the writing specialist. Success or failure in sustaining the patient’s motivation greatly affects the treatment’s long-term efficacy. Associated secondary emotional and behavioral problems should be given prompt attention, with appropriate psychiatric treatment and parental counseling. REFERENCES Archibald LMD, Cardy JO, Joanisse MF, Ansari D. Language, reading, and math learning profiles in an epidemiological sample of school age children. PLoS One. 2013;8:e77463. DOI: 10.1371/journal.pone.0077463. Badian NA. Persistent arithmetic, reading, or arithmetic and reading disability. Ann Dyslexia. 1999;49:43–70. Bergstrom KM, Lachmann T. Does noise affect learning? A short review on noise effects on cognitive performance in children. Front Psychol. 2013;4:578. Bernstein S, Atkinson AR, Martimianakis MA. Diagnosing the learner in difficulty. Pediatrics. 2013;132:210–212. Bishop DVM. Genetic influences on language impairment and literacy problems in children: Same or different? J Child Psychol Psychiatry. 2001;42:189–198. Butterworth B, Kovas Y. Understanding neurocognitive developmental disorders can improve education for all. Science. 2013;340:300–305. Catone WV, Brady SA. The inadequacy of Individual Educational Program (IEP) goals for high school students with wordlevel reading difficulties. Ann Dyslexia. 2005;55:53. Cragg L, Nation K. Exploring written narrative in children with poor reading comprehension. Educational Psychology. 2006;26:55–72. Endres M, Toso L, Roberson R, Park J, Abebe D, Poggi S, Spong CY. Prevention of alcohol-induced developmental delays and learning abnormalities in a model of fetal alcohol syndrome. Am J Obstet Gynecol. 2005;193:1028. Flax JF, Realpe-Bonilla T, Hirsch LS, Brzustowicz LM, Bartlett CW et al. Specific language impairment in families: Evidence for co-occurrence with reading impairments. J Speech Lang Hear Res. 2003:46:530–543. Fletcher JM. Predicting math outcomes: Reading predictors and comorbidity. J Learn Disabil. 2005;38:308. Gersten R, Jordan NC, Flojo JR. Early identification and interventions for students with mathematics difficulties. J Learn Disabil. 2005;38:305 Gordon N. The “medical” investigation of specific learning disorders. Pediatr Neurol. 2004;2(1):3. 13 - 31.8 Motor Disorders 31.8 Motor Disorders 14 - 31.8a Developmental Coordination Disorder 31.8a Developmental Coordination Disorder Hedges JH, Adolph KE, Amso D, Bavelier D, Fiez J, Krubitzer L, McAuley JD, Newcombe NS, Fitzpatrick SM, Ghajar J. Play, attention, and learning: How do play and timing shape the development of attention and influence classroom learning? Ann N Y Acad Sci. 2013;1292:1–20. Jura MB, Humphrey LH. Neuropsychological and cognitive assessment of children. In: Sadock BJ, Sadock VA, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Vol. 2. Philadelphia: Lippincott Williams & Wilkins; 2005;895. Lewis C, Hitch GJ, Peter W. The prevalence of specific arithmetic difficulties and specific reading difficulties in 9- to 10year-old boys and girls. J Child Psychol Psychiatry. 1994;35:283–292. Meeks J, Adler A, Kunert K, Floyd L. Individual psychotherapy of the learning-disabled adolescent. In: Flaherty LT, ed. Adolescent Psychiatry: Developmental and Clinical Studies. Vol. 28. Hillsdale, NJ: Analytic Press; 2004:231. Plomin R, Kovas Y. Generalist genes and learning disabilities. Psychol Bull. 2005;131:592. Tannock R. Reading disorder. In: Sadock BJ, Sadock VA, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Vol. 2. Philadelphia: Lippincott Williams & Wilkins; 2005:3107. Tannock R. Mathematics disorder. In: Sadock BJ, Sadock VA, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 8th ed. Vol. 2. Philadelphia: Lippincott Williams & Wilkins; 2005:3116. Tannock R. Disorder of written expression and learning disorder not otherwise specified. In: Sadock BJ, Sadock VA, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 8th ed. Vol. 2. Philadelphia: Lippincott Williams & Wilkins; 2005:3123. Vadasy PF, Sanders EA, Peyton JA. Relative effectiveness of reading practice or word-level instruction in supplemental tutoring: how text matters. J Learn Disabil. 2005;38:364. 31.8 Motor Disorders 31.8a Developmental Coordination Disorder Developmental coordination disorder is a neurodevelopmental disorder in which a child’s fine and/or gross motor coordination is slower, less accurate, and more variable than in peers of the same age. Affecting about 5 to 6 percent of school-age children, 50 percent of children with developmental coordination disorder also have comorbid attention-deficit/hyperactivity disorder (ADHD) or dyslexia. A meta-analysis of recent research on developmental coordination disorder concluded that three general areas of deficits contribute to the disorder: (1) Poor predictive control of motor movements; (2) deficits in rhythmic coordination and timing; and (3) deficits in executive functions, including working memory, inhibition, and attention. Children with developmental motor coordination struggle to perform accurately the motor activities of daily life, such as jumping, hopping, running, or catching a ball. Children with coordination problems may also agonize to use utensils correctly, tie their shoelaces, or write. A child with developmental coordination disorder may exhibit delays in achieving motor milestones, such as sitting, crawling, and walking, because of clumsiness, and yet excel at verbal skills. Developmental coordination disorder, thus, may be characterized by either clumsy gross and/or fine motor skills, resulting in poor performance in sports and even in academic achievement because of poor writing skills. A child with developmental coordination disorder may bump into things more often than siblings or drop things. In the 1930s, the term clumsy child syndrome began to be used in the literature to denote a condition of awkward motor behaviors that could not be correlated with any specific neurological disorder or damage. This term continues to be used to identify imprecise or delayed gross and fine motor behavior in children, resulting in subtle motor inabilities, but often significant social rejection. Gross and fine motor impairment in developmental coordination disorder cannot be explained on the basis of a medical condition, such as cerebral palsy, muscular dystrophy, or a neuromuscular disorder. Currently, certain indications are that perinatal problems, such as prematurity, low birth weight, and hypoxia may contribute to the emergence of developmental coordination disorders. Children with developmental coordination disorder are at higher risk for language and learning disorders. A strong association is seen between speech and language problems and coordination problems, as well as an association of coordination difficulties with hyperactivity, impulsivity, and poor attention span. Children with developmental coordination disorder may resemble younger children because of their inability to master motor activities typical for their age group. For example, children with developmental coordination disorder in elementary school may not be adept at bicycle riding, skateboarding, running, skipping, or hopping. In the middle school years, children with this disorder may have trouble in team sports, such as soccer, baseball, or basketball. Fine motor skill manifestations of developmental coordination disorder typically include clumsiness using utensils and difficulty with buttons and zippers in the preschool age group. In older children, using scissors and more complex grooming skills, such as styling hair or putting on makeup, is difficult. Children with developmental coordination disorder are often ostracized by peers because of their poor skills in many sports, and they often have long-standing difficulties with peer relationships. Developmental coordination disorder is categorized in the Fifth Edition of the American Psychiatric Association’s Diagnostic and Statistical Manual of Mental Disorders (DSM-5) as a Motor Disorder, along with stereotypic movement disorder and tic disorders. EPIDEMIOLOGY The prevalence of developmental coordination disorder has been estimated at about 5 to 6 percent of school-age children. The male-to-female ratio in referred populations tends to show increased rates of the disorder in males, but schools refer boys more often for testing and special education evaluations. Reports in the literature of the male-to-female ratio have ranged from 3 to 1 to as high as 7 to 1; however, the most current estimates are approximately 2 males for every one female. COMORBIDITY Developmental coordination disorder is strongly associated with ADHD, specific learning disorder, particularly in reading, as well as language disorder. Children with coordination difficulties have higher than expected rates of language disorder, and studies of children with language disorder report very high rates of “clumsiness.” Developmental coordination disorder is also associated, but less strongly, with specific learning disorder with impairment in mathematics, and in written expression. A study of children with developmental coordination disorder reported that, although motor coordination is critical for accuracy in tasks that require speed, poor motor coordination is not directly correlated with degree of inattention. Thus, in children comorbid for ADHD and developmental coordination disorder, children with the most severe ADHD do not necessarily have the worst developmental coordination disorder. Functional neuroimaging, pharmacological, and neuroanatomical studies suggest that motor coordination depends on the integration of sensory input and an action response, not purely through sensorimotor function and higher level thinking. Investigations of comorbid developmental coordination disorder and ADHD are trying to ascertain whether this comorbidity is due to overlapping genetic factors. Peer relationship problems are common among children with developmental coordination disorders, because of rejection that often occurs along with their poor performance in sports and games that require good motor skill. Adolescents with coordination problems often exhibit poor self-esteem and academic difficulties. Recent studies underscore the importance of attention to both victimization of children and adolescents with developmental motor coordination by peers and the potential resulting damage to self-worth. Children and adolescents with developmental coordination disorder who are bullied have higher rates of poor self-esteem that often deserves clinical attention. ETIOLOGY The causes of developmental coordination disorder are believed to be multifactorial, and likely include both genetic and developmental factors. Risk factors postulated to contribute to this disorder include prematurity, hypoxia, perinatal malnutrition, and low birth weight. Prenatal exposure to alcohol, cocaine, and nicotine has also been hypothesized to contribute to both low birth weight and cognitive and behavioral abnormalities. Developmental coordination disorder rates of up to 50 percent have been reported in children born prematurely. Researchers have proposed that the cerebellum may be the neurological substrate for comorbid cases of developmental coordination disorder and ADHD. Neurochemical abnormalities and parietal lobe lesions have also been suggested to contribute to coordination deficits. Studies of postural control, that is, the ability to regain balance after being in motion, indicate that children with developmental coordination disorder who have adequate balance when standing still, are unable to accurately correct for movement, resulting in impaired balance, compared with other children. A study concluded that, in children with developmental coordination disorder, neural signals from the brain to particular muscles involved in balance, are neither being optimally sent or received. These findings have also implicated the cerebellum as a potential anatomical site for the dysfunction of developmental coordination disorder. Two mechanisms of developmental coordination disorder have been hypothesized for the disabilities of the disorder. The first one, called the automatization deficit hypothesis, suggests that, similar to dyslexia, children with developmental coordination disorder have difficulty developing automatic motor skills. The second hypothesis, the internal modeling deficit hypothesis, suggests that children with developmental coordination disorder are unable to perform the typical internal cognitive models that predict the sensory consequences of motor commands. In both scenarios, the cerebellum is believed to play an important role in motor coordination and in developmental coordination disorder. DIAGNOSIS The diagnosis of developmental coordination disorder depends on poor performance in activities requiring coordination for a child’s age and intellectual level. Diagnosis is based on a history of the child’s delay in achieving early motor milestones, as well as on direct observation of current deficits in coordination. An informal screen for developmental coordination disorder involves asking the child to perform tasks involving gross motor coordination (e.g., hopping, jumping, and standing on one foot); fine motor coordination (e.g., finger-tapping and shoelace tying); and hand-eye coordination (e.g., catching a ball and copying letters). Judgments regarding poor performance must be based on what is expected for a child’s age. A child who is mildly clumsy, but whose functioning is not impaired, does not qualify for a diagnosis of developmental coordination disorder. The diagnosis may be associated with below-normal scores on performance subtests of standardized intelligence tests and by normal or above-normal scores on verbal subtests. Specialized tests of motor coordination can be useful, such as the Bender Visual Motor Gestalt Test, the Frostig Movement Skills Test Battery, and the Bruininks-Oseretsky Test of Motor Development. The child’s chronological age must be taken into account, and the disorder cannot be caused by a neurological or neuromuscular condition. Examination, however, may occasionally reveal slight reflex abnormalities and other soft neurological signs. CLINICAL FEATURES The clinical signs suggesting the existence of developmental coordination disorder are evident as early as infancy in some cases, when a child begins to attempt tasks requiring motor coordination. The essential clinical feature is significantly impaired performance in motor coordination. The difficulties in motor coordination may vary with a child’s age and developmental stage (Table 31.8a-1). Table 31.8a-1 Manifestations of Developmental Coordination Disorder In infancy and early childhood the disorder may be manifested by delays in developmental motor milestones, such as turning over, crawling, sitting, standing, walking, buttoning shirts, and zipping up pants. Between the ages of 2 and 4 years, clumsiness appears in almost all activities requiring motor coordination. Affected children cannot hold objects and drop them easily, their gait may be unsteady, they often trip over their own feet, and they may bump into other children while attempting to go around them. Older children may display impaired motor coordination in table games, such as putting together puzzles or building blocks, and in any type of ball game. Although no specific features are pathognomonic of developmental coordination disorder, developmental milestones are frequently delayed. Many children with the disorder also have speech and language difficulties. Older children may have secondary problems, including academic difficulties, as well as poor peer relationships based on social rejection. It has been reported widely that children with motor coordination problems are more likely to have problems understanding subtle social cues and are often rejected by peers. A recent study indicated that children with motor difficulties were found to perform more poorly on scales that measure recognition of static and changing facial expressions of emotion. This finding is likely to be correlated to the clinical observations that children with motor coordination have difficulties in social behavior and peer relationships. Billy was brought for evaluation of suicidal ideation at 8 years of age, after complaining to his parents that he was being bullied by peers for being “bad” in sports, and that nobody liked him. He only had one friend who also laughed at him sometimes, because he always dropped the ball and he looked “funny” while running. He was so upset about being rejected by peers when he tried to play sports that he refused to go to physical education class. Instead, he voluntarily went to the school counselor’s office and stayed there until the period was over. Billy was already irritated because he had been diagnosed with ADHD and was on medication, and on top of that, he had difficulty with reading. Billy became so distraught that one day he told his school counselor that he wanted to kill himself. A developmental history revealed that had been delayed for sitting, which he finally did at 10 months of age, and he could not walk without falling over until 30 months of age. Billy’s parents were aware that he was very clumsy, but they believed that he would outgrow that. Even at 8 years of age, Billy’s parents reported that, during meals, Billy often spilled his drinks and was quite awkward when he used a fork. Some of his food typically fell off of his fork or spoon before it reached his mouth, and he had great difficulty using a knife and a fork. A comprehensive assessment of fine and gross motor skills demonstrated the following: Billy was able to hop, but he could not skip without briefly stopping after each step. Billy could stand with both feet together, but was unable to stand on tiptoe. Although Billy could catch a ball, he held a ball bounced to himself at chest level, and was unable to catch a ball bounced to him on the ground from a distance of 15 feet. Billy’s agility and coordination were measured with the Bruininks-Oseretsky Test of Motor Development, which revealed functioning levels commensurate with those of an average 6-year-old child. Billy was referred to a neurologist for a comprehensive evaluation, because he appeared to be generally weak, and his muscles seemed floppy. Neurological evaluation was negative for diagnosable neurological disorders, and his muscle strength was actually found to be normal, despite his appearance. Based on the negative neurological examination and the finding of the Bruininks-Oseretsky Test of Motor Development, Billy was given a diagnosis of developmental coordination disorder. Billy’s symptoms included mild hypotonia and fine motor clumsiness. After the diagnosis of developmental motor coordination was made, in addition to his already diagnosed ADHD and reading disorder, his treatment plan included private sessions with an occupational therapist who used perceptual-motor exercises to improve Billy’s fine motor skills, targeting particularly writing and use of utensils. A written request was made for an Individualized Educational Plan (IEP) evaluation from the school with a goal of obtaining an adaptive physical education program. In addition, the request for a reading tutor, and a seat close to the front of the classroom were recommended to maximize his attention. Billy was enrolled in a treatment program using motor imagery training to reduce his clumsiness and improve coordination. Billy was relieved to be receiving help, especially for his reading and for sports activities, and no longer felt suicidal. Over a period of 3 months of treatment, Billy showed a noticeable improvement in his reading. His mood improved further, especially because he was receiving praise from his teachers and parents. Billy’s classmates were not picking on him the way they used to. As Billy began to feel better about himself, he began to play sports informally with his peers, although not competitively. Billy was granted an adaptive physical education program in school, and he was not required to play on teams. Instead, he practiced throwing and catching a ball and playing basketball with a staff member. Billy continued to show some degree of clumsiness, especially in his fine motor skills over the next few years, yet he was cooperative, with the occupational therapy interventions, his mood was bright, and he demonstrated continual improvement. (Courtesy of Caroly Pataki, M.D. and Sarah Spence, M.D.) DIFFERENTIAL DIAGNOSIS The differential diagnosis includes medical conditions that produce coordination difficulties (e.g., cerebral palsy and muscular dystrophy). In autism spectrum disorder and intellectual disability, coordination usually does not stand out as a significant deficit compared with other skills. Children with neuromuscular disorders may exhibit more global muscle impairment rather than clumsiness and delayed motor milestones. Neurological examination and workup usually reveal more extensive deficits in neurological conditions than in developmental coordination disorder. Extremely hyperactive and impulsive children may be physically careless because of their high levels of motor activity. Clumsy gross and fine motor behavior and ADHD as well as reading difficulties are highly associated. COURSE AND PROGNOSIS Historically, it was believed that developmental coordination spontaneously improved over time; however, longitudinal studies have shown that motor coordination problems can persist into adolescence and adulthood. When mild to moderate clumsiness is persistent, some children can compensate by developing interests in other skills. Some studies suggest a more favorable outcome for children who have average or aboveaverage intellectual capacity, in that they come up with strategies to develop friendships that do not depend on physical activities. Clumsiness typically persists into adolescence and adult life. One study following a group of children with developmental coordination problems over a decade found that the clumsy children remained less dexterous, showed poor balance, and continued to be physically awkward. The affected children were also more likely to have both academic problems and poor self-esteem. Children with developmental coordination disorder have also been shown to be at higher risk for obesity, have difficulties with running, and are at greater risk of future cardiovascular diseases. TREATMENT Interventions for children with developmental coordination disorder utilize multiple modalities, including visual, auditory, and tactile materials targeting perceptual motor training for specific motor tasks. Two broad categories of interventions are the following: (1) deficit-oriented approaches, including sensory integration therapy, sensorimotor-oriented treatment, and process-oriented treatment; and (2) task-specific interventions, including neuromotor task training and cognitive orientation to daily occupational performance (CO-OP). More recently, motor imagery training has been incorporated into treatment. These approaches involve visual imagery exercises using CD-ROM; they have a broad range of foci, including predictive timing for motor tasks, relaxation and mental preparation, visual modeling of fundamental motor skills, and mental rehearsal of various tasks. This type of intervention is based on the notion that improved internal representation of a movement task will improve a child’s actual motor behavior. The treatment of developmental coordination disorder generally includes versions of sensory integration programs and modified physical education. Sensory integration programs, usually administered by occupational therapists, consist of physical activities that increase awareness of motor and sensory function. For example, a child who bumps into objects often might be given the task of trying to balance on a scooter, under supervision, to improve balance and body awareness. Children who have difficulty writing letters are often given tasks to increase awareness of hand movements. Schoolbased occupational therapies for motor coordination problems in writing include utilizing mechanisms that provide resistance or vibration during writing exercises, to improve grip, and practicing vertical writing on a chalk board to increase arm strength and stability while writing. These programs have been shown to improve legibility of student’s writing, but not necessarily speed, because students learn to write with greater accuracy and deliberate letter formation. Currently, many schools also allow and may even encourage children with coordination difficulties that affect writing to use computers to aid in writing reports and long papers. Adaptive physical education programs are designed to help children enjoy exercise and physical activities without the pressures of team sports. These programs generally incorporate certain sports actions, such as kicking a soccer ball or throwing a basketball. Children with coordination disorder may also benefit from social skills groups and other prosocial interventions. The Montessori technique may promote motor skill development, especially with preschool children, because this educational program emphasizes the development of motor skills. Small studies have suggested that exercise in rhythmic coordination, practicing motor movements, and learning to use word processing keyboards may be beneficial. Parental counseling may help reduce parents’ anxiety and guilt about their child’s impairment, increase their awareness, and facilitate their confidence to cope with the child. An investigation of children with developmental coordination disorder showed positive results using a computer game designed to improve ability to catch a ball. These children were able to improve their game score by practicing virtual catching without specific instructions on how to utilize the visual cues. This has implications for 15 - 31.8b Stereotypic Movement Disorder 31.8b Stereotypic Movement Disorder treatment, in that certain types of motor task coordination can be positively influenced through the practice of specific motor tasks, even without overt instructions. REFERENCES Blank R, Smits-Engelsman B, Polatajko H, Wilson P. European Academy for Childhood Disability. European Academy of Childhood Disability: Recommendations on the definition, diagnosis and intervention of developmental coordination disorder (long version). Dev Med Child Neurol. 2012;54:54–93. Cairney J, Veldhuizen S, Szatmari P. Motor coordination and emotional-behavioral problems in children. Curr Opin Psychiatry. 2010;23:324–329. Deng S, Li WG, Ding J, Wu J, Shang Y, Li F, Shen X. Understanding the mechanisms of cognitive impairments in developmental coordination disorder. Pediatr Res. 2014;(210–216). Dewey D, Bottos S. Neuroimaging of developmental motor disorders. In: Dewey D, Tupper DE, eds. Developmental Motor Disorders: A Neuropsychological perspective. New York: Guilford Press; 2004:26. Edwards J, Berube M, Erlandson K. Developmental coordination disorder in school-aged children born very preterm and/or at very low birth weight: A systematic review. J Dev Behav Pediatr. 2011;32:678–687. Geuze RH. Postural control in children with developmental coordination disorder. Neural Plast. 2005;12:183. Groen SE, de Blecourt ACE, Postema K, Hadders-Algra M. General movements in early infancy predict neuromotor development at 9 to 12 years of age. Dev Med Child Neurol. 2005;47(11):731. Kargerer FA, Cfontreras-Vidal JL, Bo J, Clark JE. Abrupt, but not gradual visuomotor distortion facilitates adaptation in children with developmental coordination disorder. Mov Sci. 2006;25:622–633. Liberman L, Ratzon N, Bart O. The profile of performance skills and emotional factors in the context of participation among young children with developmental coordination disorder. Res Dev Disabil. 2013;34:87–94. Pataki CS, Mitchell WG. Motor skills disorder: Developmental coordination disorder. In: Sadock BJ, Sadock VA, Ruiz P, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Vol. II. Philadelphia: Lippincott Williams & Wilkins; 2009:3501. Williams J, Thomas PR, Maruff P, Butson M, Wilson PH. Motor, visual and egocentric transformations in children with developmental coordination disorder. Child Care Health Dev. 2006;32:633–647. Wilson PH, Ruddock S, Smits-Engelsman B, Polatajko H. Understanding performance deficits in developmental coordination disorder: A meta-analysis of recent research. Dev Med Child Neurol. 2013;55:217–228. Zwicker JG, Harris SR, Klassen AF. Quality of life domains affected in children with developmental coordination disorder: a systematic review. Child Care Health Dev. 2013;39:562–580. Zwicker JG, Missiuna C, Harris SR, Boyd LA. Developmental coordination disorder: A review and update. Eur J Paediatr Neurol. 2012;6:573–581. Zwicker JG, Missiuna C, Harris SR, Boyd LA. Brain activation associated with motor skill practice in children with developmental motor coordination disorder: An fMRI study. Int J Dev Neurosci. 2011;29:145–152. 31.8b Stereotypic Movement Disorder Stereotypic movements include a diverse range of repetitive behaviors that usually emerge in the early developmental period, appear to lack a clear function, and sometimes cause interruption in daily life. These movements are typically rhythmic, such as hand flapping, body rocking, hand waving, hair-twirling, lip-licking, skin picking, or self-hitting. Stereotypic movements often appear to be self-soothing or selfstimulating; however, they can result in self-injury in some cases. Stereotypic movements appear to be involuntary; however, they frequently can be suppressed with a concentrated effort. Stereotypic movement disorder occurs with increased frequency in children with autism spectrum disorder and intellectual disability, but they also exist in typically developing children. Stereotypic movements, such as head-banging, face slapping, eye poking, or hand-biting, can cause significant self-harm. Nail-biting, thumb-sucking, and nose-picking are often not included as symptoms of stereotypic movement disorder because they rarely cause impairment. When impairment occurs, however, they can be included in stereotypic movement disorder. Stereotypic movements share several features with tics, including the repetitive, seemingly involuntary, and characteristically identical nature of the movements each time they are displayed. However, distinguishing features of stereotypical movements compared to tics include a younger age of onset, lack of changing anatomical locations, lack of premonitory “urge,” and decreased response to medication management. According to the Fifth Edition of the American Psychiatric Association’s Diagnostic and Statistical Manual of Mental Disorders (DSM-5), stereotypic movement disorder is characterized by repetitive, seemingly driven, and apparently purposeless motor behavior that interferes with social, academic, or other activities and may result in selfharm. EPIDEMIOLOGY Repetitive movements are common in infants and young children, with greater than 60 percent of parents of children between the ages of 2 and 4 years reporting transient emergence of these behaviors. The most frequent age of onset is in the second year of life. Epidemiologic surveys estimate that up to 7 percent of otherwise typically developing children exhibit stereotypic behaviors. A prevalence of about 15 to 20 percent in children younger than the age of 6 years display stereotypic behavior, with diminishing rates over time. The prevalence of self-injurious behaviors, however, has been estimated to be in the range of 2 to 3 percent among children and adolescents with intellectual disability. Stereotypic movements appear to occur in about twice as many boys as girls. Determining which cases are sufficiently severe to confirm a diagnosis of stereotypic movement disorder may be difficult. Stereotypic behaviors occur in 10 to 20 percent of children with intellectual disability, with increased rates being proportional to level of severity. Self-injurious behaviors frequently occur in genetic syndromes, such as Lesch-Nyhan syndrome, and in children with sensory impairments, such as blindness and deafness. ETIOLOGY The etiology of stereotypic movement disorder includes environmental, genetic, and neurobiological factors. Although the neurobiological mechanisms of stereotypic movement disorder have yet to be proven, given their similarity to other involuntary movements, stereotypic movement disorder is hypothesized to originate from the basal ganglia. Dopamine and serotonin are likely to be involved in their emergence. Dopamine agonists tend to induce or increase stereotypic behaviors, whereas dopamine antagonists sometimes decrease them. One study found that 17 percent of typically developing children with stereotypic movement disorder had a first-degree relative with the disorder, and 25 percent had a first- or second-degree relative with stereotypic movement disorder. Transient stereotypic behaviors in very young children can be considered a normal developmental phenomenon. Genetic factors likely play a role in some stereotypic movements, such as the X-linked recessive deficiency of enzymes leading to Lesch-Nyhan syndrome, which has predictable features including intellectual disability, hyperuricemia, spasticity, and self-injurious behaviors. Other minimal stereotypic movements that do not usually cause impairment (e.g. nail-biting) appear to run in families as well. Some stereotypic behaviors seem to emerge or become exaggerated in situations of neglect or deprivation; such behaviors as head-banging have been associated with psychosocial deprivation. DIAGNOSIS AND CLINICAL FEATURES The presence of multiple repetitive stereotyped symptoms tends to occur frequently among children with autism spectrum disorder and intellectually disability, particularly when the intellectual disability is severe. Patients with multiple stereotyped movements frequently have other significant mental disorders, including disruptive behavior disorders, or neurological conditions. In extreme cases, severe mutilation and lifethreatening injuries can result from self-inflicted trauma. Head-Banging Head-banging exemplifies a stereotypic movement disorder that can result in functional impairment. Typically, head-banging begins during infancy, between 6 and 12 months of age. Infants strike their heads with a definite rhythmic and monotonous continuity against the crib or another hard surface. They seem to be absorbed in the activity, which can persist until they become exhausted and fall asleep. The head-banging is often transitory, but sometimes persists into middle childhood. Head-banging that is a component of temper tantrums differs from stereotypic head-banging and ceases after the tantrums and their secondary gains have been controlled. Nail-Biting Nail-biting begins as early as 1 year of age and increases in incidence until age 12. Most cases are not sufficiently severe to meet the DSM-5 diagnostic criteria for stereotypic movement disorder. In rare cases, children cause physical damage to the fingers themselves, usually by associated biting of the cuticles, which leads to secondary infections of the fingers and nail beds. Nail-biting seems to occur or increase in intensity when a child is either anxious or stressed. Some of the most severe nail-biting occurs in children with severe or profound intellectual disability, however many nail-biters have no obvious emotional disturbance. PATHOLOGY AND LABORATORY EXAMINATION No specific laboratory measures are helpful in the diagnosis of stereotypic movement disorder. Tim, a 14-year-old with autism spectrum disorder (ASD), and severe intellectual disability was evaluated when he transferred to a new private school for children with ASD. Observed in his classroom, he was noted to be a small boy who appeared younger than his age. He held his hands in his pockets and spun around in place. When offered a toy he took it and manipulated it for a while. When he was prompted to engage in various tasks that required that he take his hands out of his pockets, he began hitting his head with his hands. If his hands were held by the teacher, he hit his head with his knees. He was adept in contorting himself, so that he could hit or kick himself in almost any position, even while walking. Soon, his face and forehead were covered with bruises. His development was delayed in all spheres, and he never developed language. He lived at home and attended a special educational program. His self-injurious behaviors developed early in life, and, when his parents tried to stop him, he became aggressive. Gradually, he became too difficult to be managed in public school, and, at 5 years of age, he was placed in a special school. The self-abusive and self-restraining (i.e., holding his hands in his pockets) behavior was present throughout his stay there, and, virtually all of the time; he had been tried on several second-generation antipsychotics with only minimal improvement. Although the psychiatrist’s notes mentioned some improvement in his self-injurious behavior, it was described as continuing and fluctuating. He was transferred to a new school because of lack of progress and difficulties in managing him as he became bigger and stronger. His intellectual functioning was within the 34 to 40 intelligence quotient (IQ) range. His adaptive skills were poor. He required full assistance in self-care, could not provide even for his own simple needs, and required constant supervision for his safety. In a few months, Tim settled into the routine in his new school. His self-injurious behavior fluctuated. It was reduced or even absent when he restrained himself by holding his hands in his pockets or inside his shirt or even by manipulating some object with his hands. If left to himself, he could contort himself, while holding his hands inside his shirt. Because the stereotypic self-injurious and self-restraining behavior interfered with his daily activities and education, it became a primary focus of a behavior modification program. For a few months, he did well, especially when he developed a good relationship with a new teacher, who was firm, consistent, and nurturing. With him, Tim could successfully engage in some school tasks. When the teacher left, Tim regressed. To prevent injuries, the staff started blocking his self- hitting with a pillow. He was offered activities that he liked and in which he could engage without resorting to self-injury. After several months, his antipsychotic medication was slowly discontinued, over a period of 11 months, without any behavioral deterioration. (Adapted from case material from Bhavik Shah, M.D.) DIFFERENTIAL DIAGNOSIS The differential diagnosis of stereotypic movement disorder includes obsessivecompulsive disorder (OCD) and tic disorders, both of which are exclusionary criteria in DSM-5. Although stereotypic movements can often be voluntarily suppressed, and are not spasmodic, it is difficult to differentiate these features from tics in all cases. A study of stereotyped movements compared with tics found that stereotyped movements tended to be longer in duration, and displayed more rhythmic qualities than tics. Tics seemed to occur more when a child was in an “alone” condition, rather than when the child was in a play condition, whereas stereotypic movements occurred with the same frequency in these two different conditions. Stereotypic movements are often observed to seem selfsoothing, whereas tics are often associated with distress. Differentiating dyskinetic movements from stereotypic movements can be difficult. Because antipsychotic medications can sometimes suppress stereotypic movements, clinicians should note any stereotypic movements before initiating treatment with an antipsychotic agent. Stereotypic movement disorder may be diagnosed concurrently with substance-related disorders (e.g., amphetamine use disorders), severe sensory impairments, central nervous system and degenerative disorders (e.g., Lesch-Nyhan syndrome), and severe schizophrenia. COURSE AND PROGNOSIS The duration and course of stereotypic movement disorder vary, and the symptoms may wax and wane. Up to 60 to 80 percent of normal toddlers show transient rhythmic activities that seem purposeful and comforting and tend to disappear by 4 years of age. When stereotypic movements emerge more severely later in childhood they typically range from brief episodes occurring under stress, to an ongoing pattern in the context of a chronic condition, such as ASD or intellectual disability. Even in chronic conditions, stereotypic behaviors may come and go. In many cases, stereotypic movements are prominent in early childhood and diminish as a child gets older. The severity of the dysfunction caused by stereotypic movements varies with the frequency, amount, and degree of associated self-injury. Children who exhibit frequent, severe, self-injurious stereotypic behaviors have the poorest prognosis. Repetitive episodes of head-banging, self-biting, and eye-poking can be difficult to control without physical restraints. Most nail-biting is benign and often does not meet the diagnostic criteria for stereotypic movement disorder. In severe cases in which the nail beds are repetitively damaged, bacterial and fungal infections can occur. Although chronic stereotypic movement disorders can severely impair daily functioning, several treatments help control the symptoms. TREATMENT When stereotypic movements occur in the absence of any other symptoms or disorders, they may not warrant pharmacologic treatment. Treatment modalities yielding the most promising effects include behavioral techniques, such as habit reversal and differential reinforcement of other behavior, as well as pharmacological interventions. A recent report on utilizing both habit reversal (in which the child is trained to replace the undesired repetitive behavior with a more acceptable behavior) and reinforcement for reducing the unwanted behavior, indicated that these treatments had efficacy among 12 typically developing children between 6 and 14 years. One case report detailed a successful habit reversal treatment of a 3-year-old with severe stereotypic movements, which was largely implemented at home by her parents. The estimated change in stereotypic behaviors during regular recorded intervals during treatment diminished from presence in 85 percent of recordings to presence in less than 2 percent of recordings over a period of 4 weeks. Pharmacological interventions have been used in clinical practice to minimize selfinjury in children whose stereotyped movements caused significant harm to their bodies. Small open-label studies have reported benefit of atypical antipsychotics, and case reports have indicated use of selective serotonin reuptake inhibitor (SSRIs) in the management of self-injurious stereotypies. The dopamine receptor antagonists have been tried most often for treating stereotypic movements and self-injurious behavior. The SSRI agents may be influential in diminishing stereotypies; however, this is still under investigation. Open trials suggest that both clomipramine and fluoxetine may decrease self-injurious behaviors and other stereotypic movements in some patients. REFERENCES Barry S, Baird G, Lascelles K, Bunton P, Hedderly T. Neurodevelopmental movement disorders—an update on childhood motor stereotypies. Dev Med Child Neurol. 2011;53:979–985. Doyle RL. Stereotypic movement disorders. In: Sadock BJ, Sadock VA, Ruiz P, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Vol. II. Philadelphia: Lippincott Williams & Wilkins; 2009:3642. Edwards MJ, Lang AE, Bhatia KP. Stereotypies: A critical appraisal and suggestion of a clinically useful definition. Mov Disord. 2012;27:179–185. Fernandez AE. Primary versus secondary stereotypic movements. Rev Neurol. 2004; 38[Suppl 1]:21. Freeman KA, Duke DC. Power of magic hands: Parent-driven application of habit reversal to treat complex stereotypy in a 3-year-old. Health Psychol. 2013;32:915–920. Freeman RD, Soltanifar A, Baer S. Stereotypic movement disorder: Easily missed. Dev Med Child Neurol. 2010;52:733–738. Harris KM, Mahone EM, Singer HS. Nonautistic motor stereotypies: Clinical features and longitudinal follow-up. Pediatr Neurol. 2008;38:267–272. Luby JL. Disorders of infancy and early childhood not otherwise specified. In: Sadock BJ, Sadock VA, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 8th ed. Vol. 2. Philadelphia: Lippincott Williams & Wilkins; 2005:3257. Mahone EM, Bridges D, Prahme C, Singer HS. Repetitive arm and hand movements (complex motor stereotypies) in 16 - 31.8c Tourettes Disorder 31.8c Tourette’s Disorder children. J Pediatr. 2004;145:391. Melnick SM, Dow-Edwards DL. Correlating brain metabolism with stereotypic and locomotor behavior. Behav Res Methods Instrum Comput. 2003;35:452. Miller JM, Singer HS, Bridges DD, Waranch HR. Behavioral therapy for treatment of stereotypic movements in nonautistic children. J Child Neurol. 2006;21:119. Muehlmann AM, Lewis MH. Abnormal repetitive behaviours: Shared phenomenology and pathophysiology. J Intellect Disabil Res. 2012; 56:427–440. Presti MF, Watson CJ, Kennedy RT, Yang M, Lewis MH. Behavior-related alterations of striatal neurochemistry in a mouse model of stereotyped movement disorder. Pharmacol Biochem Behav. 2004;77:501. Stein DJ, Grant JE, Franklin ME, Keuthen N, Lochner C, Singer HS, Woods DW. Trichotillomania (hair pulling disorder) skin picking disorder, and stereotypic movement disorder: Toward DSM-V. Dep Anxiety. 2010;27:611–626. Zinner SH, Mink JW. Movement disorders I: Tics and stereotypies. Pediatr Rev. 2010;31:223–232. 31.8c Tourette’s Disorder Tics are neuropsychiatric events characterized by brief rapid motor movements or vocalizations that are typically performed in response to irresistible premonitory urges. Although frequently rapid, tics may include more complex patterns of movements and longer vocalizations. Converging evidence from many lines of research suggests that the production of tics involves dysfunction in the basal ganglia region of the brain, particularly of dopaminergic transmission in the cortico-striatothalamic circuits. Because tic disorders are significantly more common in children than in adults, the postulated alterations in dopamine circuitry in many affected children appear to spontaneously improve over time. Tics may be transient or chronic, with a waxing and waning course. Tics typically emerge at age 5 to 6 years of age and tend to reach their greatest severity between 10 and 12 years. About one half to two thirds of children with tic disorders will be much improved or in remission by adolescence or early adulthood. Tic disorder is distinguished by the type of tics, their frequency, and the pattern in which they emerge over time. Motor tics most commonly affect the muscles of the face and neck, such as eye-blinking, head-jerking, mouth-grimacing, or head-shaking. Typical vocal tics include throat-clearing, grunting, snorting, and coughing. Tics are repetitive muscle contractions resulting in movements or vocalizations that are experienced as involuntary, although they can sometimes be suppressed voluntarily Children and adolescents may exhibit tic behaviors that occur after a stimulus or in response to a premonitory internal urge. The most widely studied and most severe tic disorder is Gilles de la Tourette syndrome, also known as Tourette’s disorder. Georges Gilles de la Tourette (1857–1904) first described a patient with a syndrome, which became known as Tourette’s disorder in 1885, while he was studying with Jean-Martin Charcot in France. De la Tourette noted a syndrome in several patients that included multiple motor tics, coprolalia, and echolalia. Tics often consist of motions that are used in volitional movements. One half to two thirds of children with Tourette’s disorder exhibit a reduction in or complete remission of tic symptoms during adolescence. There are many common comorbid psychiatric disorders and behavioral problems likely to emerge along with Tourette’s disorder. For example, the relationship between Tourette’s disorder, attentiondeficit/hyperactivity disorder (ADHD), and obsessive-compulsive disorder (OCD) has not been clearly delineated. Epidemiological surveys indicate that more than half of children with Tourette’s disorder also meet criteria for ADHD. There appears to be a bidirectional relationship between Tourette’s disorder and OCD, with 20 to 40 percent of Tourette’s disorder patients meeting full criteria for OCD. First-degree relatives of patients with OCD have been shown to have higher rates of tic disorders compared to the general population. There have been a few small reports suggesting that the obsessivecompulsive symptoms most likely to occur in Tourette’s disorder are characteristically related to ordering and symmetry, counting, and repetitive touching, whereas OCD symptoms in the absence of tic disorders are more often associated with fears of contamination and fears of doing harm. Motor and vocal tics are divided into simple and complex types. Simple motor tics are those composed of repetitive, rapid contractions of functionally similar muscle groups—for example, eye-blinking, neck-jerking, shoulder-shrugging, and facial-grimacing. Common simple vocal tics include coughing, throat-clearing, grunting, sniffing, snorting, and barking. Complex motor tics appear to be more purposeful and ritualistic than simple tics. Common complex motor tics include grooming behaviors, the smelling of objects, jumping, touching behaviors, echopraxia (imitation of observed behavior), and copropraxia (display of obscene gestures). Complex vocal tics include repeating words or phrases out of context, coprolalia (use of obscene words or phrases), palilalia (a person’s repeating his or her words), and echolalia (repetition of the last-heard words of others). Although older children and adolescents with tic disorders may be able to suppress their tics for minutes or hours, young children are often not cognizant of their tics or experience their urges to perform their tics as irresistible. Tics may be attenuated by sleep, relaxation, or absorption in an activity. Tics often disappear during sleep. EPIDEMIOLOGY The estimated prevalence of Tourette’s disorder ranges from 3 to 8 per 1,000 school-age children. Males are affected between 2 and 4 times more often than females. The unique features of Tourette’s disorder in which tics wax and wane and may change in character, frequency, and severity over relatively short periods of time, has made ascertainment of its prevalence challenging. Furthermore, remission of tics is particularly age-dependent in that tics tend to emerge and increase from ages 5 to 10 years of age, and in many cases, decrease in frequency and severity after the age of 10 to 12 years. At age 13 years, however, using stringent criteria, the prevalence rate for Tourette’s disorder drops to 0.3 percent. The lifetime prevalence of Tourette’s disorder is estimated to be approximately 1 percent. ETIOLOGY Genetic Factors Twin studies, adoption studies, and segregation analysis studies all support a genetic basis, albeit a complex one, for Tourette’s disorder. Twin studies indicate that concordance for the disorder in monozygotic twins is significantly greater than that in dizygotic twins. Tourette’s disorder and chronic motor or vocal tic disorder are likely to occur in the same families; this lends support to the view that the disorders are part of a genetically determined spectrum. The sons of mothers with Tourette’s disorder seem to be at the highest risk for the disorder. Evidence in some families indicates that Tourette’s disorder is transmitted in an autosomal dominant fashion. Studies of a long family pedigree suggest that Tourette’s disorder may be transmitted in a bilinear mode; that is, Tourette’s disorder appears to be inherited through an autosomal pattern in some families, intermediate between dominant and recessive. A study of 174 unrelated probands with Tourette’s disorder identified a greater than chance occurrence of a rare sequence variant in SLITRK1, believed to be a candidate gene on chromosome 13q31. Up to half of all patients with Tourette’s disorder also have ADHD, and up to 40 percent of those with Tourette’s disorder also have OCD. These frequent comorbidities with Tourette’s disorder can lead to a plethora of overlapping symptoms. Family studies have provided compelling evidence for the association between tic disorders and OCD. First-degree relatives of persons with Tourette’s disorder are at high risk for the development of Tourette’s disorder, chronic motor or vocal tic disorder, and OCD. Current understanding of the genetic bases of Tourette’s disorder implicates multiple vulnerability genes that may serve to mediate the type and severity of tics. Candidate genes associated with Tourette’s disorder include dopamine receptor genes, dopamine transporter genes, several noradrenergic genes, and serotonergic genes. Neuroimaging Studies A functional magnetic resonance imaging (fMRI) study of brain activity two seconds before and after a tic, found that paralimbic and sensory association areas were involved. Furthermore, evidence suggests that voluntary tic suppression involves deactivation of the putamen and globus pallidus, along with partial activation of regions of the prefrontal cortex and caudate nucleus. Compelling, but indirect, evidence of dopamine system involvement in tic disorders includes the observations that pharmacological agents that antagonize dopamine (haloperidol [Haldol], pimozide [Orap], and fluphenazine [Prolixin]) suppress tics and that agents that increase central dopaminergic activity (methylphenidate [Ritalin], amphetamines, and cocaine) tend to exacerbate tics. The relation of tics to neurotransmitter systems is complex and not yet well understood; for example, in some cases, antipsychotic medications, such as haloperidol, are not effective in reducing tics, and the effect of stimulants on tic disorders reportedly varies. In some cases, Tourette’s disorder has emerged during treatment with antipsychotic medications. More direct analyses of the neurochemistry of Tourette’s disorder have been possible utilizing brain proton magnetic resonance spectroscopy (MRS). Neuroimaging studies using cerebral blood flow in positron emission tomography (PET) and single photon emission tomography (SPECT) suggest that alterations of activity may occur in various brain regions in patients with Tourette’s disorder compared to controls, including the frontal and orbital cortex, striatum, and putamen. An investigation examining the cellular neurochemistry of patients with Tourette’s disorder utilizing MRS of the frontal cortex, caudate nucleus, putamen, and thalamus demonstrated that these patients had a reduced amount of choline and N-acetylaspartate in the left putamen along with reduced levels bilaterally in the putamen. In the frontal cortex, patients with Tourette’s disorder were found to have lower concentrations of N-acetylaspartate bilaterally, lower levels of creatine on the right side, and reduced myoinositol on the left side. These results suggest that deficits in the density of neuronal and nonneuronal cells are present in patients with the disorder. Abnormalities in the noradrenergic system have been implicated in some cases by the reduction of tics with clonidine (Catapres). This adrenergic agonist reduces the release of norepinephrine in the central nervous system and, thus, may reduce activity in the dopaminergic system. Abnormalities in the basal ganglia are known to result in various movement disorders, such as Huntington’s disease, and are also implicated as likely sites of disturbance in Tourette’s disorder. Immunological Factors and Post Infection An autoimmune process and, in particular, one that is secondary to group A betahemolytic streptococcal infections was hypothesized as a potential mechanism for the development of tics and obsessive-compulsive symptoms in some case. Data have been conflicting and controversial, and this mechanism appears to be unlikely as an etiology of Tourette’s disorder in most cases. One case-control study found little evidence of the development or exacerbation of tics, or obsessions or compulsions, in children with welldocumented and treated group A beta-hemolytic streptococcal infections. DIAGNOSIS AND CLINICAL FEATURES A diagnosis of Tourette’s disorder depends on a history of multiple motor tics that generally emerge over a period of months or years, and the emergence of at least one vocal tic at some point. According to the American Psychiatric Association’s Fifth Edition of the Diagnostic and Statistical Manual of Mental Disorders (DSM-5), tics may wax and wane in frequency, but must have persisted for more than a year since the first tic emerged to meet the diagnosis. The average age of onset of tics is between 4 years and 6 years of age, although in some cases, tics may occur as early as 2 years of age. The peak age for severity of tics is between 10 and 12 years. To meet diagnostic criteria for Tourette’s disorder, the onset must occur before the age of 18 years. In Tourette’s disorder, typically the initial tics are in the face and neck. Over time, the tics tend to occur in a downward progression. The most commonly described tics are those affecting the face and head, the arms and hands, the body and lower extremities, and the respiratory and alimentary systems. In these areas, the tics take the form of grimacing; forehead puckering; eyebrow-raising; eyelid-blinking; winking; nosewrinkling; nostril-trembling; mouth-twitching; displaying the teeth; biting the lips and other parts; tongue-extruding; protracting the lower jaw; nodding, jerking, or shaking the head; twisting the neck; looking sideways; head-rolling; hand-jerking; arm-jerking; plucking fingers; writhing fingers; fist-clenching; shoulder-shrugging; foot, knee, or toe shaking; walking peculiarly; body writhing; jumping; hiccupping; sighing; yawning; snuffing; blowing through the nostrils; whistling; belching; sucking or smacking sounds; and clearing the throat. Several assessment instruments are currently available that are useful in making diagnoses of tic disorders, including comprehensive self-report assessment tools, such as the Tic Symptom Self Report and the Yale Global Tic Severity Scale, administered by a clinician (Table 31.8c-1). Table 31.8c-1 Clinical Assessment Tools in Tic Disorders Because Tourette’s disorder is frequently comorbid with attentional, obsessional, and oppositional behaviors, these symptoms often emerge prior to the tics. In some studies, more than 25 percent of children with Tourette’s disorder received stimulants for a diagnosis of ADHD before receiving a diagnosis of Tourette’s disorder. The most frequent initial symptom is an eye-blink tic, followed by a head tic or a facial grimace. Most complex motor and vocal symptoms emerge several years after the initial symptoms. Coprolalia, a very unusual symptom involving shouting or speaking socially unacceptable or obscene words, occurs in less than 10 percent of patients and rarely in the absence of comorbid psychiatric disturbance. Mental coprolalia—in which a patient experiences a sudden, intrusive, socially unacceptable thought or obscene word—occurs more often than coprolalia. In severe cases, physical self-injury has occurred due to tic behaviors. Jake, age 10 years, came to the Tourette Disorder Clinic for an evaluation of motor tics in the head and neck, occasional coughing and grunting, and a new symptom of throat clearing many times per day. Jake had a past history of ADHD, which included significant hyperactivity, and impulsive and oppositional behavior He is a fifth-grade student in a regular class at the local public school. Before the consultation, parent and teacher ratings, including the Child Behavior Checklist (CBCL), Swanson, Nolan, and Pelham-IV (SNAP-IV), Conners’ Parent and Teacher Questionnaires, Tic Symptom SelfReport (TSSR), and medical history survey, were sent to his family. His mother and the classroom teacher rated him well above the norm for hyperactivity, inattention, and impulsiveness. He was failing several subjects in school, often argued with adults, was occasionally aggressive, and had few friends. His tics were rated as moderate. Jake’s mother recalls difficulties with overactivity, oppositional and defiant behaviors and behavior since preschool. At age 5, due to his activity level and argumentative and aggressive behavior, his kindergarten teacher encouraged the family to obtain a psychiatric consultation. Jake’s pediatrician made a diagnosis of ADHD and recommended a trial of Concerta (methylphenidate extended-release tablets) at 36 mg per day, which was started at the beginning of the first grade. Within a week of starting medication, Jake’s overly active and impulsive behavior showed a dramatic improvement; however, he remained argumentative and oppositional. However, when on his Concerta, Jake was able to stay in his seat and complete his work and was better able to wait his turn on the playground. The next few months went well, however, by early spring, Jake seemed to be returning back to some of his old ways. He was talking out of turn in class, and getting out of his seat, which was disruptive to the class. After an increase in Concerta to 54 mg per day, in the spring of his first-grade year, however, he began showing motor and phonic tics consisting of head-jerking, facial movements, coughing, and grunting. The Concerta was discontinued to see if this made a difference and was immediately stopped and, although the tics transiently decreased, they came back in full force within a month. In hindsight, Jake’s mother recalled that Jake had exhibited eye blinking and grunting prior to starting the Concerta, but she had dismissed these events as unimportant and they did not seem to disrupt Jake’s daily life. While Jake was off Concerta during a period when he began middle school in the 6th grade, Jake was disruptive to his classes and he began to be severely teased by several classmates for his impulsivity, frequent motor tics, and loud grunting and throat clearing. Jake became despondent and began to refuse to go to school. At this point, it was decided to place Jake in a special education class. However, after several months of this placement, Jake felt worse about himself, despised school, and begged to be returned to regular classes. At this point Jake’s pediatrician made the referral to a child and adolescent psychiatrist at a local university Tourette Disorder Clinic. During his evaluation at the Tourette Disorder Clinic, Jake was reported to be a healthy child who was the product of an uncomplicated pregnancy, labor, and delivery, and whose developmental milestones were achieved at appropriate times. Intellectual testing completed by the school psychologist revealed a full scale IQ of 105. Jake’s mother noted that Jake has had long-standing trouble falling asleep but sleeps through the night. Jake has always been described as argumentative and easily frustrated with frequent outbursts of temper; however, when he is not having a tantrum, his mood is generally upbeat. Jake was noted by the child and adolescent psychiatrist to be of average height and weight with no dysmorphic features. His speech was rapid in tempo but normal in tone and volume. His speech is coherent and developmentally appropriate, without evidence of thought disorder; however, vocal tics including grunting, coughing, and obvious throat clearing were observed. Jake denied depressed mood or suicidal ideation, although he reported distress about everyday issues such as being teased by peers, not having enough friends, and his poor school performance. Jake also denied recurring worries about contamination or harm coming to him or family members, or fears of acting on unwanted impulses. Other than mild touching habits involving the need to touch objects with each hand three times or in combinations of three, Jake denies repetitive rituals. Several motor tics were also observed during the evaluation session, including blinking, head-jerking, and shoulder tics. Jake was restless and easily distracted throughout the session and often needed assistance with entertaining himself when not directly involved in conversation. Given the history of enduring motor and phonic tics, confirmed by direct observation, the diagnosis of Tourette’s disorder and ADHD, as well as oppositional defiant disorder were confirmed. Jake and his family attended several sessions with the child and adolescent psychiatrist to learn about the waxing and waning nature of tic symptoms and the natural history of Tourette’s Disorder, as well as ADHD. Jake and his family were heartened to hear that, in general, tics tend to be at their maximum around his age, and it was somewhat likely that Jake’s tics would lessen over time or possibly fully remit. Jake was referred to a behavioral psychologist specializing in habit reversal training. In this treatment Jake was taught to engage in a behavior physically incompatible with his tic (a competing response) each time he experienced the urge to perform this tic. The competing response for Jake’s shoulder tic, which consisted of raising his shoulders up as far as he could, was to gently press his shoulders down and extend his neck each time he felt the urge to engage in this tic. With repeated practice of his competing response, Jake’s urge to engage in this tic greatly diminished to the point where he was able to manage the urge without performing the tic. Jake was referred to a child and adolescent psychiatrist who decided to re-start the Concerta at 36 mg per day and titrated it back up to 54 mg per day without worsening of the tics. Jake responded well to his behavioral therapy, and over a period of 8 weeks, he had learned how to become aware of the urges that occurred prior to his tics and to voluntarily replace his usual tics with less-distressing and less-disruptive behaviors. However, when Jake entered the 7th grade, he had an exacerbation of his motor and vocal tics, and was also touching objects repeatedly throughout the day. Jake again became despondent, not wanting to go to school. It was decided by his psychologist to add relaxation training to his behavioral treatment, and his child and adolescent psychiatrist another medication to his pharmacological regimen. Jake was prescribed risperidone, 0.5 mg per day, which was titrated up to 1 mg twice daily. With the addition of these psychological and pharmacological interventions, Jake became stabilized within a month, and was able to continue in his school and even went to some parties. Jake and his parents understood the waxing and waning nature of his tics, and were hopeful that they would begin to see some decrease in his tic symptoms within the next few years. At follow-up, when Jake was 15 years of age, Jake had minimal tic symptoms; an occasional eye blink and rare throat clearing was all that was observable. Jake was not currently in behavioral treatment, however, over the years, he had, on a few occasions received some booster therapy sessions to brush up on his habit reversal training when he had a minor exacerbation of tics. Jake had been taken off his risperidone a 2 years before without an exacerbation of tics. Jake continued on Concerta 54 mg per day and was well controlled on that dose, was doing well in school, and had become more popular since he had joined the soccer team. (Adapted from L. Scahill M.S.N., Ph.D. and J.F. Leckman, M.D.) PATHOLOGY AND LABORATORY EXAMINATION No specific laboratory diagnostic test exists for Tourette’s disorder, but many patients with Tourette’s disorder have nonspecific abnormal electroencephalographic findings. Computed tomography (CT) and magnetic resonance imaging (MRI) scans have revealed no specific structural lesions, although about 10 percent of all patients with Tourette’s disorder show some nonspecific abnormality on CT scans. DIFFERENTIAL DIAGNOSIS Tics must be differentiated from other movements and movement disorders (e.g., dystonic, choreiform, athetoid, myoclonic, and hemiballismic movements) and the neurological diseases that they may characterize (e.g., Huntington’s disease, parkinsonism, Sydenham’s chorea, and Wilson’s disease), as listed in Table 31.8c-2. Tremors, mannerisms, and stereotypic movement disorder (e.g., head-banging or bodyrocking) must also be distinguished from tic disorders. Stereotypic movement disorders, including movements such as rocking, hand-gazing, and other self-stimulatory behaviors, seem to be voluntary and often produce a sense of comfort, in contrast to tic disorders. Although tics in children and adolescents may or may not feel controllable, they rarely produce a sense of well-being. Compulsions are sometimes difficult to distinguish from complex tics and may be on the same continuum biologically. Tic disorders may also occur comorbidly with mood disturbances. In a recent survey, the greater the severity of tics, the higher the probability of both aggressive and depressive symptoms in children. When a child experiences an exacerbation of tic symptoms, behavior and mood also seem to deteriorate. Table 31.8c-2 Differential Diagnosis of Tic Disorders COURSE AND PROGNOSIS Tourette’s disorder is a childhood-onset neuropsychiatric disorder characterized by both motor and vocal tics, which usually emerge in early childhood, with a natural history leading to reduction or complete resolution of tics symptoms in most cases by adolescence or early adulthood. During childhood, individual tic symptoms may decrease, persist, or increase, and old symptoms may be replaced by new ones. Severely afflicted persons may have serious emotional problems, including major depressive disorder. Impairment may also be associated with the motor and vocal tic symptoms of Tourette’s disorder; however, in many cases, interference in function is exacerbated by comorbid ADHD and OCD, both of which frequently coexist with the disorder. When the above three disorders are comorbid, severe social, academic, and occupational problems may ensue. Although most children with Tourette’s disorder will experience a decline in the frequency and severity of tic symptoms during adolescence, at present, no clinical measures exist to predict which children may have persistent symptoms into adulthood. Children with mild forms of Tourette’s disorder often have satisfactory peer relationships, function well in school, and develop adequate self-esteem, and may not require treatment. TREATMENT Once a diagnosis of Tourette’s disorder is made, psychoeducation is a useful intervention in order for families to gain an understanding of the variability of tics, the natural history of the disorder, and ways to support reduction of stress. It is particularly important for families to be well-informed advocates for their children, since tics may be misinterpreted by an uneducated observer as a child’s purposeful misbehavior, rather than a response to an irresistible urge. The need for treatment is based on subjective distress of a child with respect to tics as well as observable disruptions in functioning. In mild cases, children with tic disorders who are functioning well socially and academically may not seek, nor require treatment. In more severe cases, children with tic disorders may be ostracized by peers and have academic work compromised by the disruptive nature of tics, and a variety of interventions including psychosocial, pharmacological, and school based may be considered. A scale to measure tic severity, the Premonitory Urge for Tics Scale (PUTS), was examined psychometrically, and found to be internally consistent and correlated with overall tic severity in youth over 10 years of age. The European clinical guidelines for Tourette’s syndrome and other tic disorders summarized and reviewed the evidence-based treatments for Tourette’s disorder and developed a consensus for psychosocial and pharmacological treatments. This guideline recommends that both behavioral and pharmacological interventions be considered in more severe cases, with behavioral interventions typically the first line of treatment. Indications for treatment include, but are not limited to, the following clinical presentations. Tics require treatment when they cause social and emotional problems, depression, or isolation. Children who are prone to severe persistent complex motor tics or loud vocal tics may be the objects of bullying and social rejection. In these cases, depressive symptoms commonly result. Tic reduction and psychoeducation to the school may be indicated in order to preserve healthy social relationships, and to diminish depressive and anxiety symptoms. Tics may also lead to impairment in academic achievement, when school functioning is disrupted. School difficulties in children with Tourette’s disorder are not uncommon, and reduction in tics may support increased academic success. Tics may also lead to physical discomfort, based on the repetitive musculoskeletal exertion, especially in relation to head and neck tics. In some children with Tourette’s disorder, tics can worsen headaches and migraines. Behavioral and pharmacological interventions can both target tic reductions, which can lead to improved quality of life. Evidence-based Behavioral and Psychosocial Treatment The Canadian guidelines for the evidence-based treatment of tic disorders: behavioral therapy, deep brain stimulation and transcranial magnetic stimulation, and a large multi-site randomized controlled trial of “Comprehensive Behavioral Intervention for Tics,” (CBIT) both found converging evidence supporting habit-reversal training and exposure and response prevention as efficacious treatments for tic reduction. In a randomized controlled trial of CBIT, 61 children received habit reversal training as their main component of treatment, and they also received relaxation treatment and a functional intervention to identify situations that worsened or sustained tics and strategies to decrease exposure to these situations. The control group of 65 children received supportive psychotherapy and psychoeducation. After 10 weeks of treatment, the Yale Global Tic Severity Scale Total Tic score was significantly reduced in the behavioral intervention group compared with the control group. Habit Reversal. The primary components of habit reversal are awareness training, in which the child uses self-monitoring to enhance awareness of tic behaviors and the premonitory urges or sensations indicating that a tic is about to occur. In competingresponse training, the patient is taught to voluntarily perform a behavior that is physically incompatible with the tic, contingent on the onset of the premonitory urge or the tic itself, blocking expression of the tic. The competing-response strategy is based on the self-reported observations of patients that tics are performed in response to irresistible premonitory urges in order to diminish the urge. Because performing the tic satisfies or reduces the premonitory urges, the tics are reinforced, and over time, become repeated entrenched behaviors. Competing-response training is different from voluntary tic suppression in that the patient initiates a voluntary behavior to manage the premonitory urge and thus disrupts the reinforcement of the tic, rather than simply trying to suppress the tic. Successful competing-response training results in significant reduction in premonitory urge intensity or complete elimination of the urge altogether so that tics are no longer provoked. For motor tics, a behavior that is less noticeable may be chosen, whereas for vocal tics, slow rhythmic breathing is the most common voluntary competing response. The competing responses are designed to be performed without disrupting usual activities. Exposure and Response Prevention. The rationale for this treatment is based on the notion that tics occur as a conditioned response to unpleasant premonitory urges, and since the tics reduce the urge, they become associated with the premonitory urge. Each time the urge is reduced by the tic, their association is further strengthened. Rather than using competing responses, as in habit-reversal training, exposure and response prevention asks the patient to suppress tics for increasingly prolonged periods in order to break the association between the urges and the tics. Theoretically, if a patient learns to resist performing the tic in response to the urge for long enough periods, the urge may become more tolerable, or attenuate, and the need to perform the tic may diminish. Many other behavioral interventions such as relaxation training, self-monitoring, bio (neuro) feedback, and cognitive-behavioral treatment (CBT), have not been shown to be efficacious in the reduction of tics on their own; however, some of these strategies may be included in comprehensive treatment programs for children with tic disorders who are receiving habit-reversal training. Habit reversal has been the most extensively researched behavioral treatment for tic disorders; it has been shown to be highly effective, and is currently the first-line behavioral treatment for tic disorders. Evidence-based Pharmacotherapy Several reviews of pharmacological treatments for tics suggest that the following classes of pharmacologic agents have an evidence base for treating tics: typical and atypical antipsychotics; noradrenergic agents; and alternative treatments such as tetrabenazine, topiramate, and tetrahydrocannabinol. Atypical and Typical Antipsychotic Agents. Risperidone, with its high affinity for dopamine D2 and serotonin 5-HT2 receptors, is the most well-studied atypical antipsychotic in the treatment of tics. There is considerable evidence for its efficacy. Multiple randomized, controlled studies in children and adolescents have shown favorable results compared to placebo as well as in head-to-head studies with the typical antipsychotic agents haloperidol and pimozide. Risperidone was associated with fewer adverse events compared to typical antipsychotics; however, it was frequently associated with weight gain, metabolic side effects, and hyperprolactinemia. In a randomized, double-blind, parallel group study of Tourette’s disorder comparing risperidone to pimozide, risperidone showed superiority in reducing comorbid obsessivecompulsive symptoms as well as reducing tics. In other randomized clinical trials, efficacy of tic reduction was achieved in studies of children, adolescents, and adults with mean daily doses of 2.5 mg daily with a range of 1 to 6 mg daily. Haloperidol (Haldol) and pimozide (Orap) are the two most well-investigated and Food and Drug Administration (FDA)–approved antipsychotic agents in the treatment of Tourette’s disorder, although atypical antipsychotics such as risperidone are often chosen as first-line agents due to their safer side-effect profiles. Both haloperidol and pimozide have been shown to be efficacious in multiple randomized clinical trials in the treatment of Tourette’s disorder. Both haloperidol and pimozide present significant risks for extrapyramidal side effects; in a long-term naturalistic follow-up study, haloperidol was found to produce more significant acute dyskinesia and dystonia compared to pimozide. A third typical antipsychotic, fluphenazine, has been used in the United States for many years in the treatment of tic disorders, in the absence of robust data supporting its efficacy. A small controlled study of fluphenazine, trifluphenazine, and haloperidol found similar reductions in tics; however, haloperidol was associated with more extrapyramidal side effects and more sedation. The frequency of sedation, dystonia, and akathisia of typical antipsychotics, probably due to their predominant dopaminergic blockade in the nigrostriatal pathways, limits their use and increases the appeal of the atypical antipsychotics. Risperidone and pimozide were found to be of equal efficacy in one study of children, adolescents, and adults with Tourette’s disorder. Aripiprazole has become a pharmacological agent of interest in the treatment of tic disorders due to its mode of action; in addition to its D2 receptor antagonistic actions, aripiprazole is also a partial D2 and 5-HT1A receptor agonist and a 5-HT2A antagonist. A multisite double-blind controlled study of aripiprazole in children with Tourette’s disorder in China found a reduction in tic behaviors in about 60 percent of the aripiprazole group compared to about 64 percent reduction in a group treated with tiapride, a benzamide with selective D2 receptor antagonism. There was no significant difference between the two groups. Although sedation and sleep disturbance are common side effects with aripiprazole, weight gain is less pronounced than with risperidone. Olanzapine and ziprasidone were shown to be efficacious in the treatment of tic disorders in at least one randomized controlled trial. Sedation and weight gain were prominent side effects with olanzapine, and potential QT prolongation was an issue with ziprasidone. Quetiapine has been suggested as a potentially useful agent in the treatment of tics, with its greater affinity for 5-HT2 receptors than for D2 receptors, however, randomized clinical trials are needed. Clozapine, contrary to many other atypical antipsychotics, has not been found to be useful in the treatment of tics. Noradrenergic Agents. Noradrenergic agents including clonidine and guanfacine, as well as atomoxetine, are frequently used in children as primary treatments or adjunctive treatments for comorbid ADHD and tics. Several studies have provided some evidence for the efficacy of clonidine, an alpha 2-adrenergic agent, in the treatment of tics in children, adolescents, and adults with tic disorders. The largest randomized trial with oral clonidine compared to placebo found a modest reduction in tics with clonidine. A multisite randomized double-blind placebo controlled trial using the clonidine patch in the treatment of tic disorders in children found a significant improvement in tic symptoms (about 69 percent) compared to about 47 percent of the children in the control group. Clonidine has generally been used in dosages ranging from 0.05 mg orally three times daily to 0.1 mg four times daily; and guanfacine is usually used in dosages ranging from 1 to 4 mg per day. When used in these dosage ranges, adverse effects of the α-adrenergic agents may include drowsiness, headache, irritability, and occasional hypotension. Guanfacine has been used frequently to treat children with ADHD successfully, although its efficacy regarding reducing tics is controversial. In one randomized clinical trial treating 34 children with ADHD and tics, guanfacine was found to be superior to placebo in the reduction of tics. In another double-blind placebo-controlled trial of 24 children with Tourette’s disorder, guanfacine was not superior to placebo. Atomoxetine, a selective noradrenaline reuptake inhibitor, was found to reduce both tics and ADHD symptoms in a multicenter industry trial of 148 children. Atomoxetine also reduced both tics and ADHD in a subgroup of patients in this study who were diagnosed with Tourette’s disorder. Additional studies are needed to confirm safety and efficacy of atomoxetine in the treatment of children with Tourette’s disorder. In view of the frequent comorbidity of tic behaviors and obsessive-compulsive symptoms or disorders, the SSRIs have been used alone or in combination with antipsychotics in the treatment of Tourette’s disorder. Data, thus far, have supported the efficacy of SSRIs in the treatment of OCD, however there have not been controlled trials yet to determine the effect of SSRIs on tic reduction. Although clinicians must weigh the risks and benefits of using stimulants in cases of severe hyperactivity and comorbid tics, data suggest that methylphenidate does not increase the rate or intensity of motor or vocal tics in most children with hyperactivity and tic disorders. Alternative Agents: Tetrabenazine, Topiramate, and Tetrahydrocannabinol TETRABENAZINE. A vesicular monoamine transporter type 2 inhibitor, tetrabenazine depletes presynaptic dopamine and serotonin, and blocks postsynaptic dopamine receptors. There are no randomized clinical trials of this agent in the treatment of Tourette’s disorder in children; however, clinical experience suggests that this agent may have benefit in tic reduction. In a follow-up of 2 years of treatment in 77 children and adolescents, one study reports tic reduction improvement in 80 percent of subjects. Side effects of this agent include sedation, parkinsonism, depression, insomnia, anxiety, and akathisia. TOPIRAMATE. A γ-aminobutyric acid (GABA)ergic drug, used primarily as an anticonvulsant, topiramate was found to be efficacious compared to placebo in reducing tics in a small randomized clinical trial of children and adults with Tourette’s disorder. Side effects were minimal. Although this does not confirm its efficacy, GABA-modulating agents require further study in the treatment of tic disorders. TETRAHYDROCANNABINOL. A suggestion that tetrahydrocannabinol (THC) may be safe and efficacious in the treatment of tics, without neuropsychological impairment, is based on a randomized double-blind placebo-controlled trial with 24 patients treated with THC for 6 weeks at doses of up to 10 mg with significant improvement in tic severity. In this trial, reported adverse effects included dizziness, fatigue, and dry mouth. Potential additional side-effects include anxiety, depressive symptoms, tremor, and insomnia. This small trial does not confirm efficacy for this agent in the treatment of tics, rather it raises questions about the potential improvements in treatment-resistant tic disorders using this agent. In summary, the greatest evidence for the safe and efficacious pharmacological treatment of Tourette’s disorder seems to be associated with the atypical antipsychotics, in particular, risperidone. Pharmacological treatment may be combined with and enhanced by a variety of behavioral interventions such as habit reversal and school interventions that may diminish stressful situations in the school environment. REFERENCES Debes NM, Hansen A, Skov L, Larsson H. A functional magnetic resonance imaging study of a large clinical cohort of children with Tourette syndrome. J Child Neurol. 2011;26:560–569. Eddy CM, Rickards HE, Cavanna AE. Treatment strategies for tics in Tourette syndrome. Ther Adv Neurol Disord. 2011;4:25–45. Hartmann A, Worbe Y. Pharmacological treatment of Gilles de la Tourette syndrome. Neurosci Biobehav Rev. 2013;37:1157– 1161. Janovic J, Jimenez-Shahed J, Brown L. A randomized, double-blind, placebo-controlled study of topiramate in the treatment of Tourette syndrome. J Neurol Neurosurg Psychiatry. 2010;81:70–73. Jummani R, Coffey BJ. Tic disorders. In: Sadock BJ, Sadock VA, Ruiz P, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Vol. 2. Philadelphia: Lippincott Williams & Wilkins; 2009:3609. Knight T, Stevvers T, Day L, Lowerison M, Jette N, Pringsheim T. Prevalence of tic disorders: A systematic review and meta-analysis. Pediatr Neurol. 2012;47:77–90. Kraft JT, Dalsgaard S, Obel C, Thomsen PH, Henriksen TB, Scahill L. Prevalence and clinical correlates of tic disorders in a community sample of school-age children. Eur Child Adolesc Psychiatry. 2012;21:5–13. Liu ZS, Chen YH, Zhong YQ, Zou LP, Wang H, Sun D. A multicentre controlled study on aripiprazole treatment for children with Tourette syndrome in China. Zhonghua Er Ke Za Zhi. 2011;49:572–576. Paschou P. The genetic basis of Gilles de la Tourette Syndrome. Neurosci Biobehav Rev. 201337:1026–1039. 17 - 31.8d Persistent (Chronic) Motor or Vocal Tic 31.8d Persistent (Chronic) Motor or Vocal Tic Disorder Piacentini J, Woods DW, Scahill L, Wilhelm S, Peterson AL, Chang S. Behavior therapy for children with Tourette disorder. A randomized controlled trial. JAMA. 2010;303:1929–1937. Porta M, Sassi M, Cavallazzi M, Fornari M, Brambilla A. Servello D. Tourette’s syndrome and the role of tetrabenzine: review and personal experience. Clin Drug Investig. 2008;28:443–459. Roessner V, Plessen KJ, Rothenberger A, Ludolph AG, Rizzo R, Skov L. European clinical guidelines for Tourette syndrome and other tic disorders. Part II: Pharmacologic treatment. Eur Child Adolesc Psychiatry. 2011;20:173–196. Rothenbertger A, Roessner V. Functional neuroimaging investigations of motor networks in Tourette syndrome. Behav Neurol. 2013;27:47–55. Scharf JM, Miller LL, Mathews CA, Ben-Shlomo Y. Prevalence of Tourette syndrome and chronic tics in the populationbased Avon longitudinal study of parents and children cohort. J Am Acad Child Adolesc Psychiatry. 2012;51:192–201. Steeves T, McKinlay BD, Gorman D, Billinghurst L, Day L, Carrol A. Canadian guidelines for the evidence-based treatment of tic disorders: Behavioural therapy, deep brain stimulation and transcranial magnetic stimulation. Can J Psychiatry. 2012;57:144–151. Thomas R, Cavanna AE. The pharmacology of Tourette syndrome. J Neural Transm. 2013;120(4):689–94. Verdellen C, Griendt JVD, Hartmann A, Murphy T, the ESSTS Guidelines Group. European clinical guidelines for Tourette syndrome and other tic disorders. Part III: behavioural and psychosocial interventions. Eur Child Adolesc Psychiatry. 2011;20:97–207. Weisman H, Qureshi IA, Leckman JF, Scahill L, Bloch MH. Systematic review: Pharmacological treatment of tic disorders —Efficacy of antipsychotic and alpha-2 adrenergic agonist agents. Neurosci Biobehav Rev. 2013;37(6):1162–71. Woods DW, Piacentini JC, Scahill L, Peterson AL, Wilhelm S, Chang S. Behavior therapy for tics in children: acute and long-term effects on psychiatric and psychosocial functioning. J Child Neurol. 2011;7:858–865. 31.8d Persistent (Chronic) Motor or Vocal Tic Disorder Chronic motor or vocal tic disorder is defined as the presence of either motor tics or vocal tics, but not both. Tics may wax and wane but must have persisted for more than 1 year since the first tic onset to meet the diagnosis for persistent (chronic) motor or vocal tic disorder. According to the Fifth Edition of the American Psychiatric Association’s Diagnostic and Statistical Manual of Mental Disorders (DSM-5) criteria, this disorder must have its onset before the age of 18 years. Chronic motor or vocal tic disorder cannot be diagnosed if the criteria for Tourette’s disorder have ever been met. EPIDEMIOLOGY The rate of chronic motor or vocal tic disorder has been estimated to be 100 to 1,000 times greater than that of Tourette’s disorder in school-age children. School-age boys are at highest risk. Although the disorder was once believed to be rare, current estimates of the prevalence of chronic motor or vocal tic disorder range from 1 to 2 percent. ETIOLOGY Chronic motor or vocal tic disorder as well as Tourette’s disorder tend to aggregate in the same families. Twin studies have found a high concordance for either Tourette’s disorder or chronic motor tics in monozygotic twins. This finding supports the importance of hereditary factors in the transmission of tic disorders. DIAGNOSIS AND CLINICAL FEATURES The onset of chronic motor or vocal tic disorder typically occurs in early childhood. Chronic vocal tics are considerably rarer than chronic motor tics. Chronic vocal tics, in the absence of motor tics, are typically less conspicuous than the vocal tics in Tourette’s disorder. The vocal tics are usually not loud or intense and are not primarily produced by the vocal cords; they consist of grunts or other noises caused by thoracic, abdominal, or diaphragmatic contractions. DIFFERENTIAL DIAGNOSIS Chronic motor tics must be differentiated from a variety of other motor movements, including choreiform movements, myoclonus, restless legs syndrome, akathisia, and dystonias. Involuntary vocal utterances can occur in certain neurological disorders, such as Huntington’s disease and Parkinson’s disease. COURSE AND PROGNOSIS Children whose tics emerge between the ages of 6 and 8 years seem to have the best outcomes. Symptoms often last for 4 to 6 years and remit in early adolescence. Children whose tics involve the limbs or trunk may have less prompt remission than those with only facial tics. TREATMENT The treatment of chronic motor or vocal tic disorder depends on several factors including the severity and frequency of the tics; the patient’s subjective distress; the effects of the tics on school or work, job performance, and socialization; and the presence of any other concomitant mental disorder. Psychotherapy may be indicated to minimize the secondary social difficulties caused by severe tics. Behavioral techniques, particularly habit reversal treatments, are effective in treating chronic motor or vocal tic disorder. When severe, tics may be reduced through the use of atypical antipsychotics such as risperidone. If not effective, typical antipsychotics such as pimozide or haloperidol may be helpful. Behavioral interventions are the first line of treatment. REFERENCES Du YS, Li HF, Vance A, Zhong YQ, Jiao FY, Wang HM. Randomized double-blind multicentre placebo-controlled clinical trial of the clonidine adhesive patch for the treatment of tic disorders. Aust NZJ Psychiatry. 2008;42:807–813. Jummani R, Coffey BJ. Tic disorders. In: Sadock BJ, Sadock VA, Ruiz P, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Vol. 2. Philadelphia: Lippincott Williams & Wilkins; 2009:3609. Knight T, Stevvers T, Day L, Lowerison M, Jette N, Pringsheim T. Prevalence of tic disorders: A systematic review and 18 - 31.9 Feeding and Eating Disorders of Infancy 31.9 Feeding and Eating Disorders of Infancy or Early Childhood 19 - 31.9a Pica 31.9a Pica meta-analysis. Pediatr Neurol. 2012;47:77–90. Kraft JT, Dalsgaard S, Obel C, Thomsen PH, Henriksen TB, Scahill L. Prevalence and clinical correlates of tic disorders in a community sample of school-age children. Eur Child Adolesc Psychiatry 2012;21:5–13. Roessner V, Plessen KJ, Rothenberger A, Ludolph AG, Rizzo R, Skov L. European clinical guidelines for Tourette syndrome and other tic disorders. Part II: Pharmacologic treatment. Eur Child Adolesc Psychiatry. 2011;20:173–196. Scharf JM, Miller LL, Mathews CA, Ben-Shlomo Y. Prevalence of Tourette syndrome and chronic tics in the populationbased Avon longitudinal study of parents and children cohort. J Am Acad Child Adolesc Psychiatry 2012;51:192–201. Spencer TJ, Sallee FR, Gilbert DL, Dunn DW, McCracken JT, Coffey BJ. Atomoxetine treatment of ADHD in children with comorbid Tourette syndrome. J Atten Disord. 2008;11:470–481. Steeves T, McKinlay BD, Gorman D, Billinghurst L, Day L, Carrol A, et al. Canadian guidelines for the evidence-based treatment of tic disorders: Behavioural therapy, deep brain stimulation and transcranial magnetic stimulation. Can J Psychiatry. 2012;57:144–151. Storch EA, Murphy TK, Geffken GR, Sajid M, Allen P, Roberti JW, Goodman WK. Reliability and validity of the Yale Global Tic Severity Scale. Psychol Assess. 2005;17:486. Verdellen C, Griendt JVD, Hartmann A, Murphya T, the ESSTS Guidelines Group. European clinical guidelines for Tourette syndrome and other tic disorders. Part III: Behavioural and psychosocial interventions. Eur Child Adolesc Psychiatry. 2011;20:97–207. Woods DW, Piacentini JC, Scahill L, Peterson AL, Wilhelm S, Chang S. Behavior therapy for tics in children: acute and long-term effects on psychiatric and psychosocial functioning. J Child Neurol. 2011;7:858–865. 31.9 Feeding and Eating Disorders of Infancy or Early Childhood Feeding and eating disorders of infancy and childhood are characterized by persistent disturbances in eating or eating-related disorders that can lead to significant impairments in physical health and psychosocial functioning. The American Psychiatric Association’s Fifth Edition of the Diagnostic and Statistical Manual of Mental Disorders (DSM-5) category Feeding and Eating Disorders includes three disorders that are often, but not always, associated with infancy and early childhood: pica, rumination disorder, and avoidant/restrictive food intake disorder (formerly known as feeding disorder of infancy or early childhood). These three disorders are discussed in this section. Anorexia nervosa, bulimia nervosa, and binge-eating disorder are more often associated with young adulthood and discussed separately in Chapter 15. 31.9a Pica Pica is defined as persistent eating of nonnutritive substances. Typically, no specific biological abnormalities account for pica, and in many cases, pica is identified only when medical problems such as intestinal obstruction, intestinal infections, or poisonings arise, such as lead poisoning due to ingestion of lead containing paint chips. Pica is more frequent in the context of autism spectrum disorder or intellectual disability; however, pica is diagnosed only when it is of sufficient severity and persistence to warrant clinical attention. Pica can emerge in young children, adolescents, or adults; however, a minimum of 2 years of age is suggested by DSM-5 in the diagnosis of pica, in order to exclude developmentally appropriate mouthing of objects by infants that may accidentally result in ingestion. Pica occurs in both males and females, and in rare cases, it may be associated with a cultural belief in the spiritual or medicinal benefit of ingesting nonfood substances. In this context, a diagnosis of pica is not made. Among adults, certain forms of pica, including geophagia (clay eating) and amylophagia (starch eating), have been reported in pregnant women. EPIDEMIOLOGY The prevalence of pica is unclear. A survey of a large clinic population reported that 75 percent of 12-month-old infants and 15 percent of 2- to 3-year-old toddlers placed nonnutritive substances in their mouth; however, this behavior is developmentally appropriate and typically does not result in ingestion. Pica is more common among children and adolescents with autism spectrum disorder and intellectual disability. It has been reported that up to 15 percent of persons with severe intellectual disability have engaged in pica. Pica appears to affect both sexes equally. ETIOLOGY Pica is most often a transient disorder that typically lasts for several months and then remits. In younger children, it is more frequently seen among children with developmental speech and social developmental delays. Among adolescents with pica, a substantial number of them exhibited depressive symptoms and use of substances. Nutritional deficiencies in minerals such as zinc or iron have been anecdotally reported in some instances; however, these reports are rare. For example, cravings for dirt and ice have been reported to be associated with iron and zinc deficiencies, which are corrected by their administration. Severe child maltreatment in the form of parental neglect and deprivation has been reported in some cases of pica. Lack of supervision, as well as adequate feeding of infants and toddlers may increase the risk of pica. DIAGNOSIS AND CLINICAL FEATURES Eating nonedible substances repeatedly after 18 months of age is not typical; however, DSM-5 suggests a minimum age of 2 years when making a diagnosis of pica. Pica behaviors, however, may begin in infants 12 months to 24 months of age. Specific substances ingested vary with their accessibility, and they increase with a child’s mastery of locomotion and the resultant increased independence and decreased parental supervision. Typically, in infants, paint, plaster, string, hair, and cloth are objects that may be ingested, whereas older toddlers and young children with pica may ingest dirt, animal feces, small stones, and paper. The clinical implications can be benign or lifethreatening, depending on the objects ingested. Among the most serious complications are lead poisoning (usually from lead-based paint), intestinal parasites after ingestion of soil or feces, anemia and zinc deficiency after ingestion of clay, severe iron deficiency after ingestion of large quantities of starch, and intestinal obstruction from the ingestion of hair balls, stones, or gravel. Except in autism spectrum disorder and intellectual disability, pica often remits by adolescence. Pica associated with pregnancy is usually limited to the pregnancy itself. Chantal was 2½ years of age when her mother urgently brought her to her pediatrician due to severe abdominal pain and lack of appetite. Chantal’s mother complained that she still put everything in her mouth but refused to eat regular food. The pediatrician observed that Chantal to be pale, thin, and withdrawn. She sucked her thumb and quietly looked down while her mother reported that Chantal often chewed on newspapers and put plaster in her mouth. The medical examination revealed that Chantal was anemic and suffered from lead poisoning. She was admitted to the hospital for treatment, and a child psychiatric consultation was obtained. Further exploration of the history and the observation of mother and child during feeding and play revealed that Chantal’s mother was overwhelmed, caring for five young children and had little affection for Chantal. Chantal’s mother was a single mother, living with her five children and four other family members in a threebedroom apartment in an old housing project. Her 7-year-old daughter had behavior problems, and her 6-year-old and 4-year-old sons were impulsive and hyperactive and required constant supervision. Chantal’s 18-month-old sister was an engaging and active little girl, whereas Chantal was withdrawn, and would sit quietly, rocking herself, sucking her thumb, or chewing on newspaper. The treatment plan included the involvement of social services and protective services to remove any lead paint from the walls in their current apartment, seek better living arrangements for the family, and provide a safe environment for the children. Chantal’s mother received guidance in enrolling Chantal in a preschool program, and her older sister and two brothers in an after-school program that provided structure and stimulation, and some respite time for her mother. Chantal, her mother, and her younger sister started family therapy to help their mother’s understanding of her children’s needs and to increase her positive interactions with Chantal. Once Chantal’s mother felt more supported and less overwhelmed, she was able to become more empathic and warm toward Chantal. When Chantal began chewing on paper, her mother was coached to engage her in a play activity rather than screaming at her and grabbing her mouth. Chantal and her mother continued in therapy for a year, during which their relationship gradually became more interactive and warm, while Chantal’s chewing behaviors decreased, and even her thumb sucking abated. PATHOLOGY AND LABORATORY EXAMINATION No single laboratory test confirms or rules out a diagnosis of pica, but several laboratory tests are useful because pica has sometimes been associated with abnormal levels of lead. Levels of iron and zinc in serum should be determined and corrected if low. In rare cases when this is the etiology, pica may disappear when oral iron and zinc are administered. Hemoglobin level should be determined to rule out anemia. DIFFERENTIAL DIAGNOSIS The differential diagnosis of pica includes avoidance of food, anorexia, or rarely iron and zinc deficiencies. Pica may occur in conjunction with failure to thrive, and be comorbid with schizophrenia, autism spectrum disorder and Kleine-Levin syndrome. In psychosocial dwarfism, a dramatic but reversible endocrinological and behavioral form of failure to thrive, children often show bizarre behaviors, including ingesting toilet water, garbage, and other nonnutritive substances. Lead intoxication may be associated with pica. In children who exhibit pica that warrants clinical intervention, along with a known medical disorder, both disorders should be coded according to DSM-5. In certain regions of the world and among certain cultures, such as the Australian aborigines, rates of pica in pregnant women are reportedly high. According to DSM-5, however, if such practices are culturally accepted, the diagnostic criteria for pica are not met. COURSE AND PROGNOSIS The prognosis for pica is usually good, and typically in children with normal intellectual function, pica generally remits spontaneously within several months. In childhood, pica usually resolves with increasing age; in pregnant women, pica is usually limited to the term of the pregnancy. In some adults with pica, particularly those who also have autism spectrum disorder and intellectual disability, pica can continue for years. Followup data on these populations are too limited to permit conclusions. TREATMENT The first step in determining appropriate treatment of pica is to investigate the specific situation whenever possible. When pica occurs in the context of child neglect or maltreatment, clearly those circumstances must be immediately corrected. Exposure to toxic substances, such as lead, must also be eliminated. No definitive treatment exists for pica per se; most treatment is aimed at education and behavior modification. Treatments emphasize psychosocial, environmental, behavioral, and family guidance approaches. An effort should be made to ameliorate any significant psychosocial stressors. When lead is present in the surroundings, it must be eliminated or rendered inaccessible or the child must be moved to new surroundings. When pica persists in the absence of any toxic manifestations, behavioral techniques have been utilized. Positive reinforcement, modeling, behavioral shaping, and overcorrection treatment have been used. Increasing parental attention, stimulation, 20 - 31.9b Rumination Disorder 31.9b Rumination Disorder and emotional nurturance may yield positive results. A study found that pica occurred most frequently in impoverished environments, and in some patients, correcting an iron or zinc deficiency has eliminated pica. Medical complications (e.g., lead poisoning) that develop secondarily to the pica must also be treated. 31.9b Rumination Disorder Rumination is an effortless and painless regurgitation of partially digested food into the mouth soon after a meal, which is either swallowed or spit out. Rumination can be observed in developmentally normal infants who put their thumb or hand in the mouth, suck their tongue rhythmically, and arch their back to initiate regurgitation. This behavior pattern may be observed in infants who receive inadequate emotional interaction and have learned to soothe and may stimulate themselves through rumination. However, rumination syndromes can be found to occur in children and adolescents, and rumination is considered to a functional gastrointestinal disorder. The pathophysiology of rumination is not well understood; however, it often involves a rise in intragastric pressure, generated by either voluntary or unintentional contraction of the abdominal wall muscles causing movement of gastric contents back up into the esophagus. The onset of the disorder can occur in infancy, childhood, or adolescence. In infants, it typically occurs between 3 months and 12 months of age, and once the regurgitation occurs, the food may be swallowed or spit out. Infants who ruminate are characteristically observed to strain with their backs arched and head back to bring the food back into their mouths and appear to find the experience pleasurable. Infants who are “experienced” ruminators are able to bring up the food through tongue movements and may not spit out the food at all, but hold it in their mouths and re-swallow it. The disorder is less common in older children, adolescents, and adults. It varies in severity and is sometimes associated with medical conditions, such as hiatal hernia, that result in esophageal reflux. In its most severe form, the disorder can cause malnutrition and be fatal. The diagnosis of rumination disorder can be made even if an infant has attained a normal weight for his or her age. Failure to thrive, therefore, is not a necessary criterion of this disorder, but it is sometimes a sequela. According to DSM-5, the disorder must be present for at least 1 month after a period of normal functioning, and not better accounted for by gastrointestinal illness, or psychiatric or medical conditions. Rumination has been recognized for hundreds of years. An awareness of the disorder is important so that it is correctly diagnosed and that unnecessary surgical procedures and inappropriate treatment are avoided. Rumination is derived from the Latin word ruminare, which means, “to chew the cud.” The Greek equivalent is merycism, the act of regurgitating food from the stomach into the mouth, re-chewing the food, and reswallowing it. EPIDEMIOLOGY Rumination is a rare disorder. It seems to be more common among male infants, and emerges between 3 months and 1 year of age. It persists more frequently among children, adolescents, and adults with intellectual disability. Adults with rumination usually maintain a normal weight. ETIOLOGY Rumination is associated with high intragastric pressure and the ability to contract the abdominal wall to cause retrograde movement of the gastric contents into the esophagus. Several studies have elucidated other gastrointestinal symptoms such as gastroesophageal reflux that may accompany rumination. In a study of 2,163 children in Sri Lanka, between the ages of 10 years and 16 years, it was found that rumination behaviors were present in 5.1 percent of boys and 5.0 percent of girls. In 94.5 percent of youth who ruminated, the regurgitation occurred in the first hour after the meal, and 73.6 percent reported re-swallowing of the regurgitated food, whereas the rest spit it out. Only 8.2 percent of this sample reported daily episodes of regurgitation, whereas 62.7 percent experienced weekly symptoms. Associated gastrointestinal symptoms reported in this sample included abdominal pain, bloating, and weight loss. Approximately 20 percent of youth with rumination in this sample also experienced other gastrointestinal symptoms. Another survey of 147 patients from 5 years to 20 years of age found that in their sample, the mean age of onset of rumination was 15 years, and these patients were symptomatic after each meal; 16 percent of this sample met criteria for a psychiatric disorder, 3.4 percent had anorexia or bulimia nervosa, and 11 percent had been treated with a surgical procedure for evaluation of management of their symptoms. Additional gastrointestinal symptoms in this sample included abdominal pain in 38 percent, constipation in 21 percent, nausea in 17 percent, and diarrhea in 8 percent. In some cases, vomiting secondary to gastroesophageal reflux or an acute illness precedes a pattern of rumination that lasts for several months. In many cases, children classified as ruminators are shown to have gastroesophageal reflux or hiatal hernia. It appears, for some infants, that the rumination behavior is self-soothing or produces a sense of relief, leading to a continuation of behaviors to bring it about. In youth with autism spectrum disorder or intellectual disability, rumination may serve as a selfstimulatory behavior. Overstimulation and tension have also been suggested as contributing factor in rumination. Behaviorists attribute persistent rumination to the positive reinforcement of pleasurable self-stimulation and to the attention a baby receives from others as a consequence of the disorder. DIAGNOSIS AND CLINICAL FEATURES The DSM-5 notes that the essential feature of the disorder is repeated regurgitation and re-chewing of food for a period of at least 1 month after a period of normal functioning. Partially digested food is brought up into the mouth without nausea, retching, or disgust; on the contrary it may appear to be pleasurable. This activity may be distinguished from vomiting by painless and purposeful movements observable in some infants who induce it. The food is then ejected from the mouth or swallowed. A characteristic position of straining and arching of the back, with the head held back, is observed. The infant makes sucking movements with the tongue and gives the impression of gaining considerable satisfaction from the activity. Usually, the infant is irritable and hungry between episodes of rumination. Initially, rumination may be difficult to distinguish from the regurgitation that frequently occurs in normal infants. In infants with persistent and frequent rumination behaviors, however, the differences are obvious. Although spontaneous remissions are common, secondary complications can develop, such as progressive malnutrition, dehydration, and lowered resistance to disease. Failure to thrive, with absence of growth and developmental delays in all areas, can occur in the most severe cases. Additional complications may occur if the mother of a given infant with rumination becomes discouraged by the persistent symptoms, viewing it as her feeding failure, as this may lead to more tension and more rumination after feedings. Luca was 9-months-old when he was referred by his pediatrician to a gastroenterologist, and by his gastroenterologist for a psychiatric evaluation due to persistent and frequent rumination. Luca was born full-term and had developed typically until 6 weeks of age, when he began to regurgitate large amounts of milk just after feedings. He was evaluated and diagnosed with gastroesophageal reflux, for which it was recommended to thicken his feedings. Luca responded well to the treatment; his regurgitation was markedly diminished, and he gained weight adequately. Luca continued to do well, and his mother decided to go back to work when Lucas was 8-months-old. Luca’s mother transitioned his care to a young nanny who cared for Luca while she worked. Luca and the nanny seemed to have a warm relationship; however, he started again to regurgitate his meals soon after his mother left the house. The regurgitation seemed to increase in frequency and intensity within 2 weeks of the mother’s return to work. At this point, Luca regurgitated after almost every meal, and he was losing weight. Luca was evaluated by a gastroenterologist, and during the barium swallow, his doctor noted that Luca put his hand in his mouth, which seemed to induce the regurgitation. Luca was administered some medication for gastroesophageal reflux; however, he continued to induce regurgitation after meals with increasing frequency, prompting the psychiatric consultation. Observation of mother and infant during feeding at home revealed that as soon as Luca finished feeding, he purposefully placed his hand in his mouth and induced the regurgitation. When his mother restricted his hand, Luca moved his tongue back and forth in a rhythmic manner until he regurgitated again. Luca engaged in this rhythmic tongue movement repeatedly, even when he could not bring up any more milk, and appeared to be enjoying this behavior. Due to Luca’s poor nutritional state and moderate dehydration, he was admitted to the hospital, and a nasojejunal tube was inserted for feedings. When Luca was awake during feedings, a special duty nurse or his parents played with him and distracted him during attempts to put his hand in his mouth or thrust his tongue rhythmically. Luca became increasingly engaged in this playful activity, and his ruminatory activity decreased accordingly. After 1 week in the hospital, small feedings were started; however, Luca again successfully was able to bring up his food by his rumination activity, and the oral feedings had to be temporarily stopped. At this point, Luca’s mother decided to stop working and take Luca home to continue an intensive behavioral “distracting” intervention in order to interrupt his rumination during meals. Luca’s mother started small feedings while playing with him during and after feedings, and was able to interest him in other activities, so that he would not ruminate. After 4 weeks of slow increments in his feedings, Luca was able to take all his feedings by mouth without ruminating, and the nasojejunal tube could be removed. Luca and his mother continued to use simulating and distracting activities during and just after meals, which over time became more interesting to Luca than his previous ruminating behavior. PATHOLOGY AND LABORATORY EXAMINATION No specific laboratory examination is pathognomonic of rumination disorder; however, rumination disorder is not uncommonly associated with gastrointestinal abnormalities. Clinicians are recommended to evaluate other physical causes of vomiting, such as pyloric stenosis and hiatal hernia, before making the diagnosis of rumination disorder. Rumination disorder can lead to states of malnutrition and dehydration. In very severe cases, laboratory measures of endocrinological function, serum electrolytes, and a hematological workup may determine the need for medical intervention. DIFFERENTIAL DIAGNOSIS To make the diagnosis of rumination disorder, clinicians must rule out primary gastrointestinal congenital anomalies, infections, and other medical illnesses that could account for frequent regurgitation. Pyloric stenosis is usually associated with projectile vomiting and is generally evident before 3 months of age, when rumination has its onset. Rumination has been associated with both autism spectrum disorder and intellectual disability in which stereotypic behaviors and eating disturbances are not uncommon. Rumination behavior may occur comorbidly in youth with severe anxiety disorders as well. Rumination disorder may also occur in patients with other eating disorders, such as anorexia nervosa and bulimia nervosa. COURSE AND PROGNOSIS Rumination disorder is believed to have a high rate of spontaneous remission. Indeed, many cases of rumination disorder may develop and remit without ever being diagnosed. Limited data are available about the prognosis of rumination disorder in adolescents and adults. Behavioral interventions using habit-reversal techniques may significantly lead to improved prognosis. 21 - 31.9c AvoidantRestrictive Food Intake Disorde 31.9c Avoidant/Restrictive Food Intake Disorder TREATMENT The treatment of rumination disorder is often a combination of education and behavioral techniques. Sometimes, an evaluation of the mother–child relationship reveals deficits that can be influenced by offering guidance to the mother. Behavioral interventions, such as habit-reversal are aimed at reinforcing an alternate behavior that becomes more compelling than the behaviors leading to regurgitation. Aversive behavioral interventions, such as squirting lemon juice into the infant’s mouth whenever rumination occurs, have been used in the past to diminish rumination behavior. Although aversive behavioral interventions have been reported anecdotally to be effective in some cases, current recommendations support the use of habit-reversal techniques. When features of child maltreatment of neglect may have contributed to rumination behaviors in an infant, treatments include improvement of the child’s psychosocial environment, increased tender loving care from the mother or caretakers, and psychotherapy for the mother or both parents. Anatomical abnormalities, such as hiatal hernia, are not uncommon, and must be evaluated, in some cases leading to surgical repair. In severe cases in which malnutrition and weight loss have occurred, placement of a jejunal tube may need to be inserted before other treatments can be utilized. Medication is not a standard part of the treatment of rumination. Case reports, however, cite a variety of medications that have been tried, including metoclopramide (Reglan), cimetidine (Tagamet), and even antipsychotics such as haloperidol (Haldol) have been cited to be helpful according to anecdotal reports. The treatment of adolescents with rumination disorder is often complex and includes a multidisciplinary approach consisting of individual psychotherapy, nutritional intervention, and pharmacologic treatment for the frequent comorbid anxiety and depressive symptoms. 31.9c Avoidant/Restrictive Food Intake Disorder Avoidant/restrictive food intake disorder, formerly known as feeding disorder of infancy or early childhood, is characterized by a lack of interest in food, or its avoidance based on the sensory features of the food or the perceived consequences of eating. This newly included DSM-5 disorder adds more detail about the nature of the eating problems, and has also been expanded to include adolescents and adults. The disorder is manifested by a persistent failure to meet nutritional or energy needs as evidenced by one or more of the following: significant weight loss or failure to achieve expected weight, nutritional deficiency, dependence on enteral feedings or nutritional supplements, or marked interference with psychosocial functioning. It may take the form of outright food refusal, food selectivity, eating too little, food avoidance, and delayed self-feeding. The diagnosis should not be made in the context of anorexia nervosa or bulimia nervosa, or if caused by a medical condition, by another mental disorder, or by a true lack of available food. Infants and children with the disorder may be withdrawn, irritable, apathetic, or anxious. Because of the avoidant behavior during feeding, touching and holding between mothers and infants are diminished during the entire feeding process compared with other children. Some reports suggest that food avoidance or restriction may be relatively long-standing; however, in many cases, normal adult functioning is eventually achieved. EPIDEMIOLOGY It is estimated that between 15 percent and 35 percent of infants and young children have transient feeding difficulties. A study of restrictive eating difficulties in Swedish 9year-olds and 12-year-olds found that restrictive eating problems were present in 0.6 percent of their sample. However, another study of avoidant eating patterns in young children in Germany, found that some degree of avoidance was present in up to 53 percent of children. Thus, avoidant eating behaviors without impairment of nutritional state or psychosocial functioning must be separated from restricted eating disturbances leading to significant functional impairment. A survey of feeding problems in nursery school children revealed a prevalence of 4.8 percent with equal gender distribution. In that study, children with feeding problems exhibited more somatic complaints and mothers of affected infants exhibited increased risk of anxiety symptoms. Data from community samples estimate a prevalence of failure to thrive syndromes in approximately 3 percent of infants, with approximately half of those infants exhibiting feeding disorders. DIFFERENTIAL DIAGNOSIS The disorder must be differentiated from structural problems with the infants’ gastrointestinal tract that may be contributing to discomfort during the feeding process. Because feeding disorders and organic causes of swallowing difficulties often coexist, it is important to rule out medical reasons for feeding difficulties. A study of videofluoroscopic evaluation of children with feeding and swallowing problems revealed that clinical evaluation was 92 percent accurate in identifying those children at increased risk of aspiration. This type of evaluation is necessary before psychotherapeutic interventions in cases where a medical contribution to feeding problems is suspected. COURSE AND PROGNOSIS Most infants with feeding disorder who are identified within the first year of life and who receive treatment do not go on to develop malnutrition, growth delay, or failure to thrive. When feeding disorders have their onset later, in children 2 to 3 years of age, growth and development can be affected when the disorder lasts for several months. In older children, or adolescents, the feeding disorder typically interferes with social functioning, until treated. It is estimated that about 70 percent of infants who persistently refuse food in the first year of life continue to have some eating problems during childhood. Jennifer was 6 months old when she was referred for a psychiatric evaluation because of feeding difficulties, irritability, and poor weight gain since birth. She was small and slight, but she did not appear to be lethargic or malnourished. Her parents were college-educated, and both had pursued their professional careers until Jennifer was born. Although Jennifer was full-term and weighed 7 pounds at birth, she had been unable to be breast feed due to turning away and not ingesting enough milk. When she was 4-weeks-old, Jennifer’s mother had reluctantly switched her to bottle feedings because Jennifer was losing weight. Although her intake improved somewhat on bottle feedings, she gained weight very slowly and was still less than 8 pounds at 3 months of age. Since then, she had gained a minimal amount each month to maintain a low but adequate weight. Jennifer’s mother appeared tired and described that Jennifer would drink only up to about 6 ounces at a time, or two bites of baby food, and then wiggle and cry; and refuse to continue with the feeding. But after a few hours, she might cry again as if she were hungry. However, she could not settle her into a good rhythm of feeding, and continued attempts to feed her would lead her to cry inconsolably. Jennifer’s mother described approximately 10 to 15 attempts at feeding her both liquids and solids in a 24-hour period. Jennifer was reported to be an irritable and fussy infant, who cried multiple times during the day and at night, and woke her family often during the night with her crying. Jennifer’s developmental milestones such as sitting up, tracking, and making sounds were within normal limits. The observation of mother–infant interactions during feeding and play revealed that Jennifer was a very alert and wiggly baby who had difficulty sitting still. While drinking from the bottle she would kick her feet and move around, and if the bottle slipped out of her mouth, she did not try to recapture it. When eating baby foods, she was not interested and her mother had to coax her to open her mouth. This upset Jennifer, and she would start crying. Jennifer’s mother reported that she was always anxious during meals, and would try to convince Jennifer to take spoonfuls of baby food while sitting in her high chair. After repeated unsuccessful attempts of adequate feeding, Jennifer and her mother both appeared exhausted and took a break. The history and examination revealed that Jennifer was a very active and excitable baby who had difficulty keeping calm during feedings. After reviewing the videotape with the mother, the therapist explored ways in which the mother could better facilitate calming Jennifer before and during meals. Using a quiet corner in the house, and singing to Jennifer before meals resulted in Jennifer remaining more calm during meals, and she was able to drink larger amounts of milk, eat more solid foods, and waited longer between meals. This, in turn, relieved her mother’s anxiety and helped both to have calmer interactions. (Adapted by Caroly Pataki, M.D.) TREATMENT Most interventions for feeding disorders are aimed at optimizing the interaction between the mother and infant during feedings and identifying any factors that can be changed to promote greater ingestion. The mother is helped to become more aware of the infant’s stamina for length of individual feedings, the infant’s biological regulation patterns, and the infant’s fatigue level with a goal of increasing the level of engagement between mother and infant during feeding. A transactional model of intervention has been proposed for infants who exhibit the “difficult” temperamental traits of emotional intensity, stubbornness, lack of hunger cues, and irregular eating and sleeping patterns. The treatment includes education for the parents regarding the temperamental traits of the infant, exploration of the parents’ anxieties about the infant’s nutrition, and training for the parents regarding changing their behaviors to promote internal regulation of eating in the infant. Parents are encouraged to feed the infant on a regular basis at 3- to 4-hour intervals, and offer only water between meals. The parents are trained to deliver praise to the infant for any self-feeding efforts, regardless of the amount of food ingested. Furthermore, parents are guided to limit any distracting stimulation during meals and give attention and praise to positive eating behaviors rather than intense negative attention to inappropriate behavior during meals. This training process for parents is done in an intense manner within a short period of time. Many parents are able to facilitate improved eating patterns in the infant as a result. If the mother or caregiver is unable to participate in the intervention, it may be necessary to include additional caregivers to contribute to feeding the infant. In rare cases, an infant may require hospitalization until adequate nutrition on a daily basis is accomplished. If an infant tires before ingesting an adequate amount of nutrition, it may be necessary to begin treatment with the placement of a nasogastric tube for supplemental oral feedings. For older children with failure-to-thrive syndromes, hospitalization and nutritional supplementation may be necessary. Medication is not a standard component of treatment for feeding disorders; however, there are anecdotal reports of preadolescents with failure-to-thrive and feeding disorders who were comorbid for anxiety and mood symptoms and who received enteral nutritional interventions in addition to risperidone (Risperdal), and who were observed to have an increase in oral intake and accelerated weight gain. REFERENCES Araujo CL, Victora CG, Hallal PC, Gigante DP. Breastfeeding and overweight in childhood: Evidence from the Pelotas 1993 birth cohort study. Int J Obes. 2005;30(3):500. Berger-Gross P, Colettoi DJ, Hirschkorn K, Terranova E, Simpser EF. The effectiveness of risperidone in the treatment of three children with feeding disorders. J Child Adolesc Psychopharmacol. 2004;14:621. Bryant-Waugh R. Feeding and eating disorders in children. Curr Opin Psychiatry. 2013;26:537–542. Bryant-Waugh R. Avoidant restrictive food intake disorder: An illustrative case example. Int J Eat Disord. 2013;46:420–423. Call C, Walsh BT, Attia E. From DSM-IV to DSM-5: Changes to eating disorder diagnoses. Curr Opin Psychiatry. 22 - 31.10 Elimination Disorders 31.10 Elimination Disorders 2013;26:532–536. Chatoor I. Feeding and eating disorders of infancy or early childhood. In: Sadock BJ, Sadock VA, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Vol. II. Philadelphia: Lippincott Williams & Wilkins; 2009:3597. Chial HJ, Camilleri M, Williams DE, Litzinger K, Perrault J. Rumination syndrome in children and adolescents: Diagnosis, treatment, and prognosis. Pediatrics. 2003;111:158–162. Cohen E, Rosen Y, Yehuda B, Iancu I. Successful multidisciplinary treatment in an adolescent case of rumination. Isr J Psychiatry Relat Sci. 2004;41:222. DeMatteo C, Matovich D, Hjartarson A. Comparison of clinical and videofluoroscopic evaluation of children with feeding and swallowing difficulties. Dev Med Child Neurol. 2005;47:149. Esparo G, Canals J, Ballespi S, Vinas F, Domenech E. Feeding problems in nursery children: Prevalence and psychosocial factors. Acta Pediatr 2004;93:663. Equit M, Palmke M, Beckner N. Problems in young children: a population based study. Acta Paediatr. 2013:10. Feldaman R, Keren M, Gross-Rozval O, Tyano S. Mother-child touch patterns in infant feeding disorders: Relation to maternal, child, and environmental factors. J Am Acad Child Adolesc Psychiatry. 2004;43:1089. Hughes SO, Anderson CB, Power TG, Micheli N, Jaramillo S, Nicklas TA. Measuring feeding in low-income AfricanAmerican and Hispanic parents. Appetite. 2006;46(2):215. Jacobi C, Agras WS, Bryson S, Hammer LD. Behavioral validation, precursors, and concomitants of picky eating in childhood. J Am Acad Child Adolesc Psychiatry. 2003;42:76. Lewinsohn PM, Holm-Denoma JM, Gau JM, Joiner TE Jr, Striegel-Moore R, Bear P, Lamoureux B. Problematic eating and feeding behaviors of 36-month-old children. Int J Eat Disord. 2005;38(3):208–219. Linscheid TN. Behavioral treatments for pediatric feeding disorders. Behav Modif. 2006;30:6–23. Liu YL, Malik N, Sanger GJ, Friedman MI, Andrews PL. Pica—A model of nausea? Species differences in response to cisplatin. Physiol Behav. 2005;85(3):271–277. Ornstein RM, Rosen DS, Mammel K, Callahan ST, Forman S. Distribution of eating disorders in children and adolescents using the proposed DSM-5 criteria for feeding and eating disorders. J Adolesc Health. 2013;53:303–305. Rajindrajith S., Devanarayana NM, Perera BJC. Rumination syndrome in children and adolescents: a school survey assessing prevalence and symptomatology. BMC Gastroenterol. 2012;12:163–169. Rastam M, Taljemark J, Tajnia A. Eating problems and overlap with ADHD and autism spectrum disorders in a nationwide twin study of 9- and 12-year-old children Sci World J. 2013;15:315429. Tack J, Blondeau K, Boecxstaens V, Rommel N. Review article: The pathophysiology, differential diagnosis and management of rumination syndrome. Ailment Pharmacol Ther. 2011;33:782–788. Uher R, Rutter M. Classification of feeding and eating disorders: Review of evidence and proposals for ICD-11. World Psychiatry. 2012;11:80–92. Williams DE, McAdam D. Assessment, behavioral treatment, and prevention of pica: Clinical guidelines and recommendations for practitioners. Res Develop Disab. 2012;33:2050–2057. 31.10 Elimination Disorders The developmental milestones of mastering control over bowel and bladder function are complex processes that involve motor and sensory functions, coordinated through frontal lobe activities, and regulated by neurons in the pons and midbrain area. Mastery of bowel and bladder function is achieved over a period of months for the typical 23 - 31.10a Encopresis 31.10a Encopresis toddler. Infants generally void small volumes of urine approximately every hour, commonly stimulated by feeding, and may have incomplete emptying of the bladder. As the infant matures to be a toddler, bladder capacity increases, and between 1 and 3 years of age, cortical inhibitory pathways develop that allow the child to have voluntary control over reflexes that control the bladder muscles. The ability to have muscular control over the bowel occurs even before bladder control for most toddlers, and the assessment of fecal soiling includes determining whether the clinical presentation occurs with or without chronic constipation and overflow soiling. The normal sequence of developing control over bowel and bladder functions is the development of nocturnal fecal continence, diurnal fecal continence, diurnal bladder control, and nocturnal bladder control. Bowel and bladder control develops gradually over time. Toilet training is affected by many factors, such as a child’s intellectual capacity and social maturity, cultural determinants, and the psychological interactions between child and parents. The ability to control bowel and bladder functions depends on the maturation of neurobiological systems, so that children with developmental delays may also display delayed continence of bowel and bladder. When children exhibit incontinence of urine or feces on a regular basis, it is troubling to the child and families, and often misunderstood as voluntary misbehavior. Encopresis (repeated passage of feces into inappropriate places) and enuresis (repeated urination into bed or clothes) are the two elimination disorders described in the Fifth Edition of the American Psychiatric Association’s Diagnostic and Statistical Manual of Mental Disorders (DSM-5). These diagnoses are not made until after age 4 years, for encopresis, and after age 5 years for enuresis, the ages at which a typically developing child is expected to master these skills. Normal development encompasses a range of time in which a given child is able to devote the attention, motivation, and physiological skills to exhibit competency in elimination processes. Encopresis is characterized by a pattern of passing feces in inappropriate places, such as in clothing or other places, at least once per month for 3 consecutive months, whether the passage is involuntary or intentional. Up to about 80 percent of children with fecal incontinence have associated constipation. A child with encopresis typically exhibits dysregulated bowel function; for example, with infrequent bowel movements, constipation, or recurrent abdominal pain and sometimes pain on defecations. Enuresis is characterized by repeated voiding of urine into clothes or bed, whether the voiding is involuntary or intentional. The behavior must occur twice weekly for at least 3 months or must cause clinically significant distress or impairment socially or academically. The child’s chronological or developmental age must be at least 5 years. 31.10a Encopresis EPIDEMIOLOGY Encopresis has been estimated to affect 3 percent of 4-year-olds and 1.6 percent of 10- year-old children. Incidence rates for encopretic behavior decrease drastically with increasing age. Between the ages of 10 years and 12 years, it is estimated to affect 0.75 percent of typically developing children. Globally, community prevalence of encopresis ranges from 0.8 to 7.8 percent. In Western cultures, bowel control is established in more than 95 percent of children by their fourth birthday and in 99 percent by the fifth birthday. Encopresis is virtually absent in youth with normal intellectual function by the age of 16 years. Males are found to from three to six times more likely to have encopresis than females. A significant relation exists between encopresis and enuresis. ETIOLOGY Ninety percent of chronic childhood encopresis is considered to be functional. Children with this disorder typically withhold feces by contracting their gluteal muscles, holding their legs together, and tightening their external anal sphincter. In some cases, this is an entrenched behavioral response to previously painful bowel movements due to hard stool, which leads to fear of defecation and withholding behaviors. Encopresis involves an often-complicated interplay between physiological and psychological factors leading to an avoidance of defecation. However, when children chronically hold in bowel movements, the result is often fecal impaction and eventual overflow soiling. This pattern is observed in more than 75 percent of children with encopretic behavior. This common set of circumstances in most children with encopresis supports a behavioral intervention with a focus on ameliorating constipation while increasing appropriate toileting behavior. Inadequate training or the lack of appropriate toilet training may delay a child’s attainment of continence. Evidence indicates that some encopretic children have lifelong inefficient and ineffective sphincter control. Other children may soil involuntarily, either because of an inability to control the sphincter adequately or because of excessive fluid caused by a retentive overflow. In about 5 to 10 percent of cases, fecal incontinence is caused by medical conditions including abnormal innervation of the anorectal region, ultrashort segment Hirschsprung disease, neuronal intestinal dysplasia, or spinal cord damage. One study found encopresis to occur with significantly greater frequency among children with known sexual abuse, and other psychiatric disorders, compared with a sample of healthy children. Encopresis, however, is not a specific indicator of sexual abuse. It is evident that once a child has developed a pattern of withholding bowel movements, and attempts to defecate have become painful, a child’s fear and resistance to changing the pattern are high. Battles with parents who insist that their children attempt to defecate before they are adequately treated may aggravate the condition and cause secondary behavioral difficulties. Children with encopresis who are not promptly treated, however, frequently end up being socially ostracized and rejected. The social consequences of soiling can lead to the development of emotional problems. On the other hand, children with encopresis who clearly can control their bowel function adequately but chronically deposit feces of relatively normal consistency in abnormal places are likely to have preexisting neurodevelopmental problems. Occasionally, a child has a specific fear of using the toilet, leading to a phobia. Encopresis, in some cases can be considered secondary, that is, emerging after a period of normal bowel habits in conjunction with a disruptive life event, such as the birth of a sibling or a move to a new home. When encopresis manifests after a long period of fecal continence, it may reflect a developmental regressive behavior based on a severe stressor, such as a parental separation, loss of a best friend, or an unexpected academic failure. Megacolon Most children with encopresis retain feces and become constipated, either voluntarily or secondary to painful defecation. In some cases a subclinical preexisting anorectal dysfunction exists that contributes to the constipation. In either case, resulting chronic rectal distention from large, hard fecal masses can cause loss of tone in the rectal wall and desensitization to pressure. Thus, children in this situation become even less aware of the need to defecate, and overflow encopresis occurs, usually with relatively small amounts of liquid or soft stool leaking out. DIAGNOSIS AND CLINICAL FEATURES According to DSM-5, encopresis is diagnosed when feces are passed into inappropriate places on a regular basis (at least once a month) for 3 months. Encopresis may be present in children who have bowel control and intentionally deposit feces in their clothes or other places for a variety of emotional reasons. Anecdotal reports have suggested that occasionally encopresis is attributable to an expression of anger or rage in a child whose parents have been punitive or of hostility at a parent. In a case such as this, once a child develops this inappropriate repetitive behavior eliciting negative attention, it is difficult to break the cycle of continuous negative attention. In other children, sporadic episodes of encopresis can occur during times of stress—for example, proximal to the birth of a new sibling—but in such cases, the behavior is usually transient and does not fulfill the diagnostic criteria for the disorder. Encopresis can also be present on an involuntary basis in the absence of physiological abnormalities. In these cases, a child may not exhibit adequate control over the sphincter muscles, either because the child is absorbed in another activity or because he or she is unaware of the process. The feces may be of normal, near-normal, or liquid consistency. Some involuntary soiling occurs from chronic retaining of stool, which may result in liquid overflow. In rare cases, the involuntary overflow of stool results from psychological causes of diarrhea or anxiety disorder symptoms. The DSM-5 includes two specifiers to encopresis: with constipation and overflow incontinence and without constipation and overflow incontinence. To receive a diagnosis of encopresis, a child must have a developmental or chronological level of at least 4 years. If the fecal incontinence is directly related to a medical condition, encopresis is not diagnosed. Studies have indicated that children with encopresis who do not have gastrointestinal illnesses have high rates of abnormal anal sphincter contractions. This finding is particularly prevalent among children with encopresis with constipation and overflow incontinence who have difficulty relaxing their anal sphincter muscles when trying to defecate. Children with constipation who have difficulties with sphincter relaxation are not likely to respond well to laxatives in the treatment of their encopresis. Children with encopresis without abnormal sphincter tone are likely to improve over a short period. Jack was a 7-year-old boy with daily encopresis, enuresis, and a history of hoarding behaviors, along with hiding the feces around the house. He lived with his adoptive parents, having been removed from his biological parents at age 3 years because of neglect and physical abuse. He was reported to be cocaine addicted at birth, but was otherwise healthy. Jack’s biological mother was a known methamphetamine and alcohol user, and his father had spent time in jail for drug dealing. Jack had always been enuretic at night, and until this year, he had a history of daytime enuresis as well. Jack had a short attention span, was highly impulsive, and had great difficulty staying in his seat at school and remaining on task. He had reading difficulties and was placed in a contained special education classroom because of his disruptive behavior as well as his academic difficulties. Despite experiencing physical abuse, he has not experienced flashbacks or other symptoms that would indicate the presence of posttraumatic stress disorder. Jack was treated for attention-deficit/hyperactivity disorder (ADHD) with good response to methylphenidate (Concerta 36 mg per day). Jack’s adoptive family sought help at a university hospital’s outpatient program that had expertise in the behavioral treatments of many psychiatric disorders including encopresis. The treatment program combined use of regular laxatives and a bowel training method with cognitive-behavioral therapy for Jack and for his family. Jack was started on a regimen of daily polyethylene glycol (PEG) solution and was seen by a pediatrician who was able to perform a manual disimpaction under sedation. Following that, Jack was continued on daily PEG solution combined with therapy. He learned to empty his bowel while sitting on the toilet for 10 minutes after each meal, whether or not he felt like he had to go. He soon was eager to stay on this regular bathroom schedule, and felt proud when he was able to have a bowel movement in the toilet. Over a period of 3 months, Jack was noticeably improved, and at 6 months, he was almost completely better. (Courtesy of Edwin J. Mikkelsen, M.D. and Caroly Pataki, M.D.) PATHOLOGY AND LABORATORY EXAMINATION Although no specific test indicates a diagnosis of encopresis, clinicians must rule out medical illnesses, such as Hirschsprung’s disease, before making a diagnosis. It must be determined whether fecal retention is responsible for encopresis with constipation and overflow incontinence; a physical examination of the abdomen is indicated, and an abdominal X-ray can help determine the degree of constipation present. Tests to determine whether sphincter tone is abnormal are generally not conducted in simple cases of encopresis. DIFFERENTIAL DIAGNOSIS In encopresis with constipation and overflow incontinence, constipation can begin as early as the child’s first year and can peak between the second and fourth years. Soiling usually begins by age 4. Frequent liquid stools and hard fecal masses are found in the colon and the rectum on abdominal palpation and rectal examination. Complications include impaction, megacolon, and anal fissures. Encopresis with constipation and overflow incontinence is rarely caused by faulty nutrition; structural disease of the anus, rectum, and colon; medicinal adverse effects; or nongastrointestinal medical (endocrine or neurological) disorders. The chief differential medical problem is aganglionic megacolon or Hirschsprung’s disease, in which a patient may have an empty rectum and no desire to defecate, but may still have an overflow of feces. The disorder occurs in 1 in 5,000 children; signs appear shortly after birth. COURSE AND PROGNOSIS The outcome of encopresis depends on the etiology, the chronicity of the symptoms, and coexisting behavioral problems. In some cases, encopresis is self-limiting, and it rarely continues beyond middle adolescence. Encopresis in children who have contributing physiological factors, such as poor gastric motility and an inability to relax the anal sphincter muscles, is more difficult to treat than that in those with constipation but normal sphincter tone. Encopresis is a particularly objectionable disorder to family members, who may assume that the behavior is due to “laziness,” and family tensions are often high. Peers are intolerant of the developmentally inappropriate behavior and typically taunt and reject a child with encopresis. Many affected children have abysmally low self-esteem and are plagued by constant social rejection. Psychologically, a child may appear blunted toward the symptoms or less frequently, may be entrenched in a pattern of encopresis as a mode of expressing anger. The outcome of encopresis is influenced by a family’s willingness and ability to participate in treatment without being overly punitive and by the child’s ability and motivation to engage in treatment. TREATMENT A typical treatment plan for a child with encopresis includes daily oral administration of laxatives such as PEG at 1 g/kg per day, and often a surgical disimpaction under general anesthesia before maintenance laxatives can be administered. In addition, an ongoing cognitive-behavioral intervention to begin regular attempts to have bowel movements in the toilet, and to diminish anxiety related to bowel movement. By the time a child is brought for treatment, considerable family discord and distress are common. Family tensions about the symptom must be reduced, and a nonpunitive atmosphere established. Similar efforts should be made to reduce the child’s embarrassment at school. Many changes of underwear with a minimum of embarrassment should be arranged. Education of the family and correction of misperceptions that a family may have about soiling must occur before treatment. Laxatives are not necessary for children who are not constipated and do have good bowel control, but regular, timed intervals on the toilet may be useful with these children as well. A report confirms the success of an interactive parent–child family guidance intervention for young children with encopresis based on psychological and behavioral interventions for children younger than age 9 years. Supportive psychotherapy and relaxation techniques may be useful in treating the anxieties and other sequelae of children with encopresis, such as low self-esteem and social isolation. Family interventions can be helpful for children who have bowel control but who continue to deposit their feces in inappropriate locations. An optimal outcome occurs when a child achieves a feeling of control over his or her bowel function. REFERENCES Bahar RJ, Reid H. Treatment of encopresis and chronic constipation in young children: Clinical results from interactive parent-child guidance. Clin Pediatr. 2006;45:157. Benninga MA, Voskuijl WP, Akkerhius GW, Taminiau JA, Buller HA. Colonic transit times and behaviour profiles in children with defecation disorders. Arch Dis Child. 2004;89:13. Brazzeli M, Griffiths P. Behavioural and cognitive interventions with or without other treatments for the management of fecal incontinence in children. Cochrane Database Syst Rev. 2006;19:CD002240. Di Lorenzo C, Benninga MA. Pathophysiology of pediatric fecal incontinence. Gastroenterology. 2004;126[Suppl 1]:S533. Har AF, Croffie JM. Encopresis. Pediatr Rev. 2010;31:368–374. Kajiwara M, Inoue K, Kato M, Usui A, Kurihara M, Usui T. Nocturnal enuresis and overactive bladder in children: An epidemiological study. Int J Urol. 2006;13:36. Klages T, Geller B, Tillman R, Bolhofner K, Zimerman B. Controlled study of encopresis and enuresis in children with a prepubertal and early adolescent bipolar-I disorder phenotype. J Am Acad Child Adolesc Psychiatry. 2005;44:1050. Mellon MW, Whiteside SP, Friedrich WN. The relevance of fecal soiling as an indicator of child sexual abuse: A preliminary analysis. J Dev Behav Pediatr. 2006;27:25. Mikkelsen EJ. Elimination disorders. In: Sadock BJ, Sadock VA, Ruiz P, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Vol. II. Philadelphia: Lippincott Williams & Wilkins; 2009:3624. Mugie SM, Di Lorenzo C, Benninga MA. Constipation in childhood. Gastroenterol Hepatol. 2011;8:502–511. Rajindrajith S, Devanarayana NM, Benninga MA. Review article: Faecal incontinence in children: Epidemiology, pathophysiology, clinical evaluation and management. Aliment Pharmacol Ther. 2013;37:37–48. Reiner WG. Pharmacology in the management of voiding and storage disorders, including enuresis and encopresis. J Am Acad Child Adolesc Psychiatry. 2008;47:491–498. Rowan-Legg A. Managing functional constipation in children. Paediatr Child Health. 2011;16:661–665. Von Gontard A, Hollmann E. Comorbidity of functional urinary incontinence and encopresis: somatic and behavioral associations. J Urology. 2004;171:2644. 24 - 31.10b Enuresis 31.10b Enuresis Yilmaz S, Bigic A, Herguner S. Effect of OROS methylphenidate on encopresis in children with attentiondeficit/hyperactivity disorder. J Child Adolesc Psychopharmacol. 2013; Oct 29. [Epub ahead of print]. 31.10b Enuresis EPIDEMIOLOGY The prevalence of enuresis ranges from 5 to 10 percent in 5-year-olds, 1.5 to 5 percent in 9- to 10-year-olds, and about 1 percent in adolescents 15 years and older. The prevalence of enuresis decreases with increasing age. Enuretic behavior is considered developmentally appropriate among young toddlers, precluding diagnoses of enuresis; however, enuretic behavior occurs in 82 percent of 2-year-olds, 49 percent of 3-yearolds, and 26 percent of 4-year-olds on a regular basis. In the epidemiological Isle of Wight study, investigators reported that 15.2 percent of 7-year-old boys were enuretic occasionally and that 6.7 percent of them were enuretic at least once a week. The study reported that 3.3 percent of girls at the age of 7 years were enuretic at least once a week. By age 10, the overall prevalence of enuresis was reported to be 3 percent. The rate drops drastically for teenagers: a prevalence of 1.5 percent has been reported for 14-year-olds. Enuresis affects about 1% of adults. Although most children with enuresis do not have a comorbid psychiatric disorder, children with enuresis are at higher risk for the development of another psychiatric disorder. Nocturnal enuresis is about 50 percent more common in boys and accounts for about 80 percent of children with enuresis. Diurnal enuresis is also seen more often in boys who often delay voiding until it is too late. A spontaneous resolution of nocturnal enuresis is about 15 percent per year. Nocturnal enuresis consists of a normal volume of voided urine, whereas when small volumes of urine are voided at night, other medical causes may be present. ETIOLOGY Enuresis involves complex neurobiological systems that include contributions from cerebral and spinal cord centers, motor and sensory functions, and autonomic and voluntary nervous systems. Urination is regulated by neurons in the pons and midbrain regions. Bladder detrusor muscle contraction occurs whenever bladder capacity is reached, which can lead to enuresis in a sleeping child. Therefore, excessive volumes of urine produced at night may lead to enuresis at night in children without any physiologic abnormalities. Nighttime enuresis often occurs in the absence of a specific neurogenic cause. Daytime enuresis may develop based on behavioral habits developed over time. Daytime enuresis may occur in the absence of neurological abnormalities resulting from habitual, voluntary tightening of the external sphincter during urges to urinate. The pattern may be set in a young child who may start out with a normal or overactive detrusor muscle in the bladder, but with repeated attempts to prevent leaking or urination when there is an urge to void. Over time, the sensation of the urge to urinate is diminished and the bladder does not empty regularly, leading to enuresis at night when the bladder is relaxed and can empty without resistance. This immature pattern of urinating can account for some cases of enuresis, especially when the pattern has been in place since early childhood. Most children are not enuretic by intention or even with awareness until after they are wet. Physiological factors often play a role in the development of enuresis, and behavioral patterns are likely to maintain the maladaptive urination. Normal bladder control, which is acquired gradually, is influenced by neuromuscular and cognitive development, socioemotional factors, toilet training, and genetic factors. Difficulties in one or more of these areas can delay urinary continence. Genetic factors are believed to play a role in the expression of enuresis, given that the emergence of enuresis has been found to be significantly greater in first-degree relatives. A longitudinal study of child development found that children with enuresis were about twice as likely to have concomitant developmental delays as those who did not have enuresis. About 75 percent of children with enuresis have a first-degree relative who has or has had enuresis. A child’s risk for enuresis has been found to be more than seven times greater if the father was enuretic. The concordance rate is higher in monozygotic twins than in dizygotic twins. A strong genetic component is suggested, and much can be accounted for by tolerance for enuresis in some families and by other psychosocial factors. Studies indicate that children with enuresis with a normal anatomical bladder capacity report urge to void with less urine in the bladder than children without enuresis. Other studies report that nocturnal enuresis occurs when the bladder is full because of lower than expected levels of nighttime antidiuretic hormone. This could lead to a higher-than-usual urine output. Enuresis does not appear to be related to a specific stage of sleep or time of night; rather, bed-wetting appears randomly. In most cases, the quality of sleep is normal. Little evidence indicates that children with enuresis sleep more soundly than other children. Psychosocial stressors appear to precipitate enuresis in a subgroup of children with the disorder. In young children, the disorder has been particularly associated with the birth of a sibling, hospitalization between the ages of 2 and 4 years, the start of school, separation of a family due to divorce, or a move to a new environment. DIAGNOSIS AND CLINICAL FEATURES Enuresis is the repeated voiding of urine into a child’s clothes or bed; the voiding may be involuntary or intentional. For the diagnosis to be made, a child must exhibit a developmental or chronological age of at least 5 years. According to DSM-5, the behavior must occur twice weekly for a period of at least 3 months or must cause distress and impairment in functioning to meet the diagnostic criteria. Enuresis is diagnosed only if the behavior is not caused by a medical condition. Children with enuresis are at higher risk for ADHD compared with the general population. They are also more likely to have comorbid encopresis. DSM-5 and the 10th revision of International Statistical Classification of Diseases and Related Health Problems (ICD-10) break down the disorder into three types: nocturnal only, diurnal only, and nocturnal and diurnal. PATHOLOGY AND LABORATORY EXAMINATION No single laboratory finding is pathognomonic of enuresis; but clinicians must rule out organic factors, such as the presence of urinary tract infections, which may predispose a child to enuresis. Structural obstructive abnormalities may be present in up to 3 percent of children with apparent enuresis. Sophisticated radiographic studies are usually deferred in simple cases of enuresis with no signs of repeated infections or other medical problems. DIFFERENTIAL DIAGNOSIS To make the diagnosis of enuresis, organic causes of bladder dysfunction must be investigated and ruled out. Organic syndromes, such as urinary tract infections, obstructions, or anatomical conditions are found most often in children who experience both nocturnal and diurnal enuresis combined with urinary frequency and urgency. The organic features include genitourinary pathology—structural, neurological, and infectious—such as obstructive uropathy, spina bifida occulta, and cystitis; other organic disorders that can cause polyuria and enuresis, such as diabetes mellitus and diabetes insipidus; disturbances of consciousness and sleep, such as seizures, intoxication, and sleepwalking disorder, during which a child urinates; and adverse effects from treatment with antipsychotic agents. COURSE AND PROGNOSIS Enuresis is often self-limited, and a child with enuresis may have a spontaneous remission. Most children who master the task of control over their bladder gain selfesteem and improved social confidence when they become continent. About 80 percent of affected children have never achieved a year-long period of dryness. Enuresis after at least one dry year usually begins between the ages of 5 and 8 years; if it occurs much later, especially during adulthood, organic causes must be investigated. Some evidence indicates that late onset of enuresis in children is more frequently associated with a concomitant psychiatric difficulty than is enuresis without at least one dry year. Relapses occur in children with enuresis who are becoming dry spontaneously and in those who are being treated. The significant emotional and social difficulties of these children usually include poor self-image, decreased self-esteem, social embarrassment and restriction, and intrafamilial conflict. The course of children with enuresis may be influenced by whether they receive appropriate evaluation and treatment for common comorbid disorders such as ADHD. TREATMENT A relatively high rate of spontaneous remission of enuresis occurs over time in childhood; however, in many cases, interventions are necessary because enuresis is causing functional impairment. The first step in any treatment plan is to review appropriate toilet training. If toilet training was not attempted, the parents and the patient should be guided in this undertaking. Record-keeping is helpful in determining a baseline and following the child’s progress, and may itself be a reinforcer. A star chart may be particularly helpful. Other useful techniques include restricting fluids before bed and night lifting to toilet train the child. Interventions with alarm therapy, which is triggered by wet underwear, has been a mainstay of treatment for enuresis. Alarm therapy works by alerting a child to respond when voiding begins during sleep. The alarm is a battery-operated device that can be attached to a child’s underwear or a mat. The alarm is triggered as soon as voiding begins by emitting a loud noise that awakens the child. The success of this method is based on the child’s ability to awaken promptly and respond to the alarm by getting up and voiding in the toilet. A child who can respond optimally is at least 6 or 7 years old. Pharmacological interventions including desmopressin therapy in managing nocturnal enuresis have been shown to be effective in some patients. Desmopressin is a “synthetic analogue” of vasopressin, which can be administered as a pill, a sublingual melt, or a nasal spray. Its effect can last up to 8 hours, and it works by reducing urine production at night. This method is optimal when no fluids are ingested in the evening. Another basic intervention for those children with enuresis and bowel dysfunction is to assess whether chronic constipation is contributing to urinary dysfunction, and to consider increasing dietary fiber to diminish constipation. Behavioral Therapy Classic conditioning with the bell (or buzzer) and pad (alarm) apparatus is generally the most effective treatment for enuresis, with dryness resulting in more than 50 percent of cases. Bladder training—encouragement or reward for delaying micturition for increasing times during waking hours—has also been used. Although sometimes effective, this method is decidedly inferior to the bell and pad. Pharmacotherapy Medication is considered when enuresis is causing impairment in social, family, and school function and behavioral, dietary, and fluid restriction have not been efficacious. When the problem interferes significantly with a child’s functioning, several medications can be considered, although the problem often recurs as soon as medications are withdrawn. Desmopressin (DDAVP), an antidiuretic compound that is available as an intranasal spray, has shown success in reducing enuresis. Reduction of enuresis has varied from 10 to 90 percent with the use of desmopressin. In most studies, enuresis recurred shortly after discontinuation of this medication. Adverse effects that can occur with desmopressin include headache, nasal congestion, epistaxis, and stomachache. The most serious adverse effect reported with the use of desmopressin to treat enuresis was a hyponatremic seizure experienced by a child. Reboxetine (Edronax, Vestra), a norepinephrine reuptake inhibitor with a noncardiotoxic side effect profile has recently been investigated as a safer alternative to imipramine in the treatment of childhood enuresis. A trial in which 22 children with enuresis causing social impairment, who had not responded to an enuresis alarm, desmopressin, or anticholinergics were administered 4 to 8 mg of reboxetine at bedtime. Of the 22 children, 13 (59 percent) in this open trial achieved complete dryness with reboxetine alone, or in combination with desmopressin. Side effects were minimal and did not lead to discontinuation of the medication in this trial. Psychotherapy Psychotherapy may be useful in dealing with the coexisting psychiatric problems and the emotional and family difficulties that arise secondary to chronic enuresis. REFERENCES Baeyens D, Roeyers H, D’Haese L, Pieters F, Hoebeke P, Vande Walle J. The prevalence of ADHD in children with enuresis: Comparison between a tertiary and non-tertiary care sample. Acta Paediatr. 2006;95:347. Brown ML, Pope AW, Brown EJ. Treatment of primary nocturnal enuresis in children: A review. Child Care Health Dev. 2010;37:153–160. Butler RJ, Heron J. The prevalence of infrequent bedwetting and nocturnal enuresis in childhood: A large British cohort. Scand J Urol Nephrol. 2008;42:257–264. Feldman AS, Bauer SB. Diagnosis and management of dysfunctional voiding. Curr Opin Pediatr. 2006;18:139. Fitzgerald MP, Thom DH, Wassel-Fyr C, Subak L, Brubaker L, Van Den Deden SK, Brown JS. Childhood urinary symptoms predict adult overactive bladder symptoms. J Urol. 2006;175:989. Friedman FM, Weiss JP. Desmopressin in the treatment of nocturia: clinical evidence and experience. Ther Adv Urol. 2013;5:310–317. Kajiwara M, Inoue K, Kato M, Usui A, Kurihara M, Usui T. Nocturnal enuresis and overactive bladder in children: An epidemiological study. Int J Urol. 2006;13:36. Klages T, Geller B, Tillman R, Bolhofner K, Zimerman B. Controlled study of encopresis and enuresis in children with a prepubertal and early adolescent bipolar-I disorder phenotype. J Am Acad Child Adolesc Psychiatry. 2005;44:1050. Landgraf JM, Abidari J, Cilento BG Jr., Cooper CS, Schulman SL, Ortenberg J. Coping, commitment, and attitude: Quantifying the everyday burden of enuresis on children and their families. Pediatrics. 2004;113:334. Mikkelsen EJ. Elimination disorders. In: Sadock BJ, Sadock VA, Ruiz P, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Vol. II. Philadelphia: Lippincott Williams & Wilkins; 2009:3624. Nevus T. Reboxetine in therapy-resistant enuresis: results and pathogenetic implications. Scand J Urol Nephrol. 2006;40:31. Pennesi M, Pitter M, Borduga A, Minisini S, Peratoner L. Behavioral therapy for primary nocturnal enuresis. J Urol. 2004; 171:408. Perrin N, Sayer L, White A. The efficacy of alarm therapy versus desmopressin therapy in the treatment of primary mono- 25 - 31.11 Trauma and Stressor Related Disorders i 31.11 Trauma- and Stressor-Related Disorders in Children 26 - 31.11a Reactive Attachment Disorder and Disin 31.11a Reactive Attachment Disorder and Disinhibited Social Engagement Disorder symptomatic nocturnal enuresis: A systematic review. Prim Health Care Res Dev. 2013; 1–11 Doi: 10.1p17/S146342361300042X. Reiner WG. Pharmacotherapy in the management of voiding and storage disorders, including enuresis and encopresis. J Am Acad Child Adolesc Psychiatry. 2008;47:5:491–498. Rutter M, Tizard J, Yule W, Graham P, Whitmore K. Research report: Isle of Wight Studies, 1964–1974. Psychol Med. 1976;6:313–332. Von Gontard A, Hollmann E. Comorbidity of functional urinary incontinence and encopresis: Somatic and behavioral associations. J Urol. 2004;171:2644. 31.11 Trauma- and Stressor-Related Disorders in Children This section includes disorders in which a traumatic or significantly stressful event is a necessary diagnostic criterion, according to the American Psychiatric Association’s Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5). Included are reactive attachment disorder, disinhibited social engagement disorder, and posttraumatic stress disorder (see 31.11b). The psychological and psychiatric symptoms that follow exposure to trauma and severe stress are variable and often include symptoms of anxiety, depression, dissociation, anger, and withdrawal. Previously, in the Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition, Text Revision (DSM-IV-TR), reactive attachment disorder was divided into two subtypes: emotionally withdrawn/inhibited and indiscriminately social/disinhibited. In DSM-5, however, the preceding two subtypes have been defined as two distinct disorders, with the DSM-5 reactive attachment disorder equivalent to the previous emotionally withdrawn/inhibited subtype, and disinhibited social engagement disorder representing the previous indiscriminately social disinhibited subtype. 31.11a Reactive Attachment Disorder and Disinhibited Social Engagement Disorder Reactive attachment disorder and disinhibited social engagement disorder are clinical disorders characterized by aberrant social behaviors in a young child that reflect grossly negligent parenting and maltreatment that disrupted the development of normal attachment behavior. A diagnosis of either reactive attachment disorder or disinhibited social engagement disorder is based on the presumption that the etiology is directly linked to the caregiving deprivation experienced by the child. The diagnosis of reactive attachment disorder was first defined in the DSM, Third Edition (DSM-III) in 1980. The formation of this diagnosis is based on the building blocks of attachment theory, which describes the quality of a child’s affective relationship with primary caregivers, usually parents. This basic relationship is the product of a young child’s need for protection, nurturance, and comfort and the interaction of the parents and child in fulfilling these needs. Based on observations of a young child and parents during a brief separation and reunion, designated the “strange situation procedure,” pioneered by Mary Ainsworth and colleagues, researchers have designated a child’s basic pattern of attachment to be characterized as secure, insecure, or disorganized. Children who exhibit secure attachment behavior are believed to experience their caregivers as emotionally available and appear to be more exploratory and well adjusted than children who exhibit insecure or disorganized attachment behavior. Insecure attachment is believed to result from a young child’s perception that the caregiver is not consistently available, whereas disorganized attachment behavior in a child is believed to result from experiencing both the need for proximity to the caregiver and apprehension in approaching the caregiver. These early patterns of attachment are believed to influence a child’s future capacities for affect regulation, self-soothing, and relationship building. According to the DSM-5, reactive attachment disorder is characterized by a consistent pattern of emotionally withdrawn responses toward adult caregivers, limited positive affect, sadness, and minimal social responsiveness to others, and concomitant neglect, deprivation, and lack of appropriate nurturance from caregivers. It is presumed that reactive attachment disorder is due to grossly pathological caregiving received by the child. The pattern of care may exhibit disregard for a child’s emotional or physical needs or repeated changes of caregivers, as when a child is frequently relocated during foster care. Reactive attachment disorder is not accounted for by autism spectrum disorder, and the child must have a developmental age of at least 9 months. Pathological caretaking can result in two distinct disorders: reactive attachment disorder, in which the disturbance takes the form of the child’s constantly failing to initiate and respond to most social interactions in a developmentally normal way; and disinhibited social engagement disorder, in which the disturbance takes the form of undifferentiated, unselective, and inappropriate social relatedness, with familiar and unfamiliar adults. In disinhibited social engagement disorder, according to DSM-5, a child actively approaches and interacts with unfamiliar adults in an overly familiar way, either verbally or physically. There is diminished checking with or seeking of a known caregiver, and a willingness to go with unfamiliar adults without hesitation. These behaviors in disinhibited social engagement disorder are not accounted for by impulsivity, although socially disinhibited behavior is predominant. These patterns of disinhibited, developmentally inappropriate behaviors are presumed to be caused by pathogenic caregiving. Thus, for both reactive attachment disorder and disinhibited social engagement disorder, aberrant caretaking is presumed to be the predominant cause of the child’s inappropriate behaviors. However, there have been cases of less severe disturbances in parenting that may also be associated with young children who exhibit some characteristics of reactive attachment disorder or disinhibited social engagement disorder. The DSM-5 criteria for reactive attachment disorder are described in Table 31.11a-1 and those for disinhibited social engagement disorder are described in Table 31.11a-2. Table 31.11a-1 DSM-5 Diagnostic Criteria for Reactive Attachment Disorder Table 31.11a-2 DSM-5 Diagnostic Criteria for Disinhibited Social Engagement Disorder These disorders may also result in a picture of failure to thrive, in which an infant shows physical signs of malnourishment and does not exhibit the expected developmental motor and verbal milestones. EPIDEMIOLOGY Few data exist on the prevalence, sex ratio, or familial pattern of reactive attachment disorder and disinhibited social engagement disorder. It has been estimated for either one to occur in less than 1 percent of the population. A study of 1,646 children aged 6to 8-years-old living in a deprived sector of urban United Kingdom, found that the prevalence of reactive attachment disorder in this population was 1.4 percent. However, other studies of selected high-risk populations have estimated that about 10 percent of young children with documented neglectful and grossly pathological caregiving exhibit reactive attachment disorder, and up to 20 percent of children in this situation exhibit disinhibited social engagement disorder. In a retrospective report of children in one county of the United States who were removed from their homes because of neglect or abuse before the age of 4 years, 38 percent exhibited signs of either reactive attachment disorder or disinhibited social engagement disorder. Another study established the reliability of the diagnosis by reviewing videotaped assessments of atrisk children interacting with caregivers, along with a structured interview with caregivers. Given that pathogenic care, including maltreatment, occurs more frequently in the presence of general psychosocial risk factors, such as poverty, disrupted families, and mental illness among caregivers, these circumstances are likely to increase the risk of reactive attachment disorder and disinhibited social engagement disorder. ETIOLOGY The core features of reactive attachment disorder and disinhibited social engagement disorder are disturbances of normal attachment behaviors. The inability of a young child to develop normative social interactions that culminate in aberrant attachment behaviors in reactive attachment disorder is inherent in the disorder’s definition. Reactive attachment disorder and disinhibited social engagement disorder are presumed to be linked to maltreatment of the child, including emotional neglect, physical abuse, or both. Grossly pathogenic care of an infant or young child by the caregiver presumably causes the markedly disturbed social relatedness that is evident. The emphasis is on the unidirectional cause; that is, the caregiver does something inimical or neglects to do something essential for the infant or child. In evaluating a patient for whom such a diagnosis is appropriate, however, clinicians should consider the contributions of each member of the caregiver–child dyad and their interactions. Clinicians should weigh such things as infant or child temperament, deficient or defective bonding, a developmentally disabled child, and a particular caregiver–child mismatch. The likelihood of neglect increases with parental psychiatric disorder, substance abuse, intellectual disability, the parent’s own harsh upbringing, social isolation, deprivation, and premature parenthood (i.e. adolescent). These factors compromise parental ability to attend to the needs of the child, as they focus primarily on their own existence rather than on their child. Frequent changes of the primary caregivers, for example, from multiple foster care placements or repeated lengthy hospitalizations, may also lead to impaired attachment. In the general population, a study of 1,600 children found that those children with reactive attachment disorder/disinhibited social engagement disorder showed a constellation of symptoms characterized by early emergence of symptoms eliciting neurodevelopmental examination (ESSENCE). Some of the associated symptoms in children with reactive attachment disorder/disinhibited social engagement disorder include higher risk of failure to gain weight as neonates, feeding difficulty, and poor impulse control. These traits are likely to emerge because of both genetic and environmental factors. The authors found that children with reactive attachment disorder/disinhibited social engagement disorder were more likely to have multiple psychiatric comorbidities, lower intelligence quotients (IQs) compared to the general population, and more behavioral problems. Thus, a broad assessment may be necessary to identify symptoms and disorders associated with reactive attachment disorder/disinhibited social engagement disorder. DIAGNOSIS AND CLINICAL FEATURES Children with reactive attachment disorder and disinhibited social engagement disorder may initially be identified by a preschool teacher or by a pediatrician based on direct observation of the child’s inappropriate social responses. The DSM-5 diagnostic criteria for reactive attachment disorder and disinhibited social engagement disorder are described in Tables 31.11a-1 and 31.11a-2, respectively. The diagnoses of reactive attachment disorder and disinhibited social engagement disorder are based partially on documented evidence of pervasive disturbance of attachment leading to inappropriate social behaviors present before the age of 5 years. The clinical picture varies greatly, depending on a child’s chronological and mental ages, but expected social interaction and liveliness are not present. Often, the child is not progressing developmentally or is frankly malnourished. Perhaps the most common clinical picture of an infant with reactive attachment disorder is the nonorganic failure to thrive. Such infants usually exhibit hypokinesis, dullness, listlessness, and apathy, with a poverty of spontaneous activity. Infants look sad, joyless, and miserable. Some infants also appear frightened and watchful, with a radar-like gaze. Nevertheless, they may exhibit delayed responsiveness to a stimulus that would elicit fright or withdrawal from a normal infant. Infants with failure to thrive and reactive attachment disorder appear significantly malnourished, and many have protruding abdomens. Occasionally, foul-smelling, celiaclike stools are reported. In unusually severe cases, a clinical picture of marasmus appears. The infant’s weight is often below the third percentile and markedly below the appropriate weight for his or her height. If serial weights are available, the weight percentiles may have decreased progressively because of an actual weight loss or a failure to gain weight as height increases. Head circumference is usually normal for the infant’s age. Muscle tone may be poor. The skin may be colder and paler or more mottled than skin of a normal child. Laboratory findings may indicate coincident malnutrition, dehydration, or concurrent illness. Bone age is usually retarded. Growth hormone levels are usually normal or elevated, a finding suggesting that growth failure in these children is secondary to caloric deprivation and malnutrition. Cortisol secretion in children with reactive attachment disorder or disinhibited social engagement disorder is lower than in typical developing children. For children with failure to thrive, improvement physically and weight gain generally occur rapidly after they are hospitalized. Socially, the infants with reactive attachment disorder usually show little spontaneous activity and a marked diminution of both initiative toward others and reciprocity in response to the caregiving adult or examiner. Both mother and infant may be indifferent to separation on hospitalization or to termination of subsequent hospital visits. The infants frequently show none of the normal upset, fretting, or protest about hospitalization. Older infants usually show little interest in their environment. They may not play with toys, even if encouraged; however, they rapidly or gradually take an interest in, and relate to, their caregivers in the hospital. Psychosocial δwarfism. Classic psychosocial dwarfism or psychosocially determined short stature is a syndrome that usually is first manifest in children 2 to 3 years of age. The children are typically unusually short and have frequent growth hormone abnormalities and severe behavioral disturbances. All of these symptoms result from an inimical caregiver–child relationship. The affectionless character may appear when there is a failure, or lack of opportunity, to form attachments before the age of 2 to 3 years. Children cannot form lasting relationships, and their inability is sometimes accompanied by an inability to obey rules, a lack of guilt, and a need for attention and affection. Children with disinhibited social engagement disorder appear to be overly friendly and familiar with little fear. A 7-year-old boy was referred by his adoptive parents because of hyperactivity and inappropriate social behavior at school. He had been adopted at 4 years of age, after living most of his life in a Chinese orphanage in which he received care from a rotating shift of caregivers. Although he had been below the 5th percentile for height and weight on arrival, he quickly approached the 15th percentile in his new home. However, his adoptive parents were frustrated by his inability to bond with them. They had initially worried about an intellectual problem, although testing and his capacity to engage almost any adult and many children verbally suggested otherwise. He appeared to be too friendly, talking to anyone and often following strangers willingly. He showed little empathy when others were hurt and yet he would sit on the laps of teachers and students without asking. He was frequently injured because of seemingly reckless behavior, although he had an extremely high tolerance for pain. His parents focused on problem behaviors at home to decrease his impulsive behavior, which improved with much prompting; however, he remained oddly overfriendly at home and in school. The child was diagnosed with disinhibited social engagement disorder. (Adapted from Neil W. Boris, M.D. and Charles H. Zeanah, Jr., M.D.) PATHOLOGY AND LABORATORY EXAMINATION Although no single specific laboratory test is used to make a diagnosis, many children with reactive attachment disorder have disturbances of growth and development. Thus, establishing a growth curve and examining the progression of developmental milestones may be helpful in determining whether associated phenomena, such as failure to thrive, are present. DIFFERENTIAL DIAGNOSIS The differential diagnosis of reactive attachment disorder and disinhibited social engagement disorder must take into account that many other psychiatric disorders may arise in conjunction with maltreatment, including depressive disorders, anxiety disorders, and posttraumatic stress disorders. Psychiatric disorders to consider in the differential diagnosis include language disorders, autism spectrum disorder, intellectual disability, and metabolic syndromes. Children with autism spectrum disorders are typically well nourished and of age-appropriate size and weight, and are generally alert and active, despite their impairments in reciprocal social interactions. Significant intellectual disability is often present in children with autism spectrum disorder, whereas when intellectual disability occurs with reactive attachment disorder or disinhibited social engagement disorder, it is generally relatively mild. Children with disinhibited social engagement disorder often show comorbid attentiondeficit/hyperactivity disorder, posttraumatic stress disorder, and language disorder or delay. Furthermore, children with disinhibited social engagement disorder symptoms may have complex neuropsychiatric problems. COURSE AND PROGNOSIS Most of the data available on the natural course of children with reactive attachment disorder and disinhibited social engagement disorder come from follow-up studies of children in residential facilities with histories of serious neglect. Findings from these studies suggest that children with reactive attachment disorder, who are later adopted into caring environments, improve in their attachment behaviors and may normalize over time. Children with disinhibited social engagement disorder, however, appear to have more difficulty developing attachments to new caregivers. Children with disinhibited social engagement disorder who exhibit indiscriminate social behavior also tend to have poor peer relationships. The prognosis for children with reactive attachment disorder and disinhibited social engagement disorder is influenced by the duration and severity of the neglect and the degree of impairment that results. Constitutional and nutritional factors interact in children, who may either respond resiliently to treatment or continue to fail to thrive. After a pathological caregiving situation has been recognized, the amount of treatment and rehabilitation that the family receives affects the child. Children who have multiple problems stemming from pathogenic caregiving may recover physically faster and more completely than they do emotionally. TREATMENT The first consideration in treating reactive attachment disorder or disinhibited social engagement disorder is a child’s safety. Thus, the management of these disorders must begin with a comprehensive assessment of the current level of safety and adequate caregiving. When there is suspicion of maltreatment persisting in the home, the first decision is often whether to hospitalize the child or to attempt treatment while the child remains in the home. If neglect, or emotional, physical, or sexual abuse is suspected, legally, such must be reported to the appropriate law enforcement and child protective services in the area. The child’s physical and emotional state and the level of pathological caregiving determine the therapeutic strategy. A determination must be made regarding the nutritional status of the child and the presence of ongoing physical abuse or threat. Hospitalization is necessary for children with malnourishment. Along with an assessment of the child’s physical well-being, an evaluation of the child’s emotional condition is important. Immediate intervention must address the parents’ awareness and capacity to participate in altering the injurious patterns that have heretofore ensued. The treatment team must begin to improve the unsatisfactory relationship between caregiver and child. This usually requires extensive and intensive intervention and education with the mother or with both parents when possible. In one study, parents of 120 children between 11.7 months and 31.9 months, identified as being at risk for neglect, were randomly assigned to an intervention for atrisk parents called Attachment and Biobehavioral Catch-up (ABC) or to a control intervention. The ABC intervention was designed to decrease frightening behavior toward the infant by parents, and to increase sensitive and nurturing interactions between parents and infant. The intervention was manualized so that parents were specifically guided in how to provide those interactions with their infants. Children were evaluated after 10 sessions, and the 60 children who received the ABC intervention showed significantly lower rates of disorganized attachment (32%), and higher rates of secure attachment (52%) compared to those who received the control intervention (disorganized attachment 57%; secure attachment 33%). The authors concluded that parental nurturance and sensitivity can be enhanced by a comprehensive and explicit intervention such as the ABC intervention, and significant improvements in attachment behaviors can be measured in young children after 10 sessions. The caregiver–child relationship is the basis of the assessment of reactive attachment disorder and disinhibited social engagement disorder symptoms, and the substrate from which to modify attachment behaviors. Structured observations allow a clinician to determine the range of attachment behaviors established with various family members. The clinician may work closely with the caregiver and the child to facilitate greater sensitivity in their interactions. Three basic psychotherapeutic modalities are helpful in promoting positive bonds between children and caregiver. First, a clinician can target the caregiver to promote positive interaction with a child who does not yet have the repertoire to respond positively. Second, a clinician can work with the child and the caregiver together as a dyad to advocate for practicing appropriate positive reinforcement for each other. Through the use of videotapes, parent–child interactions can then be viewed and modifications can be suggested to increase positive engagement. The third modality for clinical intervention is through individual work with the child. Working with the child and caregiver together is often more effective in producing more emotionally meaningful exchanges than working with parent or child individually. Psychosocial interventions for families in which a child has reactive attachment disorder or disinhibited social engagement disorder include (1) psychosocial support services, including hiring a homemaker, improving the physical condition of the apartment, or obtaining more adequate housing; improving the family’s financial status; and decreasing the family’s isolation; (2) psychotherapeutic interventions, including individual psychotherapy, psychotropic medications, and family or marital therapy; (3) educational counseling services, including mother–infant or mother–toddler groups, and counseling to increase awareness and understanding of the child’s needs and to develop parenting skills; and (4) provisions for close monitoring of the progression of the patient’s emotional and physical well-being. Sometimes, separating a child from the stressful home environment temporarily, as in hospitalization, allows the child to break out of the accustomed pattern. A neutral setting, such as the hospital, is the best place to start with families who are genuinely available emotionally and physically for intervention. If interventions are unfeasible or inadequate or if they fail, placement with relatives or in foster care, adoption, or a group home or residential treatment facility must be considered. REFERENCES Bernard K, Dozier M, Carlson E, Bick J, Lewis-Morrarty, Lindheim O. Enhancing attachment organization among maltreated children: Results of a randomized clinical trial. Child Dev. 2012;83:623–636. Boris NW, Zeanah CH. Reactive attachment disorder of infancy, childhood and adolescence. In: BJ Sadock, VA Sadock, Ruiz P, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Vol. II. Philadelphia: Lippincott Williams & Wilkins; 2009:3636. Chaffin M, Hanson R, Saunders BE, Nichols T, Barnett D, Zeanah C, Berliner L, Egeland B, Newman E, Lyon T, LeTourneau E, Miller-Perrin C. Report of the APSAC task force on attachment therapy, reactive attachment disorder, and attachment problems. Child Maltreat. 2006;11:76. Heller SS, Boris NW, Fuselier SH, Pate T, Koren-Karie N, Miron D. Reactive attachment disorder in maltreated twins follow-up: From 18 months to 8 years. Attach Hum Dev. 2006;8:63. Kay C, Green J. Reactive attachment disorder following maltreatment: Systematic evidence beyond the institution. J Abnorm Child Psychol. 2013;41:571–581. Kocovska E, Puckering C, Follan M, Smillie M, Gorski C. Neurodevelopmental problems in maltreated children referred with indiscriminate friendliness. Res Dev Disabil. 2012;33:1560–1565. 27 - 31.11b Posttraumatic Stress Disorder of Infan 31.11b Posttraumatic Stress Disorder of Infancy, Childhood, and Adolescence Kocovska E., Wilson P, Young D, Wallace AM, Gorski C. Cortisol secretion in children with symptoms of reactive attachment disorder. Psychiatr Res. 2013;209:74–77. Minnis H, Macmillan S, Pritchett R, Young D, Wallace B. Prevalence of reactive attachment disorder in a deprived population. Br J Psychiatry. 2013;202:342–346. O’Connor TG, Marvin RS, Rutter M, Olrick J, Britner PA. The ERA Study Team. Child–parent attachment following early institutional deprivation. Dev Psychopathol. 2003;15:19–38. O’Connor TG, Zeanah CH. Attachment disorders: Assessment strategies and treatment approaches. Attach Hum Dev. 2003;5:223–244. Practice parameter for the assessment and treatment of children and adolescents with reactive attachment disorder of infancy and early childhood. J Am Acad Child Adolesc Psychiatry. 2005;44:1206. Pritchett R, Pritchett J, Marshall E, Davidson C, Minnis H. Reactive attachment disorder in the general population: A hidden ESSENCE disorder. Sci World J. 2013;2013:818157. Task Force on Research Diagnostic Criteria: Infancy and preschool: Research diagnostic criteria for infants and preschool children. J Am Acad Child Adolesc Psychiatry. 2003;42:1504. Zeanah CH, Scheeringa MS, Boris NW, Heller SS, Smyke AT, Trapani J. Reactive attachment disorder in maltreated toddlers. Child Abuse Negl. 2004;28:877. Zeanah CH, Smyke T, Dumitrescu A. Attachment disturbances in young children II: Indiscriminate behavior and institutional care. J Am Acad Child Adolesc Psychiatry. 2002;41:983. Zilberstein K. Clarifying core characteristics of attachment disorders: A review of current research and theory. Am J Orthopsychiatry. 2006;76:55. 31.11b Posttraumatic Stress Disorder of Infancy, Childhood, and Adolescence Posttraumatic stress disorder (PTSD), formerly grouped with anxiety disorders, currently falls under a new chapter in the Fifth Edition of the American Psychiatric Association’s Diagnostic and Statistical Manual of Mental Disorders (DSM-5) called trauma- and stressorrelated disorders, a group comprising disorders in which exposure to a traumatic or stressful event is a diagnostic criterion. PTSD is characterized by a set of symptoms including intrusive memories of the trauma, persistent avoidance of stimuli that are reminders of the traumatic event, persistent negative alterations in cognition and mood, and alterations in arousal, mainly seen as hyperarousal and irritability following the traumatic event. In DSM-5, the traumatic event criterion is defined as exposure to actual or threatened death, serious injury, or sexual violence, whether directly, by witnessing it, learning of a traumatic event to a family member, or experiencing repeated exposures to trauma precipitated by social or natural disasters. Exposure to trauma through electronic media, movies, television or photographs is excluded from the criteria. In children 6 years or younger, diagnostic criteria fall under the “preschool subtype,” in which either persistent avoidance of trauma-evoking stimuli or negative alterations in cognitions suffice as indications for PTSD. In the United States, the rates of children and adolescents being exposed to violence and traumatic events are extremely high. In a nationally representative sample of children and adolescents, exposure to a traumatic event was reported to be 60.4 percent, with a lifetime rate ranging from 80 to 90 percent. A significant number of children and adolescents who are exposed to traumatic events, ranging from direct experiences with physical or sexual abuse, domestic violence, motor vehicle accidents, severe medical illnesses, or natural or human-created disasters, will develop PTSD. In children younger than the age of 6 years, spontaneous and intrusive memories may be expressed in play, or occur in frightening dreams; these intrusive thoughts may not be easily identified as related to the traumatic event. Although posttraumatic stress symptoms have been described in adults for more than a century, PTSD was first officially recognized as a psychiatric disorder in 1980 in the DSM, Third Edition (DSM-III). Recognition of the frequency of PTSD in children and adolescents has increased over the last decade. Reports indicate that up to 6 percent of youth are likely to meet full criteria for PTSD at some point in their development. Developmental factors strongly influence the manifestations of symptoms of PTSD. In children and adolescents, reexperiencing of a traumatic event is often observed through play, recurrent nightmares without recall of the traumatic events, and behaviors that reenact the traumatic situation, along with agitation, fear, or disorganization. EPIDEMIOLOGY In the United States, it is estimated that approximately 80 percent of individuals have been exposed to at least one traumatic event; however, less than 10 percent of trauma victims develop posttraumatic stress disorder. The rates of traumatic events, including assaultive violence, exposure to unexpected deaths, being a witness of trauma to others, and bodily injury, all peak sharply between the ages of 16 to 20 years. PTSD is more common in females than in males throughout the life span mainly due to their increased risk for exposure to traumatic events. In situations of natural disaster, the rates of PTSD in males and females are similar. Lifetime risk for PTSD in the United States ranges from 6.8 percent to 12.2 percent. A consistent epidemiologic finding in the United States and in other countries is that PTSD is more prevalent in women than in men. Epidemiological studies of children 9 to 17 years of age have found 3-month prevalence rates of PTSD ranging from 0.5 to 4 percent. An epidemiological survey of preschoolers aged 4 to 5 years found a rate of 1.3 percent of PTSD. Among trauma-exposed samples of persons not referred for treatment, a wide range of 25 percent to 90 percent have been reported to exhibit the full diagnosis of PTSD. Children exposed chronically to trauma, such as child abuse, or traumas resulting in a broader disruption of entire communities, such as war, have the greatest risk of developing PTSD. In addition to the staggering rate of the full-blown disorder of PTSD among youth, several studies indicate that most children exposed to severe or chronic trauma develop PTSD symptoms sufficiently severe to disrupt functioning, even in the absence of the full diagnosis. ETIOLOGY Biological Factors Risk factors in children for developing PTSD include preexisting anxiety disorders and depressive disorders. A prospective study found that among children exposed to traumatic events, those with anxiety disorders and teacher ratings of externalizing behavior problems by the age of 6 years were at increased risk for PTSD. Furthermore, children with an IQ greater than 115 at age 6 years were at lower risk for developing PTSD. In addition, among children exposed to trauma, those who developed PTSD were also at higher risk of developing comorbid disorders such as depression. This suggests that a genetic predisposition for anxiety disorders, as well as a family history indicating increased risk of depressive disorders, may predispose a trauma-exposed child to develop PTSD. Children with PTSD have been found to exhibit increased excretion of adrenergic and dopaminergic metabolites, smaller intracranial volume and corpus callosum, memory deficits, and lower intelligence quotients (IQs) compared with agematched controls. Adults with PTSD have been found to have an overactive amygdala and decreased hippocampal volume. Whether the above findings are sequelae of PTSD or markers of vulnerability to the disorder remains a focus of investigation. Psychological Factors Although the exposure to trauma is the initial etiological factor in the development of PTSD, the enduring symptoms typical of PTSD, such as avoidance of the place where the trauma occurred, can be conceptualized, in part, as the result of both classic and operant conditioning. Extreme physiological responses may accompany fear of a given traumatic event, such as an adolescent who was terrorized by an attack by a group of students near school, who then develops an extreme negative physiological reaction each time he or she is near the school. This is an example of classic conditioning in that a neutral cue (the school) has become paired with an intensely fearful past event. Operant conditioning occurs when a child learns to avoid traumatic reminders to prevent distressing feelings from arising. For example, if a child was in a motor vehicle accident, the child may then refuse to ride in cars altogether to prevent negative physiological reactions and fear from occurring. Another mechanism in developing and maintaining symptoms of PTSD is through modeling, which is a form of learning. For example, when parents and children are exposed to traumatic events, such as natural disasters, children may emulate parental responses, such as avoidance, withdrawal, or extreme expressions of fear, and “learn” to respond to their own memories of the traumatic event in the same manner. Social Factors Family support and reactions to traumatic events in children may play a significant role in the development of PTSD, in that adverse parental emotional reactions to a child’s abuse may increase that child’s risk of developing PTSD. Lack of parental support and psychopathology among parents—especially maternal depression—have been identified as risk factors in the development of PTSD after a child has been exposed to a traumatic event. DIAGNOSIS AND CLINICAL FEATURES For PTSD to ensue, exposure to a traumatic event consisting of either a direct personal experience or witnessing an event involving the threat of death, serious injury, or serious harm must occur. Most common traumatic exposures for children and adolescents include physical or sexual abuse; domestic, school or community violence; being kidnapped; terrorist attacks; motor vehicle or household accidents; or disasters, such as floods, hurricanes, tornadoes, fires, explosions, or airline crashes. A child with PTSD experiences either intrusive memories of the event, recurrent frightening dreams, dissociative reactions including flashbacks in which the child feels as if the traumatic event is recurring, or intense psychological distress when exposed to reminders of the trauma (Fig. 31.11b-1). FIGURE 31.11b-1 The face of a boy in Pakistan shortly after a 7.6 magnitude earthquake hit South Asia leaving millions homeless. (Courtesy of Samoon Ahmad, M.D.) Symptoms of PTSD include reexperiencing the traumatic event in at least one of the following ways. Children may have intrusive thoughts, memories, or images that spontaneously recur, or body sensations that remind them of the event. In very young children, it is common to observe play that includes elements of the traumatic event, or behaviors, such as sexual behaviors that are not developmentally expected. Children may experience periods during which they either act or feel as though the event is taking place presently; this is a dissociative event usually described by adults as “flashbacks.” Another critical symptom cluster of PTSD is avoidance, which in childhood may be displayed by making active physical efforts to avoid the places, people, or situations that would present traumatic reminders of the event. A third cluster of diagnostic criteria for PTSD is negative alterations in cognition and mood following the trauma. In children 6 years or younger, according to DSM-5, negative alterations in cognitions may take the form of socially withdrawn behavior, reduction of expressing positive emotions, diminished interest in play, and feelings of shame, fear, and confusion. In children older than 6 years of age, these may take the form of an inability to remember parts of a traumatic event, that is, psychological amnesia, or persistent negative feelings about oneself, including horror, anger, guilt or shame. After a traumatic event, children may experience a sense of detachment from their usual play activities (“psychological numbing”) or a diminished capacity to feel emotions. Older adolescents may express a fear that they expect to die young (sense of foreshortened future). Other typical responses to traumatic events include symptoms of hyperarousal that were not present before the traumatic exposure, such as difficulty falling asleep or staying asleep; hypervigilance regarding safety and increased checking that doors are locked; or exaggerated startle reaction. In some children, hyperarousal can present as a generalized inability to relax with increased irritability, outbursts, and impaired ability to concentrate. To meet the diagnostic criteria for PTSD, according to the DSM-5 the symptoms must be present for at least 1 month, and cause distress and impairment in important functional areas of life. When all of the diagnostic symptoms of PTSD are met following the traumatic event, persist for at least 3 days, but resolve within 1 month, acute PTSD is diagnosed. When the full syndrome of PTSD persists beyond 3 months, it is designated as chronic PTSD. In some cases, the PTSD symptoms increase over time, and it is not until more than 6 months have elapsed after the exposure to the trauma that the whole syndrome emerges; in that case, the diagnosis is PTSD, delayed onset. DSM-5 criteria for PTSD are described in Table 11.1-3. It is not uncommon for children and adolescents with PTSD to experience feelings of guilt, especially if they have survived the trauma and others in the situation did not. They may blame themselves for the demise of the others and may go on to develop a comorbid depressive episode. Childhood PTSD is also associated with increased rates of other anxiety disorders, depressive episodes, substance use disorders, and attentional difficulties. DSM-5 includes a specifier With dissociative symptoms, which can present as either Depersonalization, in which there are recurrent experiences of feeling detached, as if outside of one’s own body; or Derealization, in which the world feels unreal, dreamlike, and distant. A final specifier, With delayed expression, indicates that the full diagnostic criteria were not met until 6 months after the traumatic event, although some symptoms may present earlier. PATHOLOGY AND LABORATORY EXAMINATION Although reports indicate some alterations in both neurophysiological and neuroimaging studies of children and adolescents with PTSD, no current laboratory tests can help in making this diagnosis. DIFFERENTIAL DIAGNOSIS A number of overlapping symptoms are seen between childhood PTSD and presentations of childhood anxiety disorders, such as separation anxiety disorder, obsessive-compulsive disorder (OCD) or social phobia, in which recurrent intrusive thoughts or avoidant behaviors occur. Children with depressive disorders often exhibit withdrawal and a sense of isolation from peers as well as guilt about life events over which they realistically have no control. Irritability, poor concentration, sleep disturbance, and decreased interest in usual activities can also be observed in both PTSD and major depressive disorder. Children who have lost a loved one in a traumatic event may go on to experience both PTSD and a major depressive disorder when bereavement persists beyond its expected course. Children with PTSD may also be confused with children who have disruptive behavior disorders, because they often show poor concentration, inattention, and irritability. It is critical to elicit a history of traumatic exposure and evaluate the chronology of the trauma and the onset of the symptoms to make an accurate diagnosis of PTSD. COURSE AND PROGNOSIS For some children and adolescents with milder forms of PTSD, symptoms may persist for one to two years, after which they diminish and attenuate. In more severe circumstances, however, PTSD syndromes persist for many years or decades in children and adolescents, with spontaneous remission in only a portion of them. The prognosis of untreated PTSD has become an issue of growing concern for researchers and clinicians who have documented a variety of serious comorbidities and psychobiological abnormalities associated with PTSD. In one study, children and adolescents with severe PTSD were at risk for decreased intracranial volume, diminished corpus callosum area, and lower IQs compared to children without PTSD. Children and adolescents with histories of physical and sexual abuse have been found to exhibit higher rates of depression and suicidality themselves and in their offspring as well. This highlights the importance of early recognition and treatment of PTSD that may significantly improve the long-term outcome among youth. TREATMENT Trauma-Focused Cognitive-Behavior Therapy Randomized clinical trials have provided evidence for the efficacy of trauma-focused cognitive-behavior therapy (CBT) in the treatment of PTSD in children and adolescents. This treatment is generally administered over 10 to 16 treatment sessions, including nine components itemized in the acronym PRACTICE. Trauma-focused CBT as detailed by Cohen, Mannarino, and Deblinger in their text Treating Trauma and Traumatic Grief in Children and Adolescents entails the inclusion of gradual exposure to feared stimuli as a critical element. Such stimuli encompass places, people, sounds, and situations. The first component of trauma-focused CBT is psychoeducation regarding the nature of typical emotional and physiological reactions to traumatic events and PTSD. Next, Parenting Skills involve sessions focused on guiding parents on providing praise, administering a time out, contingency reinforcement programs, and troubleshooting for specific symptoms in a given child. Component 3 is Relaxation, in which children are taught to utilize muscle relaxation, focused breathing, affective modulation, thought-stopping, and other cognitive techniques to diminish feelings of helplessness and distress. Component 4 is Affective Expression and Modulation, geared to help children and their parents to identify their feelings, interrupt disturbing thoughts with positive imagery, and teach positive self-talk and social skills building. Component 5 is Cognitive coping and processing, which deals specifically with reviewing the Cognitive Triangle, in which the relationship between thoughts, feelings, and behaviors is explored. Unhelpful thoughts are challenged with practice. In Component 6, Trauma narrative, the story of the traumatic event and its sequelae are developed over time by the child, with the therapist’s support, using a depiction of words, art, or other creative form. Eventually this is shared with the parent. Component 7, In Vivo Exposure and Mastery of Trauma Reminders, is a session that reviews with the child how to deal with situations that are a reminder of the trauma and how to maintain control over distressing feelings associated with it. Component 8 is Conjoint Child-Parent Sessions; this component may involve several sessions in which the child and parent share their understanding of the process of the therapy and the gains that they have made. Finally, Component 10, Enhancing future safety, involves sessions that focus on the changes made in the family to ensure the safety of the child. These final sessions also promote healthy communication between the child and the parents. A variant of trauma-focused CBT for PTSD is called eye movement desensitization and reprocessing (EMDR), in which an exposure and cognitive reprocessing interventions are paired with directed eye movements. This technique is not as well accepted as the more extensive trauma-focused CBT detailed above. Cognitive Behavioral Intervention for Trauma in Schools (CBITS) CBITS is an intervention that administers treatment in the school setting for children who screen positive for PTSD and whose parents agree to treatment in school. It consists of ten weekly group sessions, one to three individual imaginal exposure sessions, two to four optional sessions with parents, and one parent education session. Similar to trauma-focused CBT (TF-CBT), CBITS incorporates psychoeducation, relaxation training, cognitive coping skills, gradual exposure to traumatic memories through a narrative, in vivo exposure, and affect modulation, cognitive restructuring, and social problem solving. In one randomized controlled trial, 86% of students in the CBITS group reported significantly decreased PTSD symptoms compared to the waitlist controls. Students who received CBITS also reported lower depression scores. Among parents whose children received CBITS treatment, 78% reported decreased psychosocial problems in their children. After CBITS treatment, the improvements in both the PTSD and depression symptoms were sustained at 6 months. Structured Psychotherapy for Adolescents Responding to Chronic Stress (SPARCS) SPARCS consists of a group intervention, generally administered in 16 sessions, with a focus on the needs of adolescents between the ages of 12 and 19 years who have lived with chronic trauma and may also carry a diagnosis of PTSD. SPARCS was tested in a trial of multicultural teens and young adults with moderate or severe trauma exposure. Most of the participants were female, and comprised multiple ethnic groups: 67% African American; 12% Latino; 21% Caucasian. SPARCS demonstrated efficacy in reducing traumatic stress symptoms, mainly in the largest group, the African American group. SPARCS utilizes cognitive behavioral techniques, and also incorporates many of the components of TF-CBT. In addition, SPARCS includes mindfulness techniques and relaxation. Trauma Affect Regulation: Guide for Education and Therapy (TARGET) TARGET, an affect regulation therapy, combines CBT components, such as cognitive processing, with affect modulation. It is administered to adolescents between the ages of 13 and 19 who have been exposed to maltreatment and/or chronic traumatic exposure to such things as community violence or domestic violence. It is generally administered in 12 sessions, which focus on past or current situations. As with SPARCS treatment, gradual exposure may occur in the context of recounting past trauma but is not a core component of the treatment. A randomized trial with 59 delinquent girls aged 13 to 17 years who met full or partial criteria for PTSD found that TARGET reduced anxiety, anger, depression, and PTSD cognitions. TARGET is a promising treatment for girls with histories of delinquency, especially to reduce anger and to enhance optimism and selfefficacy. Crisis Intervention/Psychological Debriefing Crisis intervention/psychological debriefing typically consists of several sessions immediately after an exposure to a traumatic event in which a traumatized child or adolescent is encouraged to describe the traumatic event in the context of a supportive environment. Psychoeducation is provided and guidance about the management of initial emotional reactions may be provided. Anecdotal reports suggest that this intervention may be helpful, but no controlled studies have yet provided evidence that this intervention leads to a more positive outcome. Psychopharmacological Treatment Several pharmacologic agents have been utilized to treat children and adolescents with PTSD, often focused on diminishing intrusive thoughts, hyperarousal, and avoidance, with some success and mixed results. Given the frequent comorbidity of depressive disorder, anxiety disorders, and behavioral problems associated with PTSD, a multitude of psychopharmacological agents have been utilized to ameliorate symptoms associated with PTSD in youth. Antidepressant agents have been used as adjuncts to psychosocial treatments in youth with PTSD. Despite the fact that sertraline and paroxetine are approved by the Food and Drug Administration (FDA) in the treatment of PTSD in adults, there is scant evidence to support its use for the core symptoms of PTSD in youth. A randomized controlled trial of TF-CBT plus sertraline compared to TF-CBT plus placebo in 24 children with PTSD found that both groups had significant reduction in PTSD symptoms, with no significant difference between the groups. A multicenter study of 131 children aged 6 to 17 years with PTSD were treated with 10 weeks of sertraline or placebo. Results showed sertraline to be a safe treatment; however, it was not demonstrated to have efficacy compared to placebo. A randomized controlled trial using citalopram did not show superiority of citalopram over placebo in treatment of core PTSD symptoms. There is, however, evidence suggesting that the use of selective serotonin reuptake inhibitors (SSRIs) in traumatized children with burns may be preventive regarding the development of PTSD. Published literature demonstrates that up to 50 percent of children with moderate to severe burns develop PTSD, thus preventive strategies are important. A randomized controlled study of sertraline to prevent PTSD found that children who received sertraline, flexibly dosed between 25 mg and 150 mg per day, had a decrease in parent-reported symptoms of PTSD over 8 weeks compared to a placebo group. Among the child-reported symptoms, however, there was no significant difference between the two groups. Antiadrenergic agents have been tried to treat dysregulation of the noradrenergic system in adults and youth with PTSD. α-2-agonists such as clonidine and guanfacine, for example, have been used to decrease norepinephrine release, whereas centrally acting β-antagonists such as propranolol, and α-1-antagonists such as prazosin, are hypothesized to improve hyperarousal and intrusive thoughts through attenuation of norepinephrine postsynaptically. In adults, clonidine (Catapres) and propranolol (Inderal) have been used to treat PTSD, especially nightmares and exaggerated startle response, with evidence of improvement. Although there are some data in adults with PTSD to support the use of these agents, data in youth are limited largely to case reports. There is a suggestion that guanfacine may reduce nightmares in children with PTSD and that clonidine may diminish symptoms of reenactment of traumatic events in children. One report of propranolol treatment in 11 pediatric patients with PTSD from sexual or physical abuse with a mean age of 8.5 years, who exhibited agitation and hyperarousal, indicated some decrease in symptoms in 8 of the 11 children studied. Another open study of transdermal clonidine treatment of preschoolers with PTSD suggests that clonidine may be efficacious in this population in decreasing activation and hyperarousal. An additional open trial of oral clonidine with dosage ranges of 0.05 to 0.1 mg twice daily similarly suggests that this medication may provide some relief for the symptoms of hyperarousal, impulsivity, and agitation in young children with PTSD. Second-generation antipsychotics such as risperidone, olanzapine, quetiapine, ziprasidone, and aripiprazole have been studied in adults with PTSD with mixed results. Risperidone and aripiprazole have both been given FDA approval for use in children and adolescents with aggression, severe behavioral dyscontrol, and severe psychiatric disorders; however, controlled trials have not been done with children with PTSD. A report of three preschool-aged children who exhibited symptoms of acute stress disorder and who had severe thermal burns were reported to improve after being treated with risperidone. Mood-stabilizing agents including divalproex, carbamazepine, topiramate, and gabapentin have been utilized for adults with PTSD with modest improvement. In children and adolescents with PTSD, one open-label trial of carbamazepine and one trial of divalproex have been undertaken. In the carbamazepine trial, all 28 patients were reported to be either asymptomatic or improved at blood levels of the agent of 10 to 11.5 micrograms/ml. In the divalproex trial, 12 males who carried diagnoses of conduct disorder comorbid with PTSD were randomly assigned to high- or low-dose divalproex with reported improvement in those receiving the higher doses. Benzodiazepines are often prescribed to treat anxiety symptoms in patients with PTSD, although there are no controlled trials to support their use in youth with PTSD at this time. Given that many children and adolescents with PTSD have comorbid depressive and anxiety disorders, SSRIs are recommended in the treatment of these coexisting disorders. REFERENCES Breslau N. The epidemiology of trauma, PTSD, and other posttraumatic disorders. Trauma Violence Abuse. 2009;10:198– 210. Cohen JA. Posttraumatic stress disorder in children and adolescents. In: Sadock BJ, Sadock VA, Ruiz P, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Vol 2. Philadelphia: Lippincott Williams and Wilkins; 2009:3678. Cohen JA, Mannarino AP, Deblinger E. Treating Trauma and Traumatic Grief in Children and Adolescents. New York: The Guilford Press; 2009. Cohen JA, Mannarino AP, Perel JM, Staron V. A pilot randomized controlled trial of combined trauma-focused CBT and sertraline for childhood PTSD symptoms. J Am Acad Child Adolesc Psychiatry. 2007;46:811–819. Davis TE III, May A, Whiting SE. Evidence-based treatments of anxiety and phobia in children and adolescents: Current status and effects on the emotional response. Clin Psychol Rev. 2011;31:592–602. Dorsey S, Briggs EC, Woods BA. Cognitive behavioral treatment for posttraumatic stress disorder in children and adolescents. Child Adolesc Psychiatr Clin N Am. 2011;20:255–269. Finkelhor D, Turner H, Omrod R, J, Hamby SL. Violence, abuse, and crime exposure in a national sample of children and youth. Pediatrics. 2009;124:1–13. Finkelhor D, Ormrod RK, Turner HA. The developmental epidemiology of childhood victimization. J Interpers Violence. 2009;24:711–731. Ford JD, Steinberg KL, Hawke J, Levine J, Xhang W. Randomized trial comparison of emotion regulation and relational psychotherapies for PTSD in girls involved in delinquency. J Clin Child Adolesc Psychol. 2012;41:27–37. Huemer J, Erhart F, Steiner H. Posttraumatic stress disorder in children and adolescents: A review of psychopharmacological treatment. Child Psychiatry Hum Dev. 2010;41:624–640. Jaycox LH, Cohen JA, Mannarino AP, Walker DW, Langley AK, Gegenheimer KL, Children’s mental health care following Hurricane Katrina: A field trial of trauma-focused psychotherapies. J Traum Stress. 2010;23:223–231. Jaycox, LH, Langley AK, Dean KL. Support for students exposed to trauma: The SSET program: group leader training 28 - 31.12 Mood Disorders and Suicide in Children 31.12 Mood Disorders and Suicide in Children and Adolescents 29 - 31.12a Depressive Disorders and Suicide in Ch 31.12a Depressive Disorders and Suicide in Children and Adolescents manual, lesson plans and lesson materials and worksheets. Santa Monica, CA: RAND Health. 2009. Meighen KG, Hines LA, Lagges AM. Risperidone treatment of preschool children with thermal burns and acute stress disorder. J Child Adolesc Psychopharmacol. 2007;17:223–232. Robb AS, Cueva JE, Sporn J, Vanderberg DG. Sertraline treatment of children and adolescents with posttraumatic stress disorder: A double-blind placebo-controlled trial. J Child Adolesc Psychopharmacol. 2010;20:463–471. Rynn M, Puliafico A, Heleniak C, Rikhi P, Ghalib K, Vidair H. Advances in pharmacotherapy for pediatric anxiety disorders. Depress Anxiety. 2011;28:76–87. 31.12 Mood Disorders and Suicide in Children and Adolescents 31.12a Depressive Disorders and Suicide in Children and Adolescents Depressive disorders in youth represent a significant public health concern, in that they are prevalent and result in long-term adverse effects on the individual’s cognitive, social, and psychological development. These disorders affect approximately 2 to 3 percent of children and up to 8 percent of adolescents, so the need for early identification and access to evidence-based interventions such as cognitive-behavioral therapies (CBTs) and antidepressant agents, is essential. Although major depression runs in families, with the highest risk in children whose parents experienced early onset depression, twin studies have demonstrated that major depression is only moderately heritable, approximately 40 to 50%, highlighting environmental stressors and adverse events as major contributors to major depressive disorder in youth. The core features of major depression in children, adolescents, and adults bear a striking resemblance; however, clinical presentation is strongly influenced by the developmental level of the child or adolescent. The American Psychiatric Association’s Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5) utilizes the same criteria for major depressive disorder in youth as in adults, except that for children and adolescents, irritable mood may replace a depressed mood in the diagnostic criteria. Most children and adolescents with depressive disorders neither attempt nor complete suicide; however, severely depressed youth often have suicidal ideation, and suicide remains the most serious risk of major depression. Nevertheless, many depressed youth do not ever have suicidal ideation, and many children and adolescents who engage in suicidal behavior do not have a depressive disorder. There is epidemiological evidence to suggest that depressed youth with recurrent active suicidal ideation, including a plan, and who have made prior attempts, are at higher risk to complete suicide, compared to youth who express only passive suicidal ideation. Mood disorders in children and adolescents have been studied increasingly over the last two decades, culminating in large sample multisite randomized controlled trials such as the Treatment of Adolescent Depression (TADS) study, which provides evidence of the efficacy of both cognitive-behavioral therapy as well as selective serotonin reuptake inhibitors (SSRIs). Furthermore, when the preceding modalities are combined, the greatest efficacy is achieved. Increased recognition of depressive disorders in preschool populations has sparked clinicians and researchers to develop psychosocial interventions such as the Parent-Child Interaction Therapy Emotion Development (PCITED), which target treatment specifically for this age group. The expression of disturbed and depressed mood appears to vary with developmental stage. Very young children with major depression are often observed to be sad, listless, or apathetic, even though they may not articulate these feelings verbally. Perhaps surprisingly, mood-congruent auditory hallucinations are not infrequently observed in young children with major depression. Somatic complaints such as headaches and stomachaches, withdrawn and sad appearance, and poor self-esteem are more universal symptoms. Patients in late adolescence with more severe forms of depression often display pervasive anhedonia, severe psychomotor retardation, delusions, and a sense of hopelessness. Symptoms that appear with the same frequency, regardless of age and developmental status, include suicidal ideation, depressed or irritable mood, insomnia, and diminished ability to concentrate. Developmental issues, however, influence the expression of depressive symptoms. For example, unhappy young children who exhibit recurrent suicidal ideation are rarely able to propose a realistic suicide plan or to carry out such a plan. Children’s moods are especially vulnerable to the influences of severe social stressors, such as chronic family discord, abuse and neglect, and academic failure. Many young children with major depressive disorder have histories of abuse, neglect, and families with significant psychosocial burdens such as parental mental illness, substance abuse, or poverty. Children who develop depressive disorders in the midst of acute toxic family stressors may have remission of depressive symptoms when the stressors diminish or when a more nurturing family environment is introduced. Depressive disorders are generally episodic, albeit typically lasting close to a year; however, their onset may be insidious and remain unidentified until significant impairment in peer relationships, deterioration in academic function, or withdrawal from activities emerges. Attentiondeficit/hyperactivity disorder (ADHD), oppositional defiant disorder, and conduct disorder are not infrequently comorbid with a major depressive episode. In some cases, conduct disturbances or disorders occur in the context of a major depressive episode and resolve with the resolution of the depressive episode. Clinicians must clarify the chronology of the symptoms to determine whether a given behavior (e.g., poor concentration, defiance, or temper tantrums) was present before the depressive episode and is unrelated to it or whether the behavior is occurring for the first time and is related to the depressive episode. EPIDEMIOLOGY Depressive disorders increase in frequency with increasing age in the general population. Mood disorders among preschool-age children are estimated to occur in about 0.3 percent of community samples, and 0.9 percent in clinic settings. The prevalence of major depression in school age children is 2 to 3 percent. Depression in referred samples of school-age children is found to be the same frequency in boys as in girls, with some surveys indicating a slightly increased rate among boys. In adolescents, prevalence rate of major depression is from 4 to 8 percent and two to three times more likely in females than males. By age 18 years, the cumulative incidence of major depression is 20 percent. Children with a family history of major depression in a firstdegree relative are about three times more likely to develop the disorder than in those without family histories of affective disorders. The prevalence of persistent depressive disorder in children ranges from 0.6 to 4.6 percent and in adolescence increases to 1.6 to 8 percent. Children and adolescents with persistent depressive disorder have a high likelihood of developing major depressive disorder at some point after 1 year of the persistent depressive disorder. The rate of developing a major depression on top of persistent depressive disorder (double depression) within a 6-month period of persistent depressive disorder is estimated to be about 9.9 percent. Among psychiatrically hospitalized children and adolescents, the rates of major depressive disorder have been estimated to be close to 20 percent for children and 40 percent for adolescents. ETIOLOGY Considerable evidence indicates that major depression in youth is the same fundamental disorder experienced by adults, and that its neurobiology is likely to be an interaction of genetic vulnerability and environmental stressors. Genetic Studies Converging evidence suggests that an interaction between genetic susceptibility and environmental stressors contributes to an emerging major depression and is associated with brain volume, especially in the hippocampal region. The serotonin transporter gene and, in particular, the serotonin transporter promoter polymorphism (5-HTTLPR) have become a focus of investigation. Patients with the short S-allele of the serotonin polymorphism who also experienced a significant environmental adverse event such as neglect, developed smaller hippocampal volumes compared to patients with only one of the above risk factors. The S-allele of the polymorphism leads to decreased serotonin (5HT) reuptake and thus potentially to decreased uptake of serotonin into the brain. A large longitudinal study in New Zealand found that the S-allele of the serotonin transporter gene was associated with early environmental stress and subsequent depression. This study demonstrated a relationship between early environmental stress and subsequent depression in children with one or two short alleles, but not in children with two long alleles. Because the short alleles are less efficient in transcription, this finding suggests that the availability of the transporter gene may provide a marker for vulnerability to depression. The findings that the combination of a decreased volume in the hippocampus is associated with the S-allele of the serotonin transporter gene polymorphism and early adverse events in depression, may represent a mechanism by which the risk of major depression is mediated by both genetics and environmental stressors. Familiality Twin studies have demonstrated that major depression is approximately 40 to 50 percent heritable. There is an increased risk of depression in the children of parents with the disorder, and this risk is further increased for the child when the parents developed depressive disorders at an early age. Studies suggest age-related differences in the heritability of major depression such that in younger children, environmental influences appear to be more dominant and in first episodes in adolescence, heritability may play a larger role. Family studies suggest that for children with a parent with a history of major depressive disorder, the risk of developing an episode of major depressive disorder is doubled, whereas with two depressed parents, the risk of an episode of major depressive disorder quadruples in the offspring before age 18 years. Similarly, children with the largest number of severe episodes starting at younger ages exhibit the densest family histories of major depressive disorder. Neurobiology Neuroendocrine studies have examined the hypothalamic-pituitary-adrenal axis, the hypothalamic growth hormone, the hypothalamic-pituitary-thyroid, and the hypothalamic-pituitary-gonadal axes, seeking to demonstrate consistent markers in depressed youth. These studies have yielded inconsistent results. For example, depressed prepubertal children secrete significantly more growth hormone during sleep than nondepressed children or youth with other psychiatric disorders. In addition, depressed children secrete significantly less growth hormone in response to insulin-induced hypoglycemia than do nondepressed patients. Both findings appear to persist for months after partial or full remission. Thyroid hormone studies have found lower free total thyroxine (FT4) levels in depressed adolescents than in a matched control group. These values were associated with normal thyroid-stimulating hormone (TSH). This finding suggests that, although values of thyroid function remain in the normative range, FT4 levels have shifted downward. These downward shifts in thyroid hormone possibly contribute to the clinical manifestations of depression. Sleep studies are also inconclusive in depressed children and adolescents. Polysomnography in depressed children have only occasionally shown characteristic sleep markers of adults with major depressive disorder: reduced rapid eye movement (REM) latency and an increased number of REM periods. Magnetic Resonance Imaging Neuroimaging studies of depressed youth demonstrate smaller frontal white matter volumes, larger frontal gray matter volumes, and larger lateral ventricle volumes. Depressed youth have been found to have a blunted amygdala response to fearful faces compared to non-depressed children and depressed children have been found to have smaller amygdale volumes compared to healthy controls. Because twin studies and adoption studies have demonstrated that depression appears to be only 40 to 50 percent heritable, with environmental contributions more predominant in younger children, family and environmental contributions must be examined. Adverse events during childhood such as maltreatment, abuse or neglect, parental death, parental psychiatric illness, substance abuse, parent–child conflict, and lack of family cohesion are all risk factors for childhood depression. Data from twin and genetic studies support the conclusion that the interaction of genetic and environmental factors plays a critical role in depressive disorders, since correlation of adverse life events and depression is stronger in children and adolescents with known genetic susceptibility. Once a child or adolescent has experienced one major depressive episode, the psychosocial “scars” increase his or her vulnerability for a subsequent episode. The psychosocial impairments in depressed children remain far after recovery from the episode. Among depressed preschoolers, the sooner that adverse life events promoting the depression are identified, the more rapidly interventions may be administered to treat the depression. DIAGNOSIS AND CLINICAL FEATURES Major Depressive Disorder Major depressive disorder in children is diagnosed most easily when it is acute and occurs in a child without previous psychiatric symptoms. Often, however, the onset is insidious, and the disorder occurs in a child who has had several years of difficulties with hyperactivity, separation anxiety disorder, or intermittent depressive symptoms. According to the DSM-5, diagnostic criteria for a major depressive episode consist of at least five symptoms, for a period of 2 weeks, including either (1) depressed or irritable mood, or (2) a loss of interest or pleasure. Additional symptoms may include failure to make expected weight gains, daily insomnia or hypersomnia, psychomotor agitation or retardation, daily fatigue or loss of energy, feelings of worthlessness or inappropriate guilt, diminished ability to think or concentrate, and recurrent thoughts of death. These symptoms must produce social or academic impairment. To meet the diagnostic criteria for major depressive disorder, the symptoms cannot be due to the direct effects of a substance (e.g., alcohol) or a general medical condition. In contrast to the Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition, Text Revision (DSM-IV-TR), in which a diagnosis of major depressive disorder was not made within 2 months of the loss of a loved one, except when marked functional impairment, morbid preoccupation with worthlessness, suicidal ideation, psychotic symptoms, or psychomotor retardation was present, in DSM-5, a diagnosis of major depressive disorder can be made at any time following a loss, even without the preceding symptoms. This change reflects the understanding that grief typically lasts 1 to 2 years, rather than 2 months, and that major depressive disorder may occur in the presence of grief at any time after a loss. A major depressive episode in a prepubertal child is likely to be manifest by somatic complaints, psychomotor agitation, and mood-congruent hallucinations. Anhedonia is also frequent, but anhedonia, as well as hopelessness, psychomotor retardation, and delusions, are more common in adolescent and adult major depressive episodes than in those of young children. Adults have more problems than depressed children and adolescents with sleep and appetite. In adolescence, negativistic or frankly antisocial behavior and the use of alcohol or illicit substances can occur and may justify the additional diagnoses of oppositional defiant disorder, conduct disorder, and substance abuse or dependence. Feelings of restlessness, irritability, aggression, reluctance to cooperate in family ventures, withdrawal from social activities, and isolation from peers often occur in adolescents. School difficulties are likely. Depressed adolescents may become less attentive to personal appearance and show increased sensitivity to rejection by peers, and in romantic relationships. Children can be reliable reporters about their emotions, relationships, and difficulties in psychosocial functions. They may, however, refer to depressive feelings in terms of anger, or feeling “mad” rather than sad. Clinicians should assess the duration and periodicity of the depressive mood to differentiate relatively universal, short-lived, and sometimes frequent periods of sadness, usually after a frustrating event, from a true, persistent depressive mood. The younger the child, the more imprecise his or her time estimates are likely to be. Mood disorders tend to be chronic if they begin early. Childhood onset may be the most severe form of mood disorder and tends to appear in families with a high incidence of mood disorders and alcohol abuse. The children are likely to have such secondary complications as conduct disorder, alcohol and other substance abuse, and antisocial behavior. Functional impairment associated with a depressive disorder in childhood extends to practically all areas of a child’s psychosocial world; school performance and behavior, peer relationships, and family relationships all suffer. Only highly intelligent and academically oriented children with no more than a moderate depression can compensate for their difficulties in learning by substantially increasing their time and effort. Otherwise, school performance is invariably affected by a combination of difficulty concentrating, slowed thinking, lack of interest and motivation, fatigue, sleepiness, depressive ruminations, and preoccupations. Depression in a child may be misdiagnosed as a learning disorder. Learning problems secondary to depression, even when long-standing, are corrected rapidly after a child’s recovery from the depressive episode. Children and adolescents with severe forms of major depressive disorder may have hallucinations and/or delusions. Usually, these psychotic symptoms are thematically consistent with the depressed mood, occur with the depressive episode (usually at its worst), and do not include certain types of hallucinations (such as conversing voices and a commenting voice, which are specific to schizophrenia). Depressive hallucinations usually consist of a single voice speaking to the person from outside his or her head, with derogatory or suicidal content. Depressive delusions center on themes of guilt, physical disease, death, nihilism, deserved punishment, personal inadequacy, and (sometimes) persecution. These delusions are rare in prepuberty, probably because of cognitive immaturity, but are present in about half of psychotically depressed adolescents. Adolescent onset of a mood disorder can be complicated by use of alcohol or drugs. One study found that up to 17 percent of adolescents with depressive disorder received an initial evaluation due to substance abuse. Persistent Depressive Disorder (Dysthymia) Persistent depressive disorder, in DSM-5, represents a consolidation of chronic major depressive disorder and what DSM-IV-TR termed dysthymic disorder. In children and adolescents it consists of a depressed or irritable mood for most of the day, for more days than not, over a period of at least 1 year. DSM-5 notes that in children and adolescents, irritable mood can replace the depressed mood criterion for adults and that the duration criterion is not 2 years but 1 year for children and adolescents. According to the DSM-5 diagnostic criteria, two or more of the following symptoms must accompany the depressed or irritable mood: low self-esteem, hopelessness, poor appetite or overeating, insomnia or hypersomnia, low energy or fatigue, or poor concentration or difficulty making decisions. During the year of the disturbance, these symptoms do not resolve for more than 2 months at a time. In addition, the diagnostic criteria for dysthymic disorder specify that during the first year, no major depressive episode emerges. To meet the DSM-5 diagnostic criteria for persistent depressive disorder, a child must not have a history of a manic or hypomanic episode. Persistent depressive disorder is also not diagnosed if the symptoms occur exclusively during a chronic psychotic disorder or if they are the direct effects of a substance or a general medical condition. DSM-5 provides specifiers for early onset (before 21 years of age) or late onset (after 21 years of age). A child or adolescent with persistent depressive disorder may have had a major depressive episode before developing persistent depressive disorder; however, it is much more common for a child with persistent depressive disorder for more than 1 year to develop a concurrent episode of major depressive disorder. In this case, both depressive diagnoses apply (double depression). Persistent depressive disorder in youth is known to have an average age of onset that is several years earlier than the typical onset of major depressive disorder. Occasionally, youth fulfill the criteria for persistent depressive disorder, except that their episode does not last for a whole year, or they experience remission from symptoms for more than a 2-month period. These mood presentations in youth may predict additional mood disorder episodes in the future. Current knowledge suggests that the longer, more recurrent, and less directly related to social stress these episodes are, the greater the likelihood of future severe mood disorder. When minor depressive episodes follow a significant stressful life event by less than 3 months, it may be classified as an adjustment disorder. Cyclothymic Disorder Cyclothymia is a chronic and fluctuating mood disturbance of hypomanic symptoms and periods of depressive symptoms that do not meet diagnostic criteria for major depressive disorder. The difference in the DSM-5 diagnostic criteria for youth with cyclothymic disorder compared to adults is that a period of 1 year, rather than 2 years, of numerous mood swings is applied. Bipolar II disorder is distinguished from cyclothymia by a history of episodes of major depressive disorder. When an episode of major depressive disorder occurs after a diagnosis of cyclothymia has been present for at least 2 years, a concurrent diagnosis of Bipolar II disorder is made. Bereavement Bereavement is a state of grief related to the death of a loved one, which presents with an overlap of symptoms characteristic of a major depressive episode. Typical depressive symptoms associated with bereavement include feelings of sadness, insomnia, diminished appetite, and, in some cases, weight loss. Grieving children may become withdrawn and appear sad, and they are not easily drawn into even favorite activities. In DSM-5, bereavement is not a mental disorder; however, uncomplicated bereavement is included as a category documented with a v code, indicating that a normal grief reaction to the loss of a loved one has become a focus of clinical attention. Children in the midst of a typical bereavement period may also meet the criteria for major depressive disorder. Symptoms indicating major depressive disorder exceeding typical bereavement include intense guilt related to issues beyond those surrounding the death of the loved one, preoccupation with death other than thoughts about being dead to be with the deceased person, morbid preoccupation with worthlessness, marked psychomotor retardation, prolonged serious functional impairment, and hallucinations other than transient perceptions of the voice of the deceased person. The duration of bereavement varies; in children, the duration may depend partly on the support system in place. For example, a child who must be removed from home because of the death of the only parent in the home may feel devastated and abandoned for a long period. Children who lose loved ones may feel a sense of guilt, that the death may have occurred because they were “bad” or did not perform as expected. Ryan was a 12-year-old 7th grader in middle school who was brought to the emergency room in handcuffs by police after walking into oncoming traffic right after school. Ryan walked in front of a city bus; the driver began honking at the boy who kept walking slowly into the traffic. Two police stationed in their car across the street from the school heard the bus honking and noticed Ryan and confronted him. The police were about to issue the boy a citation for crossing against the red light; however, when they inquired as to why he had crossed against the traffic light he informed them that he was trying to kill himself. The police handcuffed Ryan, placed him in the police car without a struggle and brought him to the local hospital’s emergency room. Ryan’s mother was contacted and met her son in the emergency room. Ryan was found to be physically intact, without injury, by the emergency room doctors, and psychiatric evaluation was initiated by a team of child psychiatrists including an attending child psychiatrist and two child and adolescent psychiatry residents. Ryan became tearful when asked what had happened, and reported that he had purposefully walked in front of the bus in the hope of being hit by the bus in order to die. Ryan reported that he has been bullied by numerous peers over the last 2 years and is picked on because he is short and overweight. Ryan reported that on this day, a girl in his class had pushed him down and started hitting him and laughing at him. Ryan reported that he had been teased and physically assaulted repeatedly by peers in his grade and that they call him stupid and fat. Ryan has some friends, who usually defend him, but on this day, his friends were not close by and he became desperate. Ryan disclosed, however, that even before this day, he has been consistently sad in school for the past year, and that he has thought about suicide recurrently over the last year, mainly due to feeling ostracized and worthless after being picked on and bullied. Ryan continues to be actively suicidal, disclosing his strong feeling that for him, life is not worth living. Ryan is a relatively good student, earning good grades, especially in math, although he is currently failing history. Upon separate interview with Ryan’s mother, she reports that she has no knowledge of any problems that Ryan has been struggling with, and that she feels that Ryan is not depressed, not severely bullied, or seriously unhappy in school, and reports that this must have been a mistake and that she is ready to take him home. Ryan reported that he had previously seen a counselor in school a few times last year when he was bullied, but that he has received no intervention for his depression or his suicidality and that he has not shared these feeling with his family and that he is generally content at home. Ryan has an older brother and a younger brother who are well adjusted. When Ryan and his mother were interviewed together, Ryan was able with some encouragement to let his mother know how depressed, hopeless, and suicidal he feels, and why. Ryan’s mother burst into tears and Ryan tried to comfort his mother, although he was crying as well. Ryan was placed on a 72-hour hold for “danger to self,” and referred to a children’s psychiatric inpatient unit for further evaluation and treatment. A trial of an SSRI antidepressant was recommended as well as psychoeducation and family sessions so that Ryan and his family would reach an understanding about his current psychiatric disorder, and that together they could work on a safe and productive plan for Ryan. Ongoing psychosocial intervention was recommended for Ryan and his family after hospitalization. PATHOLOGY AND LABORATORY EXAMINATION No laboratory test is useful in making a diagnosis of a major depression. If a child or adolescent also complains of symptoms of hypothyroidism, that is, dry skin, coldness, lethargy, and so on, then a screening test for thyroid function may be indicated. Rating scales for depressive symptoms administered by the clinician to the child and parent may be helpful in the evaluation. The Children’s Depression Rating Scale-Revised (CDRS-R) is a 17-item instrument administered by the clinician separately to the parent and child or adolescent. The clinician scores a rating for each item using the information from both the parent and the child. The scale assesses affective, somatic, cognitive, and psychomotor symptoms. A cumulative score of 40 is a marker for moderate depression and a score of 45 or greater for significant depression. DIFFERENTIAL DIAGNOSIS Substance-induced mood disorder may be difficult to differentiate from other mood disorders until detoxification occurs. Anxiety symptoms and disorders often coexist with depressive disorders. Of particular importance in the differential diagnosis is the distinction between agitated depressive or manic episodes and ADHD, in which the persistent excessive activity and restlessness can cause confusion. Prepubertal children generally do not show classic forms of agitated depression, such as hand-wringing and pacing. Instead, an inability to sit still, irritability, and frequent temper tantrums are the most common symptoms. Sometimes, the correct diagnosis becomes evident only after remission of the depressive episode. COURSE AND PROGNOSIS The course and prognosis of major depression in children and adolescents depends on the severity of illness, the rapidity of interventions, and the degree of response to the interventions. In general, 90 percent of youth recover from a first episode of moderate to severe major depressive disorder within 1 to 2 years. The age of onset, episode severity, and the presence of comorbid disorders also influence course and prognosis. In general, the younger the age of onset, the greater the recurrence of multiple episodes, and the presence of comorbid disorders predict a poorer prognosis. The mean length of an untreated episode of major depression in children and adolescents is about 8 to 12 months; the cumulative probability of recurrence is 20 to 60 percent within 2 years and 70 percent by 5 years. The greatest risk for relapse is in the 6 months to 1 year after treatment is discontinued. Depressed children who live in families with high levels of chronic conflict are more likely to have relapses. The relapse rates for childhood major depression into adulthood are also high. In a community sample, 45 percent of adolescents with a history of major depression developed another episode of major depression in early adulthood. Youth with major depression are at higher risk for the development of future bipolar disorder, compared to adults. Overall estimates of children with an episode of major depression developing bipolar disorder are about 20 to 40 percent. Clinical characteristics of a depressive episode in youth, suggesting the highest risk of developing bipolar I disorder include hallucinations and delusions, psychomotor retardation, and a family history of bipolar illness. In a longitudinal study of prepubertal children with major depression, 33 percent developed bipolar I disorder, whereas 48 percent went on to develop bipolar II or bipolar disorder not otherwise specified by early adulthood. Depressive disorders are associated with short-term and long-term peer relationship difficulties and complications, compromised academic achievement, and persistently low self-esteem. Persistent depressive disorder has an even more protracted recovery than major depressive disorder; the mean episode length is about 4 years. Early onset persistent depressive disorder is associated with significant risks of comorbidity with major depressive disorder (70 percent), bipolar disorder (13 percent), and future substance abuse (15 percent). The risk of suicide, which accounts for about 12 percent of adolescent mortalities, is significant among adolescents with depressive disorders. TREATMENT The American Academy of Child and Adolescent Psychiatry practice parameters, as well as a consensus of experts who developed the Texas Children’s Medication Algorithm Project (TMAP) made evidence-based recommendations for the treatment of children and adolescents with depressive disorders. These include psychoeducation and supportive interventions for youth with mild forms of depression. For youth with moderate to severe depression or recurrent episodes of major depression, with significant impairment and with active suicidal thoughts or behaviors, or psychosis, optimal intervention includes both psychopharmacological and CBT. CBT or interpersonal therapy (IPT) alone may be effective for moderate depression, especially when treatment is continued for 6 months or longer. Psychiatric Hospitalization Assessment of suicidal thoughts, behaviors, and past history of suicidal behavior is indicated in evaluating every child or adolescent with major depression. Safety is the most immediate consideration in assessing depression in youth, that is, a determination as to whether immediate psychiatric hospitalization is necessary. Depressed children and adolescents who express suicidal thoughts or behaviors most often require some extended evaluation in the safety of the psychiatric hospital to provide maximal protection from self-destructive impulses and behaviors. EVIDENCE-BASED TREATMENT STUDIES The Treatment for Adolescents with Depression Study (TADS) divided 439 adolescents between 12 years and 17 years of age into three treatment groups of 12 weeks, composed of either fluoxetine (Prozac) alone (10 to 40 mg per day), fluoxetine with the same dose range in combination with CBT, or CBT alone. Based on ratings of the Children’s Depression Rating Scale-Revised (CDRS-R) combination treatment had significantly superior response rates compared with either treatment alone. Based on CGI scores, at 12 weeks, rates of much or very much improved were 71 percent for the combined treatment group, 60.6 percent for the fluoxetine group, and 43.2 percent for the CBT alone group; and 34.4 percent for the placebo group. At 12 weeks, combination treatment was rated the optimal strategy in the treatment of adolescent depression. By the end of 9 months of treatment, however, response rates for each group had converged so that response for the combination group was 86 percent, fluoxetine group response was about 81 percent, and CBT-alone group response rate was 81 percent. The long-term effectiveness of treatments for adolescent depression demonstrates that for moderately ill adolescents, fluoxetine, CBT, or the combination is efficacious. However, the addition of CBT to fluoxetine decreased persistent suicidal ideation and potential treatment-related emergence of suicidal ideation. A second large multicenter randomized placebo controlled trial, Treatment of SSRIResistant Depression in Adolescents (TORDIA), included adolescents with major depression, who had not responded to a 2-month trial with an SSRI antidepressant. In this study, 334 adolescents between 12 and 18 years of age were randomly assigned to a different SSRI agent (either citalopram, paroxetine, citalopram, or another antidepressant class, venlafaxine) with or without concurrent CBT. The SSRI plus CBT group and the venlafaxine plus CBT group had higher response rates of improvement (54.8 percent) than the group on medications alone (40.5 percent). There were no differences found in the response rates between antidepressant agents. Psychosocial Interventions CBT is widely recognized as an efficacious intervention for the treatment of moderately severe depression in children and adolescents. CBT aims to challenge maladaptive beliefs and enhance problem-solving abilities and social competence. A review of controlled cognitive-behavioral studies in children and adolescents revealed that, as with adults, both children and adolescents showed consistent improvement with these methods. Other “active” treatments, including relaxation techniques, were also shown to be helpful as adjunctive treatment for mild to moderate depression. Findings from one large controlled study comparing cognitive-behavioral interventions with nondirective supportive psychotherapy and systemic behavioral family therapy showed that 70 percent of adolescents had some improvement with each of the interventions; cognitivebehavioral intervention had the most rapid effect. Another controlled study comparing a brief course of CBT with relaxation therapy favored the cognitive-behavioral intervention. At a 3- to 6-month follow-up, however, no significant differences existed between the two treatment groups. This effect resulted from relapse in the cognitivebehavioral group, along with continued recovery in some patients in the relaxation group. Factors that seem to interfere with treatment responsiveness include the presence of comorbid anxiety disorder that probably was present before the depressive episode. It has been shown, however, that longer term CBT is efficacious in the treatment of depression, and has the advantage of mitigating suicidal ideation. Interpersonal psychotherapy (IPT) focuses on improving depression through a focus on ways in which depression interferes with interpersonal relationships and overcoming these challenges. The four main areas of focus with interpersonal psychotherapy include loss, interpersonal disputes, role transition, and interpersonal deficits. A modification of interpersonal therapy to more specifically address depression for adolescents (IPT-A) includes a focus on separation from parents, authority figures, peer pressures, and dyadic relationships. IPT-A has been studied on an outpatient basis as well as in a school-based clinic setting. A 12-week study of 48 adolescents with major depression randomly assigned to IPT-A or clinical monitoring found that the group receiving IPT-A showed decreased depressive symptoms, increased social functioning, and improved problem solving compared to the other group. In the school-based health clinic, depressed adolescents were randomly assigned to IPT-A or treatment as usual for a period of 16 weeks. Clinic staff were trained and administered the treatment. At the end of 16 weeks, those adolescents receiving IPT-A had greater symptom reduction and improved overall functioning; especially older and more severely depressed adolescents seemed to benefit most significantly. Parent-Child Interaction Therapy Emotion Development (PCIT-ED) for preschool depression, a modification of Parent-Child Interaction Therapy (PCIT) historically used in the treatment of disruptive disorders for children, was piloted in a randomized controlled trial for 54 depressed preschoolers. Fifty-four depressed young children from age 3 to 7 years were randomly assigned to either PCIT-ED or psych-education with their caregivers. PCIT-ED was manualized and consists of three modules conducted over 14 sessions in 12 weeks. The core modules of PCIT—Child-Directed Interaction (CDI) and Parent-Directed Interaction (PDI)—were utilized and limited to four sessions each. The focus of these modules is to strengthen the parent–child relationship by coaching parents in positive play techniques, giving effective directives to the child, and responding to disruptive behavior in firm but not punitive ways. The novel portion of the treatment targeting the preschool depression consisted of a 6-week Emotion Development (ED) module, which focused on helping the parent to be a more effective emotion guide and affect regulator for the child. As part of the ED module, the parent learned to accurately recognize his or her own emotions as well as the child’s and serves to help regulate the child’s emotions. A psychoeducation control condition, Developmental Education and Parenting Intervention (DEPI) was developed and administered to parents in small group sessions. The DEPI condition was designed to educate parents about child development and emphasized emotional and social development without individual coaching or practice with behavioral techniques as provided in the PCIT-ED group. Primary outcome measures included parent’s report of the child’s symptoms of depression using a structured instrument, the Preschool Age Psychiatric Assessment (PAPA), and depression severity was measured pretreatment and posttreatment using parent ratings on the Preschool Reelings Checklist Scale Version (PFC-S) a 20-item checklist. Results revealed that both groups showed significant improvement with particular improvement in the PCIT-ED group with respect to emotion recognition, child executive functioning, and parenting stress. This pilot study indicates that PCIT-ED is a promising novel intervention for preschool depression that deserves further investigation. Pharmacotherapy Fluoxetine (Prozac) and escitalopram (Lexapro) have Food and Drug Administration (FDA) approval in the treatment of major depression in adolescents. Three randomized controlled trials (RCTs) using fluoxetine with depressed children and adolescents demonstrate its efficacy. Common side effects observed with fluoxetine include headache, gastrointestinal symptoms, sedation, and insomnia. Short-term randomized clinical trials have demonstrated efficacy of citalopram (Celexa), and sertraline (Zoloft) compared with placebo in the treatment of major depression in children and adolescents. Sertraline has been shown to provide efficacy in two multicenter, double-blind, placebo-controlled trials of 376 children and adolescents who were treated with sertraline at doses ranging from 50 mg to 200 mg a day, or placebo. greater than 40 percent decrease in depression rating scale scores were found in nearly 70 percent of the patients treated with sertraline, compared with 56 percent in the placebo group. Most common side effects are anorexia, vomiting, diarrhea, and agitation. Citalopram has been demonstrated in one RCT in the United States to be efficacious in 174 children and adolescents treated with citalopram at doses of 20 to 40 mg a day or placebo for 8 weeks. Significantly more of the group on citalopram showed improvement compared with placebo on the depression rating scale (CDRS-R). A significantly increased response rate (response defined as less than 28 on CDRS-R) of 35 percent was found in the citalopram group, compared with 24 percent of the placebo group. Common side effects that emerged included headache, nausea, insomnia, rhinitis, abdominal pain, dizziness fatigue, and flu-like symptoms. Similar to the literature for adult depression, as many negative as positive study findings have emerged in RCTs of the treatment of childhood and adolescent depression. RCTs to date that have not shown efficacy on primary outcome measures include those using mirtazapine (Remeron), and tricyclic antidepressants. A meta-analysis of SSRI trials in depressed children and adolescents found efficacy of SSRIs compared to placebo with an average response rate of 60 percent for the SSRI compared to 49 percent for placebo. Starting doses of SSRIs for prepubertal children are lower than doses recommended for adults, and adolescents are generally treated at the same doses recommended for adults. Venlafaxine (Effexor), which blocks both serotonin and norepinephrine uptake, has been found to be effective in the TORDIA study; however, adverse effects including increased blood pressure have made this agent a second-line choice compared to the SSRIs. Tricyclic antidepressants are not generally recommended for the treatment of depression in children and adolescents due to a lack of proved efficacy along with the potential risk of cardiac arrhythmia associated with their use. A potential side effect of selective serotonin reuptake inhibitors (SSRIs) in depressed children is behavioral activation, or induction of hypomanic symptoms. In such situations, the medication should be discontinued to determine whether the activation resolves with discontinuation of the medication, or evolves into a hypomanic or manic episode. Activation due to SSRIs, however, do not necessarily predict a diagnosis of bipolar disorder. FDA Warning and Suicidality In September 2004, the FDA received information from their Psychopharmacologic Drug and Pediatric Advisory Committee indicating, based on their review of reported suicidal thoughts and behavior among depressed children and adolescents who participated in randomized clinical trials with nine different antidepressants, an increased risk of suicidality in those children who were on active antidepressant medications. Although no suicides were reported, the rates of suicidal thinking and behaviors were 2 percent for patients on placebo, versus 4 percent among patients on antidepressant medications. The FDA, in accordance with the recommendation of their advisory committees, instituted a “black-box” warning to the health professional label of all antidepressant medication indicating the increased risk of suicidal thoughts and behaviors in children and adolescents being treated with antidepressant medications, and the need for close monitoring for these symptoms. Several reviews since 2004, however, concluded that the data do not indicate a significant increase in the risk of suicide or serious suicide attempts after starting treatment with antidepressant drugs. Duration of Treatment Based on available longitudinal data and the natural history of major depression in children and adolescents, current recommendations include maintaining antidepressant treatment for 1 year in a depressed child who has achieved a good response, and to then discontinue the medication at a time of relatively low stress for a medication-free period. Pharmacologic Treatment Strategies for Resistant Depression Pharmacological recommendations, in accordance with an expert consensus panel that developed the Texas Children’s Medication Algorithm Project (TMAP), as well as the Treatment of SSRI-resistant Adolescents with Depression study (TORDIA) in the treatment of children or adolescents who have not responded to treatment with an SSRI agent is to change to another SSRI medication. If a child is not responsive to the second SSRI medication, then either a combination of antidepressants or augmentation strategies may be reasonable choices as well as an antidepressant from another class of medications. Electroconvulsive Therapy Electroconvulsive therapy (ECT) has been used for a variety of psychiatric illnesses in adults, primarily severe depressive and manic mood disorders and catatonia. ECT is used rarely for adolescents, although published case reports indicate its efficacy in adolescents with depression and mania. Currently case reports suggest that ECT may be a relatively safe and useful treatment for adolescents who have persistent severe affective disorders, particularly with psychotic features, catatonic symptoms, or persistent suicidality. SUICIDE In the United States, suicide is the third leading cause of death among adolescents, after accidental death and homicide. Throughout the world, suicide rarely occurs in children who have not reached puberty. In the last 15 years, the rates of both completed suicide and suicidal ideation rates have decreased among adolescents. This decrease appears to coincide with the increase in SSRI medications prescribed to adolescents with mood and behavioral disturbance. Suicidal Ideation and Behavior Suicidal ideation, gestures, and attempts are frequently, but not always, associated with depressive disorders. Reports indicate that as many as half of suicidal individuals express suicidal intentions to a friend or a relative within 24 hours before enacting suicidal behavior. Suicidal ideation occurs in all age groups and with greatest frequency in children and adolescents with severe mood disorders. More than 12,000 children and adolescents are hospitalized in the United States each year because of suicidal threats or behavior, but completed suicide is rare in children younger than 12 years of age. A young child is hardly capable of designing and carrying out a realistic suicide plan. Cognitive immaturity seems to play a protective role in preventing even children who wish they were dead from committing suicide. Completed suicide occurs about five times more often in adolescent boys than in girls, although the rate of suicide attempts is at least three times higher among adolescent girls than among boys. Suicidal ideation is not a static phenomenon; it can wax and wane with time. The decision to engage in suicidal behavior may be made impulsively without much forethought, or the decision may be the culmination of prolonged rumination. The method of the suicide attempt influences the morbidity and completion rates, independent of the severity of the intent to die at the time of the suicidal behavior. The most common method of completed suicide in children and adolescents is the use of firearms, which accounts for about two thirds of all suicides in boys and almost one half of suicides in girls. The second most common method of suicide in boys, occurring in about one fourth of all cases, is hanging; in girls, about one fourth commit suicide through ingestion of toxic substances. Carbon monoxide poisoning is the next most common method of suicide in boys, but it occurs in less than 10 percent; suicide by hanging and carbon monoxide poisoning are equally frequent among girls and account for about 10 percent each. Additional risk factors in suicide include a family history of suicidal behavior, exposure to family violence, impulsivity, substance abuse, and availability of lethal methods. Gender differences in nonfatal suicidal behavior among 9th grade adolescents in a recent survey of students in 100 high schools found that serious suicidal thoughts were reported in 19.8 percent of female students and 10.8 percent of females had made an attempt. In male students, 9.3 percent had a history of suicidal thoughts and 4.9 percent had made an attempt. In this study, female students showed evidence of higher levels of mood and anxiety problems, whereas males had a slightly higher level of disruptive behavior problems. Female students reported higher levels of depression, anxiety, somatic complaints, and increased levels of emotional and behavioral problems than males. In young adolescents, even without meeting full criteria for psychiatric disorders, females report more psychopathology along with higher likelihood of nonfatal suicidal behavior. Epidemiology In a study of 9- to 16-year-olds in a 3-month period. passive suicidal thoughts were approximately 1 percent, suicidal ideation with a plan was 0.3 percent, and suicide attempt was 0.25 percent. In adolescents 14 to 18 years, the current rate of suicidal ideation was found to be 2.7 percent and annual incidence was 4.3 percent. Among this population of adolescents, lifetime prevalence of suicide attempt was 7.1 percent with a much higher rate of suicidal behavior for girls than for boys: 10.1 percent compared to 3.8 percent. Completed suicide rates in youth are much less common in children and younger teens 10 to 14 years, with a slighter lower rate of 0.95 per 100,000 for females compared to 1.71 per 100,000 for males. In older adolescents 15 to 19 years of age, completed suicide is considerably lower for females, 3.52 per 100,000 compared to males, 12.65 per 100,000 in the United States in 2004. Etiology Universal features in adolescents who resort to suicidal behaviors are the inability to synthesize viable solutions to ongoing problems and the lack of coping strategies to deal with immediate crises. Therefore, a narrow view of the options available to deal with recurrent family discord, rejection, or failure contributes to a decision to commit suicide. Genetic Factors. Completed suicide and suicidal behavior is two to four times more likely to occur in individuals with a first-degree family member with similar behavior. Evidence of a genetic contribution to suicidal behavior is based on family suicide risk studies and the higher concordance for suicide among monozygotic twins compared to dizygotic twins. Recent studies have investigated the possible contributions of the short allele of the serotonin transporter promoter polymorphism (5-HTTLPT) to suicidal behaviors, although to date, the evidence has not been consistent. Current studies are seeking to investigate correlations between genetic vulnerability and environment and timing interactions as multiple variables that may interact to increase the risk of suicidal behavior. Biological Factors. A relationship between altered central serotonin with suicide as well as impulsive aggression has been found in children and adolescents, and has been demonstrated in adults. Studies have documented a reduction in the density of serotonin transporter receptors in the prefrontal cortex, and serotonin receptors among individuals with suicidal behaviors. Postmortem studies in adolescents who have completed suicide show the most significant alterations in the prefrontal cortex and hippocampus, brain regions that are also associated with emotion regulation and problem solving. These studies have found altered serotonin metabolites, alteration in 5HT2a binding and decreased activity of protein kinase A and C. Decreased levels of serotonin metabolite 5-hydroxyindoleacetic acid (5-HIAA) have also been found in the cerebrospinal fluid of depressed adults who attempted suicide by violent methods. Metaanalyses suggest an association between the short S-allele of the serotonin transporter promoter gene and depression as well as suicidal behavior, particularly when combined with adverse life events. Psychosocial Factors. Although severe major depressive illness is the most significant risk factor for suicide, increasing its risk by 20 percent, many severely depressed individuals are not suicidal. A sense of hopelessness, impulsivity, recurrent substance use, and a history of aggressive behavior, have been associated with an increased risk of suicide. A wide range of psychopathological symptoms are associated with exposure to violent and abusive homes. Aggressive, self-destructive, and suicidal behaviors seem to occur with greatest frequency among youth who have endured chronically stressful family lives. The most significant family risk factor for suicidal behavior is maltreatment, including physical and sexual abuse and neglect. The single largest association is between sexual abuse and suicidal behavior. Large community studies have provided data suggesting that youth at risk for suicidal behavior include those who feel disconnected, isolated, or alienated from peers. Sexual orientation is a risk factor, with increased rates of suicidal behavior of two to six times among youth who identify themselves as gay, lesbian, or bisexual. Protective factors mitigating the risk of suicidal behavior are youth who have a strong connection to school and peers even in the face of other risk factors. Diagnosis and Clinical Features The characteristics of adolescents who attempt suicide and those who complete suicide are similar and up to 40 percent of suicidal persons have made a previous attempt. Direct questioning of children and adolescents about suicidal thoughts is necessary, because studies have consistently shown that caregivers are frequently unaware of these ideas in their children. Suicidal thoughts (i.e., children talking about wanting to harm themselves) and suicidal threats (e.g., children stating that they want to jump in front of a car) are more common than suicide completion. Most older adolescents with suicidal behavior meet criteria for one or more psychiatric disorders, often including major depressive disorder, bipolar disorder, and psychotic disorders. Youth with mood disorders in combination with substance abuse and a history of aggressive behavior are at particularly high risk for suicide. The most common precipitating factors in younger adolescent suicide completers appear to be impending disciplinary actions, impulsive behavioral histories, and access to loaded guns, particularly in the home. Adolescents without mood disorders with histories of disruptive and violent, aggressive, and impulsive behavior may be susceptible to suicide during family or peer conflicts. High levels of hopelessness, poor problem-solving skills, and a history of aggressive behavior are risk factors for suicide. A less common profile of an adolescent who completes suicide is one of high achievement and perfectionistic character traits facing a perceived failure, such an academically proficient adolescent humiliated by a poor grade on an exam. Findings from a World Health Organization mental health survey reveals that a range of psychiatric disorders increase the risk of suicidal ideation across the lifespan. Youth with psychiatric disorders characterized by severe anxiety and poor impulse control are at higher risk to act on suicidal ideation. In psychiatrically disturbed and vulnerable adolescents, suicide behavior may represent impulsive responses to recent stressors. Typical precipitants of suicidal behavior include conflicts and arguments with family members and boyfriends or girlfriends. Alcohol and other substance use can further predispose an already vulnerable adolescent to suicidal behavior. In other cases, an adolescent attempts suicide in anticipation of punishment after being caught by the police or other authority figures for a forbidden behavior. About 40 percent of youth who complete suicide had previous psychiatric treatment, and about 40 percent had made a previous suicide attempt. A child who has lost a parent by any means before age 13 is at higher risk for mood disorders and suicide. The precipitating factors include loss of face with peers, a broken romance, school difficulties, unemployment, bereavement, separation, and rejection. Clusters of suicides among adolescents who know one another and go to the same school have been reported. Suicidal behavior can precipitate other such attempts within a peer group through identification—so-called copycat suicides. Some studies have found a transient increase in adolescent suicides after television programs in which the main theme was the suicide of a teenager. The tendency of disturbed young persons to imitate highly publicized suicides has been referred to as Werther syndrome, after the protagonist in Johann Wolfgang von Goethe’s novel, The Sorrows of Young Werther. The novel, in which the hero kills himself, was banned in some European countries after its publication more than 200 years ago because of a rash of suicides by young men who read it; some dressed like Werther before killing themselves or left the book open at the passage describing his death. In general, although imitation may play a role in the timing of suicide attempts by vulnerable adolescents, the overall suicide rate does not seem to increase when media exposure increases. In contrast, direct exposure to peer suicide is associated with increased risk of depression and posttraumatic stress disorder rather than suicide. Treatment The prognostic significance of suicidal ideation and behaviors in adolescents ranges from relatively low lethality, to high risk for completion. One of the challenges in addressing suicide is to identify children and adolescents with suicidal ideation, and particularly to treat those who have untreated psychiatric disorders, as the risk of completed suicide increases with age, as does the onset of an untreated psychiatric disorder. Adolescents who come to medical attention because of suicidal attempts must be evaluated before determining whether hospitalization is necessary. Pediatric patients who present to the emergency room with suicidal ideation benefit from an intervention that occurs in the emergency room to ensure that the patient is transitioned to outpatient care when hospitalization is not necessary. Those who fall into high-risk groups should be hospitalized until the acute suicidality is no longer present. Adolescents at higher risk include those who have made previous suicide attempts, especially with a lethal method, males older than 12 years of age with histories of aggressive behavior or substance abuse, use of a lethal method, and severe major depressive disorder with social withdrawal, hopelessness, and persistent suicidal ideation. Relatively few adolescents evaluated for suicidal behavior in a hospital emergency room subsequently receive ongoing psychiatric treatment. Factors that may increase the probability of psychiatric treatment include psychoeducation for the family in the emergency room, diffusing acute family conflict, and setting up an outpatient follow-up during the emergency room visit. Emergency room discharge plans often include providing an alternative if suicidal ideation reoccurs, and a telephone hot-line number provided to the adolescent and the family in case suicidal ideation reappears. Scant data exist to evaluate the efficacy of various interventions in reducing suicidal behavior among adolescents. CBT alone and in combination with SSRIs have been shown to decrease suicidal ideation in depressed adolescents over time in the Treatment of Adolescent Depression (TADS) study, a large multisite study; however, these interventions do not work immediately, so safety precautions must be taken for highrisk situations. Dialectical behavior therapy (DBT), a long-term behavioral intervention that can be applied to individuals or groups of patients, has been shown to reduce suicidal behavior in adults, but has yet to be investigated in adolescents. Components of DBT include mindfulness training to improve self-acceptance, assertiveness training, instruction on avoiding situations that may trigger self-destructive behavior, and increasing the ability to tolerate psychological distress. This approach warrants investigation among adolescents. Given the reduction in completed suicide among adolescents over the last decade, during the same period in which SSRI treatment in the adolescent population has markedly risen, it is possible that SSRIs have been instrumental in this effect. Given the risk of increased rate of suicidal thoughts and behaviors among depressed children and adolescents (indicated in randomized clinical trials with antidepressant medications and leading to the “black-box” warning for all antidepressants for depressed youth), close monitoring for suicidality is mandatory for any child or adolescent being treated with antidepressants. REFERENCES Bayer JK, Rapee RM, Hiscock H, Ukoumunne OC, Mihalopoulos C, Wake M. Translational research to prevent internalizing problems in early childhood. Depress Anxiety. 2011;28:50–57. Brent D, Emslie E, Clarke G, Wagner KD, Asarnow JR, Keller M, Ritz, L, Iyengar S, Abebe K, Birmaher B, Ryan N, Kennard B, Hughers C, DeBar L, McCracken J, Strober M, Suddath R, Spirito A, Leonard H, Meham N, Pora G, Onorato M, Zelazny J. Switching to another SSRI or to venlafaxine with or without cognitive behavioral therapy for adolescents with SSRI-resistant depression: The TORIDA Randomized Controlled Trial. JAMA. 2008:299:901–913. Correll CU, Kratocvil CJ, March J. Developments in pediatric psychopharmacology: Focus on stimulants, antidepressants and antipsychotics. J Clin Psychiatry. 2011;72:655–670. Christiansen E, Larsen KJ. Young people’s risk of suicide attempts after contact with a psychiatric department—A nested case-control design using Danish register data. J Child Psychol Psychiatry. 2011;52:102. Field T. Prenatal depression effects on early development: A review. Infant Behav Dev. 2011;34:1–14. Frodl T, Reinhold E, Koutsoulieris N, Donohoe G, Bondy B, Reiser M, Moller Hj, Meisenzahl EM. Childhood stress, serotonin transporter gene and brain structures in major depression. Neuropsychopharmacology. 2010;35:1383–1390. Gould MS, Greenberg T, Velting DM, Shaffer D. Youth suicide risk and preventive interventions: A review of the past ten years. J Am Acad Child Adolesc Psychiatry. 2003;42:386. Hall WD. How have the SSRI antidepressants affected suicide risk? Lancet. 2006;367(9527):1959. Harro J, Kiive E. Droplets of black bile? Development of vulnerability and resilience to depression in young age. Psychoneuroendocrinology. 2011;36:380–392. Heiligenstein JH, Hoog SL, Wagner KD, Findling RL, Galil N, Kaplan S, Busner J, Nilsson ME, Brown EB, Jacobson JG. Fluoxetine 40–60 mg versus fluoxetine 20 mg in the treatment of children and adolescents with a less-than-complete response to nine-week treatment with fluoxetine 10–20 mg: A pilot study. J Child Adolesc Psychopharmacol. 2006;1/2:207. Hughes CW, Emslie GJ, Crimson ML, Posner K, Birmaher B, Ryan N, Jensen P, Curry J, Vitiello B, Lopez M, Shon SP, Piszka SR, Trivedi MH, and The Texas Consensus Conference Panel on Medication Treatment of Childhood Major Depressive Disorder. Texas Children’s Medication Algorithm Project: Update from Texas Consensus Conference Panel on medication treatment of childhood major depressive disorder. J Am Acad Child Adolesc Psychiatry. 2007;46:667–686. Kaess M, Parzer P, Haffner J, Steen Rm, Roos J, Klett M, Brunner R, Resch F. Explaining gender differences in non-fatal suicidal behavior among adolescents: A population-based study. BMC Pub Health. 2011:597–603. Luby J, Lenze S, Tillman R. A novel early intervention for preschool depression: Findings from a pilot randomized controlled trial. J Child Psychol and Psychiatry. 2011:1–10. March J, Silva S, Petrycki S. The TADS Team. The Treatment for Adolescents with Depression Study (TADS): Long-term effectiveness and safety outcomes. Arch Gen Psychiatry. 2007;64:1132–1143. 30 - 31.12b Early Onset Bipolar Disorder 31.12b Early-Onset Bipolar Disorder Newton AS, Hamm MP, Bethell J, Rhodes AE, Bryan CJ, Tjosvold L, Ali S, Logue E, Manion ID. Pediatric suicide-related presentations: A systematic review of mental health care in the emergency room department. Ann Emerg Med. 2010;56:649–659. Nock MK, Hwang I, Sampson N, Kessler RC, Angermeyer M, Beautrais A, Borges G, Bromet E, Bruffaerts R, de Girolamo G, de Graaf R, Florescu S, Gureje O, Haro JM, Hu C, Huang Y, Karam EG, Kawakami N, Kovess V, Levinson D, Postada-Villa J, Sagar R, Tomov T, Viana MC, Williams DR. Cross-national analysis of the associations among mental disorders and suicidal behavior: Findings from the WHO World Mental Health Surveys. PLoS Med. 2009;6:1–13. Olfson M, Shaffer D, Marcus SC, Greenberg T. Relationship between antidepressant medication treatment and suicide in adolescents. Arch Gen Psychiatry. 2003;60:978. Rosso IM, Cintron CM, Steingard RJ, Renshaw PF, Young AD, Yurgelun-Todd DA. Amygdala and hippocampus volumes in pediatric major depression. Biol Psychiatry. 2005;57(1):21. Von Knorring AL, Olsson GI, Thomson PH, Lemming OM, Hulten A. A randomized, double-blind, placebo-controlled study of citalopram in adolescents with major depressive disorder. J Clin Psychopharmacol. 2006;26:311. Wagner KD. Pharmacotherapy for major depression in children and adolescents. Prog Neuropsychopharmacol Biol Psychiatry. 2005;29:819. Wagner KD, Brent DA. Depressive disorders and suicide in children and adolescents. In: Sadock BJ, Sadock VA, Ruiz P, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Vol. 2. Lippincott Williams & Wilkins; 2009:3652. Whittington CJ, Kendall T, Fonagy P, Cotrell D, Cotgrove A, Boddington E. Selective serotonin reuptake inhibitors in childhood depression: Systematic review of published versus unpublished data. Lancet. 2004;363:1341. Zalsman G. Timing is critical: gene, environment and timing interactions in genetics of suicide in children and adolescents. Eur Psychiatry. 2010:25:284–286. 31.12b Early-Onset Bipolar Disorder Early onset bipolar disorder has been recognized in children as a rare disorder with greater continuity with its adult counterpart when it occurs in adolescents than in prepubertal children. Over the last decade there has been a significant increase in the diagnosis of bipolar I disorder made in youth referred to psychiatric outpatient clinics and inpatient units. Questions have arisen regarding the phenotype of bipolar disorder in youth, particularly in view of the continuous irritability and mood dysregulation and lack of discrete mood episodes in most prepubertal children who have received the diagnosis. The “atypical” bipolar symptoms among prepubertal children often include extreme mood dysregulation, severe temper tantrums, intermittent aggressive or explosive behavior, and high levels of distractibility and inattention. This constellation of mood and behavior disturbance in the majority of prepubertal children with a current diagnosis of bipolar disorder is nonepisodic, although some fluctuation in mood may occur. The high frequency of the above symptoms in combination with chronic irritability has led to the inclusion of a new mood disorder in youth in the Fifth Edition of the American Psychiatric Association’s Diagnostic and Statistical Manual of Mental Disorders (DSM-5) called Disruptive Mood Dysregulation Disorder, which is discussed in the next section (31.12c). Many children with nonepisodic mood disorders often have past histories of severe attention-deficit/hyperactivity disorder (ADHD), making the diagnosis of bipolar disorder even more complicated. Family studies of children with ADHD have not revealed an increased rate of bipolar I disorder. Children with “atypical” bipolar disorders, however, are frequently seriously impaired, are difficult to manage in school and at home, and often require psychiatric hospitalization. Longitudinal follow-up studies are under way with groups of children diagnosed with subthreshold bipolar disorders and nonepisodic mood disorders, to determine how many will develop classic bipolar disorder. In one recent study of 140 children with bipolar disorder not otherwise specified (that is, the presence of distinct manic symptoms but subthreshold for manic episodes), 45 percent developed bipolar I or bipolar II illness over a follow-up period of 5 years. In another study, 84 children who were labeled with “severe mood dysregulation” (that is, a persistent nonepisodic negative mood along with severe anger outbursts) who also exhibited at least three manic symptoms (either pressured speech, agitation, insomnia, or flight of ideas) plus distractibility (also common to ADHD), followed for approximately 2 years, found that only one child experienced a hypomanic or mixed episode. Although childhood severe mood dysregulation has been found to be common in community samples—one study reported a lifetime prevalence of 3.3 percent in youth 9 to 19 years of age—its relationship to future bipolar disorder remains questionable. A longitudinal community-based study that followed children and adolescents with nonepisodic irritability over a 20-year period, found that these children were at higher risk to develop depressive disorders and generalized anxiety disorder, rather than bipolar disorders over time. Among adults and older adolescents with bipolar disorder who present with classic manic episodes, a major depressive episode typically precedes a manic episode. A classic manic episode in an adolescent, similar to in a young adult, may emerge as a distinct departure from a preexisting state often characterized by grandiose and paranoid delusions and hallucinatory phenomena. According to DSM-5, the diagnostic criteria for a manic episode are the same for children and adolescents as for adults (see Table 8.16). The diagnostic criteria for a manic episode include a distinct period of an abnormally elevated, expansive, or irritable mood that lasts at least 1 week or for any duration if hospitalization is necessary. In addition, during periods of mood disturbance, at least three of the following significant and persistent symptoms must be present: inflated self-esteem or grandiosity, decreased need for sleep, pressure to talk, flight of ideas or racing thoughts, distractibility, an increase in goal-directed activity, and excessive involvement in pleasurable activities that may result in painful consequences. According to the DSM-5, in contrast to DSM-IV-TR, diagnostic criteria for bipolar disorder now include changes in both mood and activity or energy level. Furthermore, whereas previously, full criteria for both mania or hypomania and major depressive disorder were required to make a diagnosis of a mixed episode, in DSM-5, this requirement no longer applies; instead a specifier, “with mixed features,” has been added. This specifier can be applied to a current manic episode, hypomanic episode, or depressive episode. Thus, for example, in order to add the “mixed features” specifier to a manic or hypomanic episode, three of the following symptoms must be present during the majority of days of the current or most recent episode of mania or hypomania: prominent depressed mood, diminished interest in most activities, psychomotor retardation nearly every day, fatigue or loss of energy, feelings of excessive guilt or worthlessness, or recurrent thoughts of death. To apply the “with mixed features” specifier to a full major depressive episode, three of the following hypomanic/manic symptoms must be present: elevated or expansive mood, grandiosity, pressured speech or increased speech, flight of ideas, increased energy, or decreased need for sleep. When mania appears in an adolescent, there is a high incidence of psychotic features including both delusions and hallucinations, which most typically involve grandiose notions about their power, worth, and relationships. Persecutory delusions and flight of ideas are also common. Overall, gross impairment of reality testing is common in adolescent manic episodes. In adolescents with major depressive disorder destined for bipolar I disorder, those at highest risk have family histories of bipolar I disorder and exhibit acute, severe depressive episodes with psychosis, hypersomnia, and psychomotor retardation. EPIDEMIOLOGY The prevalence rates of bipolar disorder among youth vary depending on the age group studied, and on whether the diagnostic criteria are applied narrowly, restricting it to discrete mood episodes or more broadly, to include nonepisodic mood and behavioral states. In younger children, bipolar disorder is extremely rare, with no cases of bipolar I disorder identified in children between the ages of 9 years and 13 years by the Great Smokey Mountain Study. However, severe mood dysregulation, often a prominent feature in prepubertal children receiving a diagnosis of bipolar disorder, was found in 3.3 percent of an epidemiological sample. In adolescents, bipolar disorder is more frequent, found to range from 0.06 to 0.1 percent of the general population of 16-yearolds in studies using a narrow definition of bipolar I disorder. Prevalence of subthreshold symptoms of bipolar illness was found to be 5.7 percent in one study to at least 10 percent in another. Follow-up studies into adulthood revealed that the subthreshold manic symptoms predicted high levels of impairment with progression to depression and anxiety disorders, not bipolar I or II disorders. Community use of the diagnosis of bipolar disorder in youth has increased markedly over the last 15 years in both outpatient and inpatient psychiatric settings. A recent survey indicated a 40-fold increase in the diagnosis of bipolar disorder in youth being treated at outpatient clinics from the mid-1990s to the mid-2000s. Furthermore, from 2000 through 2006, the rate of youth hospitalized with a primary diagnosis of bipolar disorder increased from 3.3 per 10,000 to 5.7 per 10,000. ETIOLOGY Genetic Factors Estimates of the heritability of bipolar disorder based on adult twin studies range from approximately 60 to 90 percent, with shared environmental variables accounting for 30 to 40 percent and the nonshared environmental factors accounting for approximately 10 to 20 percent. High rates of bipolar disorder have been reported in the relatives of the narrow phenotype of early onset bipolar disorder compared to young adult-onset of bipolar disorder. The high rates of comorbid ADHD among children with early onset bipolar disorder has led to questions regarding the co-transmission of these disorders in family members. However, children with the broader phenotype of bipolar disorder, that is, severe mood dysregulation without episodes of mania, have not been found to have higher rates of bipolar disorder in family members, which suggests that the narrow and broad phenotypes of bipolar disorder may be distinct and separate entities. Nearly 25 percent of adolescent offspring of families with probands with bipolar disorder experienced a mood disorder by the age of 17 years old, compared to 4 percent of controls, with approximately 8 percent representing bipolar I, bipolar II, or bipolar disorder not otherwise specified. Most of the risk in the offspring, therefore, is for unipolar major depressive disorder. Disruptive behavior disorders were not found to be increased, in a longitudinal study, in the offspring of families with a bipolar proband, compared to controls. The combination of ADHD and bipolar disorder is not found as frequently in relatives of children with only ADHD compared with first-degree relatives of children with the combination. Although bipolar disorder appears to have a significant heritable component, its mode of inheritance remains unknown. A number of research groups have concluded that early onset bipolar disorder is a more severe form of the illness, characterized by more mixed episodes, greater psychiatric comorbidity, more lifetime psychotic symptoms, poorer response to prophylactic lithium treatment, and a greater heritability. The European collaborative study of early-onset bipolar disorder (France, Germany, Ireland, Scotland, Switzerland, England, and Slovenia) carried out a genome-wide linkage analysis of both the narrow and the broad early onset bipolar disorder. This group concluded that a genetic factor located in the 2q14 region is either specifically involved in the etiology of early onset bipolar disorder, or that a gene in this region exerts influence as a modifier of other genes in the development of bipolar disorder in this age group. Other linkage regions that were found by this collaborative did not find specific genome regions that pertained only to the early onset group of bipolar disorder, suggesting that there may be some genetic factors common to early-onset and adultonset bipolar disorder. This is consistent with the increased incidence of adult-onset bipolar disorder among siblings of early onset disease. Further genome-wide studies are needed to elucidate the genetic etiology of early onset bipolar disorder. Neurobiological Factors Converging data suggest that early-onset bipolar disorder is associated with both structural and functional brain alterations in prefrontal cortical and subcortical regions associated with the processing and regulation of emotional stimuli. Structural magnetic resonance imaging (MRI) studies suggest that altered development of white matter and a decreased amygdalar volume are found more frequently in this population than in the general population. Functional MRI (fMRI) studies are important in that they can identify altered brain function in vulnerable populations such as youth with early-onset bipolar disorder at baseline, and can also be utilized to elucidate functional changes toward normalization in brain functioning after various treatments, and potentially identify pretreatment neural predictors of good response to various treatments. A recent fMRI study of pediatric bipolar patients documented pretreatment brain activity and posttreatment effects of a trial of risperidone versus divalproex. This double-blind study included 24 unmedicated manic patients with a mean age of 13 years, randomized to either risperidone or divalproex treatment, and 14 healthy controls examined over a 6-week period. Prior to treatment, the patient group showed increased amygdala activity compared to healthy controls, which was poorly controlled by the higher ventrolateral prefrontal cortex (VLPFC) and the dorsolateral prefrontal cortex (DLPFC), which are believed to exert influence on the amygdala to control emotional regulation and processing. Increased amygdala activity at baseline predicted a poorer treatment response to both the risperidone and the divalproex in the patient group. Patients were given an affective color-matching word task involving matching positive words (i.e., happiness, achievement, success), negative words (i.e., disappointment, depression, or rejection), or neutral words, with one of two colored circles displayed on a screen while fMRI was administered. Greater pretreatment right amygdala activity during a word task with positive and negative words in the risperidone group, and greater pretreatment left amygdala activity with a positive word task in the divalproex group, predicted poor response on the Young Mania Rating Scale. Increased amygdala activity in early-onset bipolar patients is hypothesized to be a potential biomarker predicting resistance and poor treatment response to both risperidone and divalproex. Neuropsychological Studies Impairments in verbal memory, processing speed, executive function, working memory, and attention are commonly found in early-onset bipolar disorder. Data suggest that on tasks of working memory, processing speed, and attention, children and adolescents with comorbid bipolar disorder and ADHD demonstrate more pronounced impairments compared with those without ADHD. Other studies found that children with bipolar disorder make a greater number of emotion recognition errors compared with controls. They more frequently identified faces as “angry” when presented with adult faces; however, these errors did not occur when children’s faces were shown. Impaired perception of facial expression has also been reported in studies of adults with bipolar disorder. DIAGNOSIS AND CLINICAL FEATURES Early onset bipolar disorder is often characterized by extreme irritability that is severe and persistent, and may include aggressive outbursts and violent behavior. In between outbursts, children with the broad diagnosis may continue to be angry or dysphoric. It is rare for a prepubertal child to exhibit grandiose thoughts or euphoric mood; for the most part, children diagnosed with early onset bipolar disorder are intensely emotional with a fluctuating but overriding negative mood. Current diagnostic criteria for bipolar disorders in children and adolescents in DSM-5 are the same as those used in adults (see Tables 8.1-6 and 31.12b-1). The clinical picture of early-onset bipolar disorder, however, is complicated by the prevalence of comorbid psychiatric disorders. Table 31.12b-1 DSM-5 Diagnostic Criteria for Bipolar II Disorder Comorbidity with ADHD ADHD is the most common comorbid condition among youth with early onset bipolar disorder and has been reported in up to 90 percent of prepubertal children and up to 50 percent of adolescents diagnosed with bipolar disorder. One of the main sources of diagnostic confusion regarding children with early onset bipolar disorder is the comorbid ADHD, since the two disorders share many diagnostic criteria, including distractibility, hyperactivity, and talkativeness. Even when the overlapping symptoms are removed from the diagnostic count, a significant percentage of children with bipolar disorder continued to meet the full criteria for ADHD. This implies that both disorders with their own distinct features are present in many cases. Comorbidity with Anxiety Disorders Children and adolescents with bipolar disorder have been reported to have higher than expected rates of panic and other anxiety disorders. In youth with the narrow phenotype of bipolar disorders, up to 77 percent have been reported to exhibit an anxiety disorder. Lifetime prevalence of panic disorder was found to be 21 percent among subjects with the broader phenotype of bipolar disorder compared with 0.8 percent in those without mood disorders. Patients diagnosed with bipolar disorder who have comorbid high levels of anxiety symptoms are reported as adults to have higher risks of alcohol abuse and suicidal behavior. On the other hand, children who exhibit the broader phenotype of bipolar disorder are at higher risk to go on to have anxiety disorders as well as depressive disorders. Jeanie is a 13-year-old adopted teen who was admitted to the hospital after assaulting her adoptive mother, causing bruises on her arms and legs from Jeanie’s kicks and punches. Jeanie has had a long history of excessively severe tantrums, which include assaultive and self-injurious behavior since before she was adopted at the age of 3 years. Jeanie had always been a child who was irritable and explosive, with a short fuse, who could blow up with very little provocation, even when things were going her way. Jeanie had become increasingly hard to manage at home, refused to go to school, yelled and screamed for hours on a daily basis, and often hit and kicked her adoptive parents by the time she was 10 years old. Jeannie had been placed in residential treatment for about a year and a half from age 11 and a half to almost 13, where she had been given a diagnosis of bipolar disorder and placed on lithium and citalopram. She was doing so well there after a year that Jeanie’s adoptive mother decided to take her home. After a few weeks at home, however, Jeanie began to decompensate, having daily explosive tantrums during which she became aggressive and out of control. On multiple occasions she had hurt herself and her adoptive mother and father. Upon arriving at the hospital, Jeanie was calm by the time she was brought to her hospital room; however, her adoptive mother refused to consider taking her home until she had received a full psychiatric evaluation and something new was done to control Jeanie’s unsafe behaviors. Jeanie was initially evaluated by the child and adolescent psychiatrist on-call, after which she was admitted to a pediatric inpatient unit, where she awaited a bed on a psychiatric adolescent inpatient unit. The psychiatrist learned that Jeanie had been born prematurely to a teenage mother and placed in multiple foster homes until she was adopted. Jeanie was a small girl who appeared younger than her stated age, although her demeanor was bossy and pedantic. Jeanie’s biological family history was unknown, and although she had at least one stigmata of fetal alcohol syndrome, her IQ was in the average range and there was no other evidence to corroborate this possibility. On mental status examination in the hospital, Jeanie reported that things were fine, that she was not depressed, and that she did not get along with kids her own age but that she had a few friends. Jeanie admitted that she had a bad temper and that she did not remember what she did after she was in a rage. Jeanie’s affect was odd, and she seemed to like having the psychiatrist as her audience. Jeanie denied suicidal ideation or past attempts, and she denied having been a danger to herself or her adoptive parents. Jeanie seemed annoyed when she was asked about the reasons for her placement in a residential facility, and she became irritable when questioned about the reasons for her current admission. Jeanie was referred for admission to an adolescent psychiatric inpatient unit with the following recommendations: Jeanie was referred for a trial of an atypical antipsychotic, such as risperidone or olanzapine, and a reconsideration of a return to a more structured school program, either a day program or residential facility. The diagnosis of bipolar disorder remained in question, as she did not meet the narrow phenotype for this disorder. PATHOLOGY AND LABORATORY EXAMINATION No specific laboratory indices are currently helpful in making the diagnosis of bipolar disorders among children and adolescents. DIFFERENTIAL DIAGNOSIS The most important clinical entities to distinguish from early onset bipolar disorder are also the disorders with which it is most frequently comorbid. Included are ADHD, oppositional defiant disorder, conduct disorder, anxiety disorders, and depressive disorders. Although childhood ADHD tends to have its onset earlier than pediatric mania, current evidence from family studies supports the presence of ADHD and bipolar disorders as highly comorbid in children, and the concurrence is not because of the overlapping symptoms that the two disorders share. In a recent study of more than 300 children and adolescents who attended a psychopharmacology clinic and received a diagnosis of ADHD, bipolar disorder was also evident in almost one third of those children with ADHD who had combined–type and hyperactive-types, and occurred with much less frequency (i.e., in less than 10 percent) in children with ADHD, inattentive-type. COURSE AND PROGNOSIS There are several pathways regarding the course and prognosis of children diagnosed with early onset bipolar disorder. Those who present with severe mood dysregulation at an early age, without discrete mood cycles, are most likely to develop anxiety and depressive disorders as they mature. Youth who present in adolescence with a recognizable manic episode are most likely to continue to meet criteria for bipolar I disorder in adulthood. In both cases, the long-term impairment is considerable. A longitudinal study of 263 child and adolescent inpatients and outpatients with bipolar disorder followed for an average of 2 years found that approximately 70 percent recovered from their index episode within that period. Half of these patients had at least one recurrence of a mood disorder during this time, more frequently a depressive episode than a mania. No differences were found in the rates of recovery for children and adolescents whose diagnosis was bipolar I disorder, bipolar II disorder, or bipolar disorder not otherwise specified; however, those youth whose diagnosis was bipolar disorder not otherwise specified had a significant longer duration of illness before recovery, with less frequent recurrences once they recovered. About 19 percent of patients changed polarity once per year or less, 61 percent shifted five or more times per year, about half cycled more than ten times per year, and about one third cycled more than 20 times per year. Predictors of more rapid cycling included lower socioeconomic status (SES), presence of lifetime psychosis, and bipolar disorder not otherwise specified diagnosis. Over the follow-up period, about 20 percent of subjects who were diagnosed with bipolar II disorder converted to bipolar I disorder, and 25 percent of the bipolar disorder not otherwise specified subjects developed bipolar I disorder or bipolar II disorder during the follow-up period. Similar to the natural history of bipolar disorders in adults, children have a wide range of symptom severity in manic and depressed episodes. The more frequent diagnostic conversions from bipolar II disorder to bipolar I disorder among children and adolescents, compared with adults, highlight the lack of stability of the bipolar II disorder diagnosis in youth. This is also the case with respect to conversion from bipolar disorder not otherwise specified to other bipolar disorders. When bipolar disorder occurs in young children, recovery rates are lower. In addition, a greater likelihood is seen of mixed states and rapid cycling, and higher rates of polarity changes compared with those who develop bipolar disorders in late adolescence or early adulthood. TREATMENT Treatment of early onset bipolar disorder incorporates multimodal interventions including pharmacotherapy, psychoeducation, psychosocial intervention with the family and the child, and school interventions to optimize a child’s school adjustment and achievement. Pharmacotherapy Two classes of medications—atypical antipsychotics and mood stabilizing agents—are the most well-studied agents that provide efficacy in the treatment of early-onset bipolar disorders. Eight randomized controlled trials have shown efficacy of atypical antipsychotic agents in the treatment of bipolar disorder in youth between the ages of 10 and 17 years. These studies compared an atypical antipsychotic to placebo, or compared an atypical antipsychotic to a mood stabilizer, or added an antipsychotic to a mood-stabilizing agent. The atypical antipsychotics included olanzapine, quetiapine, risperidone, aripiprazole, and ziprasidone. All five of the atypical antipsychotic studies demonstrated significant efficacy in the treatment of early onset bipolar manic or mixed states. A recent trial comparing quetiapine and valproate found that both were efficacious, but the quetiapine was superior in the speed of its effect. In another trial comparing risperidone and divalproex treatment for bipolar disorder in youth, risperidone was found to have a more rapid improvement and a greater final reduction in manic symptoms compared to divalproex. Mood-stabilizing agents have been used in open trials and anecdotally with early onset bipolar illness with little evidence of efficacy at this time. In trials using lithium or divalproex for treatment of early onset bipolar disorder, responses were less robust compared to results with atypical antipsychotics. Controlled trials have provided some evidence suggesting that lithium is efficacious in the management of aggression behavior disorders. Although lithium has been approved for use in adolescent mania, more research is needed to know if lithium is effective for more classic forms of mania in adolescents. The Collaborative Lithium Trials (CoLT) established a set of protocols to establish the safety and potential efficacy of lithium in youth, and to develop studies to provide evidence-based dosing of lithium for youth. A group of researchers recently studied the first-dose pharmacokinetics of lithium carbonate in youth and found that clearance and volume are correlated with total body weight in youth, and particularly with fat-free mass. Difference in body size was consistent with the pharmacokinetics of lithium metabolism in children and adults. An open-label trial of lamotrigine (Lamictal) in the treatment of bipolar depression among youth provides possible support for its use in children and adolescents. Current evidence suggests a faster response and more robust effect with atypical antipsychotics compared to mood-stabilizing agents in the treatment of early-onset bipolar disorder. However, given the severity and impairment of bipolar disorder in youth, when only partial recovery is achieved, consideration of adding an additional agent may be necessary. Psychosocial Treatment Psychosocial treatment interventions for early onset bipolar illness have included a family-focused treatment. This treatment consists of several sessions of psychoeducation, then sessions focusing on current stressors and mood management plan, and then several sessions of communication enhancement training and problem-solving skills training. The use of this type of intervention for youth diagnosed with bipolar disorder as well as youth at risk for the disorder by virtue of their family history or subthreshold conditions has been of value. Adjunctive family-focused psychoeducational treatment modified for children and adolescents has been shown to reduce relapse rate. Children and adolescents treated with mood-stabilizing agents in addition to a psychosocial intervention showed improvement in depressive symptoms, manic symptoms, and behavioral disturbance over 1 year. A year-long trial of a modified Family Focused Treatment-High Risk in youth with bipolar disorder showed significant improvement in mood disturbance, especially depressive mood and hypomania, and improved psychosocial functioning. Familyfocused treatment for high-risk youth is a promising intervention that deserves further investigation as a longitudinal follow-up to determine the course of youth at risk to develop bipolar disorder. REFERENCES Axelson DA, Birmaher B, Strober M, Goldstein BI, Ha W, Gill MK, Goldstein TR, Yen S, Hower H, Hunt JI, Liao F, Iyengar S, Dickstein D, Kim E, Ryan ND, Frankel E, Keller MB. Course of subthreshold bipolar disorder in youth: Diagnostic progression from bipolar disorder not otherwise specified. J Am Acad Child Adolesc Psychiatry. 2011;50:1001–1016. Carlson GA. Bipolar disorder and mood dysregulation. Proceedings; AACAP 2011 Psychopharmacology Update Institute: Controversies in child and adolescent psychopharmacology. 2011;257–284. Carlson GA, Myer SE. Early-onset bipolar disorder In: Sadock BJ, Sadock VA, Ruiz P, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Vol. 2. Lippincott Williams & Wilkins; 2009:3663. Correll CU, Sheridan EM, DelBello MP. Antipsychotic and mood stabilizer efficacy and tolerability in pediatric and adult 31 - 31.12c Disruptive Mood Dysregulation Disorder 31.12c Disruptive Mood Dysregulation Disorder patients with bipolar I mania: a comparative analysis of acute, randomized, placebo-controlled trials. Bipolar Disorders. 2010;12:116–141. Correll CU, Kratochvil CJ, March JS. Developments in pediatric psychopharmacology: Focus on stimulants, antidepressants and antipsychotics. J Clin Psychiatry. 2011;72:655–670. Findling RL, Landersdorfer CB, Kafantaris V, Pavulari M, McNamara NK, McClellan J, Frazier JA, Sikich L, Kowatch R, Lingler J, Faber J, Taylor-Zapata, Jusko WJ. First-dose pharmacokinetics of lithium carbonate in children and adolescents. J Clin Psychopharmacol. 2010;30:404–410. Larsky T, Krieger A, Elixhauser A, Vitiello B. Children’s hospitalizations with a mood disorder diagnosis in general hospitals in the United States 2000-2006. Child Adolesc Psychiatry Mental Health. 2011;5:27–34. Mathieu F, Dizier M-H, Etain B, Jamain S, Rietschel M, Maier W, Albus M, McKeon P, Roche S, Blackwood D, Muir W, Henry C, Malafosse A, Preisig M, Ferrero F, Cichon S, Schumacher J, Ohlraun S, Propping P, Jamra RA, Schulze TG, Zelenica D, Charon C, Marusic A, Dernovsek MC, Gurling H, Nothen M, Lathrop M, Leboyer M, Bellivier F. European collaborative study of early-onset bipolar disorder: Evidence for heterogeneity on 2q14 according to age at onset. Am J Med Genet Part B. 2010;153B:1425–1433. McNamara RK, Nandagopal JJ, Strakowski SM, DelBello M. Preventive strategies for early-onset bipolar disorder. Toward a clinical staging model. CNS Drugs. 2010; 24:983-996. Miklowitz DJ, Chang KD, Taylor DO, George EL, Singh MK, Schneck CD, Dickinson LM, Howe ME, Garber J. Early psychosocial intervention for youth at risk for bipolar I or II disorder: A one-year treatment development trial. Bipolar Disorders. 2011;13:67–75. Moreno C, Laje G, Blancvo C, Jiang H, Schmidtg AB, Olfson M. National trends in the outpatient diagnosis and treatment of bipolar disorder in youth. Arch Gen Psychiatry. 2007;64:1032–1039. Nieto RG, Castellanos FX. A meta-analysis of neuropsychological functioning in patients with early onset schizophrenia and pediatric bipolar illness. J Clin Child Adolesc Psychol. 2011;40:266–280. Nurnberger JI, McInnis M, Reich SW, Kastelic E, Wilcox HC, Glowinski A, Mitchell P, Fisher C, Erpe M, Gershon E, Berrettini W, Laite G, Schweitzer R, Rhoadarmer K, Coleman VV, Cai X, Azzouz F, Liu H, Kamali M, Brucksch C, Monahan PO. A high-risk study of bipolar disorder. Childhood clinical phenotypes as precursors of major mood disorders. Arch Gen Psychiatry. 2011;68:1012–1020. Pavulari MN, Passarotti AM, Lu LH, Carbray JA, Sweeney JA. Double-blind randomized trial of risperidone versus divalproex in pediatric bipolar disorder: fMRI outcomes. Psychiatry Res: Neuroimaging. 2011;193:28–37. Pavulari MN, Henry DB, Findling RL, Parnes S, Carbray JA, Mohammed T, Janicak PG, Sweeney JA. Double-blind randomized trial of risperidone versus divalproex in pediatric bipolar disorder. Bipolar Disorders. 2010;12:593–605. Stringaris A, Baroni A, Haimm C, Brotman M, Lowe CH, Myers F, Rustgi E, Wheeler W, Kayser R, Towbin K, Leibenluft E. Pediatric bipolar disorder versus severe mood dysregulation: Risk for manic episodes on follow-up. J Am Acad Child Adolesc Psychiatry. 2010;49:397–405. Versace Am Ladouceur CD, Romero S, Birmaher B, Axelson DA, Kupfer DJ, Phillips ML. Altered development of white matter in youth at high familial risk for bipolar disorder: a diffusion tensor imaging study. J Am Acad Child Adolesc Psychiatry. 2010;49:1249–1259. 31.12c Disruptive Mood Dysregulation Disorder Disruptive mood dysregulation disorder, a new inclusion in the American Psychiatric Association’s Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5), is characterized by severe, developmentally inappropriate, and recurrent temper outbursts at least three times per week, along with a persistently irritable or angry mood between temper outbursts. In order to meet diagnostic criteria, the symptoms must be present for at least a year, and the onset of symptoms must be present by the age of 10 years old. Children with these symptoms have typically been diagnosed with bipolar disorder, or a combination of oppositional defiant disorder, ADHD and intermittent explosive disorder. Recent longitudinal data suggest, however, that these children do not typically develop classic bipolar disorder in late adolescence or early adulthood. Instead, studies suggest that youth with chronic irritability and severe mood dysregulation are at higher risk for future unipolar depressive disorders and anxiety disorders. Although the initial studies of children and adolescents with severe mood dysregulation included several symptoms of hyperarousal (such as distractibility, physical restlessness, insomnia, racing thoughts, flight of ideas, pressured speech, or intrusiveness), the current DSM-5 diagnostic criteria for disruptive mood dysregulation do not include any hyperarousal criteria. Youths diagnosed with mood dysregulation disorder who also exhibit multiple symptoms of hyperarousal may be comorbid for ADHD. EPIDEMIOLOGY Most of the epidemiological data applied to disruptive mood dysregulation disorder was gathered from children and adolescents with severe mood dysregulation, which includes hyperarousal symptoms. Because disruptive mood dysregulation disorder differs from severe mood dysregulation disorder only in the absence of hyperarousal symptoms, the epidemiological data from the severe mood dysregulation disorder studies can be viewed as a useful proxy for disruptive mood dysregulation disorder. Severe mood dysregulation has a lifetime prevalence of 3 percent in children age 9 to 19 years. Within that percentage, males (78 percent) are more prevalent than females (22 percent). The mean age of onset is 5 to 11 years of age. COMORBIDITY Disruptive mood dysregulation disorder often co-occurs with other psychiatric disorders. The most common comorbidities are ADHD (94 percent), oppositional defiant disorder (84 percent), anxiety disorders (47 percent), and major depressive disorder (20 percent). The relationship of severe mood dysregulation and disruptive mood dysregulation disorder to bipolar disorder has been a topic of clinical investigation. Youth with severe mood dysregulation and hyperarousal symptoms have been conceptualized as a “broad phenotype” of pediatric bipolar disorder, however, the term “severe mood dysregulation” was utilized by researchers for these youth because it remains unclear whether these youth go on to meet criteria for a bipolar disorder. Disruptive mood dysregulation disorder is conceptualized as a disorder that is not episodic, and may coexist with ADHD. However, current evidence does not support its continuity with an emerging bipolar disorder. DIAGNOSIS AND CLINICAL FEATURES The DSM-5 diagnostic criteria for disruptive mood dysregulation disorder (Table. 31.12c1) requires outbursts that are grossly out of proportion to the situation. These temper outbursts present with verbal rages and/or physical aggression toward people or property, and are inappropriate for the child’s developmental level. Temper outbursts occur, on average, three or more times per week, with variations in mood between outbursts. Symptoms must exhibit before age 10 years, be present for at least 12 months, and be present within at least two settings (i.e., home and school). The diagnosis is not made for the first time in youth younger than 6 years or older than 18 years. In between temper outbursts, the child’s mood is persistently irritable and angry, and this mood is observable by others such as parents, teachers, or peers. There has never been period lasting more than one day in which full criteria for a manic or hypomanic episode (except for duration) are fulfilled. The above behaviors do not occur exclusively in the context of an episode of major depression and are not better accounted for by another psychiatric disorder. The DSM-5 diagnostic criteria for disruptive mood dysregulation disorder are found in Table 31.12c-1. Table 31.12c-1 DSM-5 Diagnostic Criteria for Disruptive Mood Dysregulation Disorder Daniel, a 12-year-old 7th grade boy was brought to his pediatrician by his mother, who was exasperated with Daniel’s rages and inappropriate tantrums. Daniel was on the floor in the waiting room, pounding his hands on the floor, yelling at his mother “get me out of here!” and crying. His mother had bruises on both legs from Dylan’s kicks, and she appeared distressed. Daniel’s mother walked into the office, leaving Daniel on the floor in the waiting room and burst into tears. “I can’t deal with him anymore.” She recounted the problems that Daniel had been having for the last 2 years: Severe recurrent tantrums four to five times/week. “He tantrums like a 6-yearold, and even when he is not having a tantrum, he is perpetually angry and irritable.” She reported that Daniel had lost all of his friends due to his short fuse and frequent verbal and physical outbursts. He was almost always irritable, even on his birthday. Daniel’s mother wonders whether there is anything physical wrong with him, but physical examination and routine blood tests reveal no abnormalities. Daniel’s tantrums had lessened somewhat last summer during the 2-month summer vacation; however, as soon as school resumed, he was back to consistent irritability. After an interview with Daniel, his pediatrician determined that he was not acutely suicidal; however, he required urgent psychotherapeutic intervention. Daniel was referred to a clinical psychologist for cognitive-behavioral treatment, and a child and adolescent psychiatrist for a medication evaluation. Daniel resisted psychotherapy; however, after several sessions, Daniel’s parents felt more hopeful than they had in a long time, and learned that Daniel’s problems were not “all their fault.” Daniel agreed to begin a trial of fluoxetine, which was titrated up to 30 mg over several weeks, and after about a month, it became clear that his irritability had diminished noticeably. Daniel still had many problems with peers, and he still had one or two tantrums per week; however, the tantrums were becoming less prolonged and less intense. Daniel seemed genuinely happy when he was invited to a classmate’s birthday party, and he was able to interact successfully with his peers during the party without any conflicts. Daniel continues to benefit from CBT, and he remains on fluoxetine 40 mg a day. Daniel is still described as a “temperamental” boy, but he is doing well in school, has rekindled several friendships, and is able to participate in family gatherings without a major tantrum. DIFFERENTIAL DIAGNOSIS Bipolar Disorder Disruptive mood dysregulation disorder closely resembles the “broad phenotype” of bipolar disorder. Although not episodic, it has been theorized by some clinicians and researchers that the chronic and persistent symptoms of mood disturbance and irritability may be an early developmental presentation of bipolar disorder. Disruptive mood dysregulation, however, does not meet formal diagnostic criteria for mania in bipolar disorder, because irritability in disruptive mood dysregulation disorder is chronic and nonepisodic. Oppositional Defiant Disorder Disruptive mood dysregulation disorder is similar to oppositional defiant disorder in that they both include irritability, temper outbursts, and anger. Many patients with disruptive mood dysregulation disorder meet the criteria for oppositional defiant disorder; however, most patients with oppositional defiant disorder do not meet the criteria for disruptive mood dysregulation disorder. Oppositional defiant disorder includes symptoms of annoyance and defiance that are not found in disruptive mood dysregulation disorder. Disruptive mood dysregulation disorder requires that irritable outbursts be present in at least two settings, whereas oppositional defiant disorder requires that they be present in only one setting. COURSE AND PROGNOSIS Disruptive mood dysregulation disorder is a chronic disorder. Longitudinal studies thus far have shown that patients with disruptive mood dysregulation disorder in childhood have a high risk of progressing to major depressive disorder, dysthymic disorder, and anxiety disorders over time. TREATMENT The current treatment of disruptive mood dysregulation is based on symptomatic interventions, in view of the fact that its etiology is not well understood at this time. If disruptive mood dysregulation disorder is confirmed to resemble unipolar depression and anxiety disorders in its pathophysiology, and it is often comorbid with ADHD, then SSRIs and stimulants would likely be the pharmacological agents of first choice. However, if the pathophysiology of disruptive mood dysregulation disorder is similar to that of bipolar disorder, then first-line treatments for youth would include atypical antipsychotic agents and mood stabilizers. There are scant treatment studies of disruptive mood dysregulation disorder in the current literature. One controlled trial of youths with symptoms of severe mood dysregulation and ADHD symptoms who did not respond to stimulants, responded to divalproex (Depakote) combined with behavioral psychotherapy compared to placebo and behavioral psychotherapy. There are treatment studies underway of youth who exhibit symptoms of severe mood dysregulation utilizing an SSRI plus a stimulant compared to a stimulant and placebo. Psychosocial interventions such as cognitive-behavioral psychotherapy are likely to be an essential component of treatment for youth with disruptive dysregulation disorder, and psychosocial interventions targeting children diagnosed with bipolar disorder may be beneficial. REFERENCES Blader JC, Schooler NR, Jensen PS, Pliszka SR, Kafantaris V. Adjunctive divalproex versus placebo for children with ADHD and aggression refractory to stimulant monotherapy. Am J Psychiatry. 2009;166:1392–1401. Brotman MA, Schmajuk M, Rich BA, Dickstein DP, Guyer AE, Costello EJ, Egger HL, Angold A, Pine DS, Leibenluft E. Prevalence, clinical correlates, and longitudinal course of severe mood dysregulation in children. Biol Psychiatry. 2006;60:991–997. Copeland WE, Angold A, Costello J, Egger H. Prevalence, comorbidity, and correlates of DSM-5 proposed disruptive mood dysregulation disorder. Am J Psychiatry. 2013;170:173. Fristad MA, Verducci JS. Walters K, Young ME. Impact of multifamily psychoeducational psychotherapy in treating children aged 8 to 12 years with mood disorder. Arch Gen Psychiatry. 2009;66:1013–1021. Leibenluft E. Severe mood dysregulation, irritability, and the diagnostic boundaries of bipolar disorder in youths. Am J Psychiatry. 2011;168:129. 32 - 31.12d Oppositional Defiant Disorder 31.12d Oppositional Defiant Disorder Leibenluft E, Cohen P, Gorrindo T, Brook JS, Pine DS. Chronic versus episodic irritability in youth: A community based longitudinal study of clinical and diagnostic associations. J Child Adolesc Psychopharmacol. 2006;16:456–466. Margulies DM, Weintraub S, Basile J, Grover PJ, Carlson GA. Will disruptive mood dysregulation disorder reduce false diagnosis of bipolar disorder in children? Bipolar Disord. 2012;14:488. Stringaris A, Barona A, Haimm C, Brotman MA, Lowe CH, Myers F, Rustgi E, Wheeler W, Kayser R, Towbin K, Leibenluft E. Pediatric bipolar disorder versus severe mood dysregulation: Risk for manic episodes on follow-up. J Am Acad Child Adolesc Psychiatry. 2010;49:397. Yearwood EL, Meadows-Oliver M. Mood dysregulation disorders. In: Yearwood EL, Pearson GS, Newland JA, eds. Child and Adolescent Behavioral Health: A Resource for Advance Practice Psychiatric and Primary Care Practitioners in Nursing. Hoboken, NJ: John Wiley & Sons Inc.; 2012:165. West Ae, Pavuluri MN. Psychosocial treatments for childhood and adolescent bipolar disorder. Child Adolesc Psychiatr Clin N Am. 2009;18:471–482. Yearwood EL, Meadows-Oliver M. Mood dysregulation disorders. In: Yearwood EL, Pearson GS, Newland JA, eds. Child and Adolescent Behavioral Health: A Resource for Advance Practice Psychiatric and Primary Care Practitioners in Nursing. Hoboken, NJ: John Wiley & Sons Inc.; 2012:165. Zonneyvlle-Bender MJ, Matthys W, van de Wiel NM, Lochman JE. Preventive effects of treatment of disruptive behavior disorder in middle childhood on substance use and delinquent behavior. J Am Acad Child Adolesc Psychiatry. 2007;46:33. 31.12d Oppositional Defiant Disorder Disruptive behaviors, especially oppositional patterns and aggressive behaviors, are among the most frequent reasons for children and adolescents to be referred for psychiatric evaluation. Demonstration of impulsive and oppositional behaviors are developmentally normative in young children; many youth who continue to display excessive patterns in middle childhood will find other forms of expression as they mature and will no longer demonstrate these behaviors in adolescence or adulthood. The origin of stable patterns of oppositional defiant behavior is widely accepted as a convergence of multiple contributing factors, including biological, temperamental, learned, and psychological conditions. Risk factors for the development of aggressive behavior in youth include childhood maltreatment such as physical or sexual abuse, neglect, emotional abuse, and overly harsh and punitive parenting. The American Psychiatric Association’s Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5), has divided oppositional defiant disorder into three types: Angry/Irritable Mood, Argumentative/Defiant Behavior, and Vindictiveness. A child may meet diagnostic criteria for oppositional defiant disorder with a 6-month pattern of at least four symptoms from the three types above. Angry/Irritable children with oppositional defiant disorder often lose their tempers, are easily annoyed, and feel irritable much of the time. Argumentative/Defiant children display a pattern of arguing with authority figures, and adults such as parents, teachers, and relatives. Children with this type of oppositional defiant disorder actively refuse to comply with requests, deliberately break rules, and purposely annoy others. These children often do not take responsibility for their actions, and often blame others for their misbehavior. Children with the Vindictive type of oppositional defiant disorder are spiteful, and have shown vindictive or spiteful actions at least twice in 6 months to meet diagnostic criteria. Oppositional defiant disorder is characterized by enduring patterns of negativistic, disobedient, and hostile behavior toward authority figures, as well as an inability to take responsibility for mistakes, leading to placing blame on others. Children with oppositional defiant disorder frequently argue with adults and become easily annoyed by others, leading to a state of anger and resentment. Children with oppositional defiant disorder may have difficulty in the classroom and with peer relationships, but generally do not resort to physical aggression or significantly destructive behavior. In contrast, children with conduct disorder engage in severe repeated acts of aggression that can cause physical harm to themselves and others and frequently violate the rights of others. In oppositional defiant disorder, a child’s temper outbursts, active refusal to comply with rules, and annoying behaviors exceed expectations for these behaviors for children of the same age. The disorder is an enduring pattern of negativistic, hostile, and defiant behaviors in the absence of serious violations of the rights of others. EPIDEMIOLOGY Oppositional and negativistic behavior, in moderation, is developmentally normal in early childhood and adolescence. Epidemiological studies of negativistic traits in nonclinical populations found such behavior in 16 to 22 percent of school-age children. Although oppositional defiant disorder can begin as early as 3 years of age, it typically is noted by 8 years of age and usually not later than early adolescence. Oppositional defiant disorder has been reported to occur at rates ranging from 2 to 16 percent with increased rates reported in boys before puberty, and an equal sex ratio reported after puberty. The prevalence of oppositional defiant behavior in males and females diminishes in youth older than 12 years of age. ETIOLOGY The most dramatic example of normal oppositional behavior peaks between 18 and 24 months, the “terrible twos,” when toddlers behave negativistically as an expression of growing autonomy. Pathology begins when this developmental phase persists abnormally, authority figures overreact, or oppositional behavior recurs considerably more frequently than in most children of the same mental age. Among the criteria included in oppositional defiant disorder, irritability appears to be the one most predictive of later psychiatric disorders, whereas the other elements may be considered components of temperament. Children exhibit a range of temperamental predispositions to strong will, strong preferences, or great assertiveness. Parents who model more extreme ways of expressing and enforcing their own will may contribute to the development of chronic struggles with their children that are then reenacted with other authority figures. What begins for an infant as an effort to establish self-determination may become transformed into an exaggerated behavioral pattern. In late childhood, environmental trauma, illness, or chronic incapacity, such as mental retardation, can trigger oppositionality as a defense against helplessness, anxiety, and loss of self-esteem. Another normative oppositional stage occurs in adolescence as an expression of the need to separate from the parents and to establish an autonomous identity. Classic psychoanalytic theory implicates unresolved conflicts as fueling defiant behaviors targeting authority figures. Behaviorists have observed that in children, oppositionality may be a reinforced, learned behavior through which a child exerts control over authority figures; for example, if having a temper tantrum when a request or demand is made of the child coerces the parents to withdraw their request, then tantrum behavior becomes strongly reinforced. In addition, increased parental attention during a tantrum can reinforce the behavior. DIAGNOSIS AND CLINICAL FEATURES Children with oppositional defiant disorder often argue with adults, lose their temper, and are angry, resentful, and easily annoyed by others at a level and frequency that is outside of the expected range for their age and developmental level. Frequently, youth with oppositional defiant disorder actively defy adults’ requests or rules and deliberately annoy other persons. They tend to blame others for their own mistakes and misbehavior, more often than is appropriate for their developmental age. Manifestations of the disorder are almost invariably present in the home, but they may not be present at school or with other adults or peers. In some cases, features of the disorder from the beginning of the disturbance are displayed outside the home; in other cases, the behavior starts in the home, but is later displayed outside. Typically, symptoms of the disorder are most evident in interactions with adults or peers whom the child knows well. Thus, a child with oppositional defiant disorder may not show signs of the disorder when examined clinically. Although children with oppositional defiant disorder may be aware that others disapprove of their behavior, they may still justify it as a response to unfair or unreasonable circumstances. The disorder appears to cause more distress to those around the child than to the child. Chronic oppositional defiant disorder or irritability almost always interferes with interpersonal relationships and school performance. These children are often rejected by peers, and may become isolated and lonely. Despite adequate intelligence, they may do poorly or fail in school, due to their lack of cooperation, poor participation, and inability to accept help. Secondary to these difficulties are low self-esteem, poor frustration tolerance, depressed mood, and temper outbursts. Adolescents who are ostracized may turn to alcohol and illegal substances as a modality to fit in with peers. Children who are chronically irritable often develop mood disorders in adolescence or adulthood. Pathology and Laboratory Examination No specific laboratory tests or pathological findings help diagnose oppositional defiant disorder. Because some children with oppositional defiant disorder become physically aggressive and violate the rights of others as they get older, they may share some characteristics with people with high levels of aggression, such as low central nervous system serotonin. DIFFERENTIAL DIAGNOSIS Oppositional behaviors are both normal and adaptive within an expected range at specific developmental stages. Periods of normative negativism must be distinguished from oppositional defiant disorder. Developmentally appropriate oppositional behavior is neither considerably more frequent nor more intense than that seen in other children of the same mental age. Oppositional defiant disorder must be distinguished from Disruptive Mood Dysregulation Disorder in so far as they are both characterized by chronic irritability and inappropriate temper outbursts. According to the DSM-5, oppositional defiant disorder cannot be diagnosed in the presence of disruptive mood dysregulation disorder. (See Section 31.12c for a further discussion of disruptive mood dysregulation disorder.) Oppositional defiant behavior occurring temporarily in reaction to a stressor should be diagnosed as an adjustment disorder. When features of oppositional defiant disorder appear during the course of conduct disorder, schizophrenia, or a mood disorder, the diagnosis of oppositional defiant disorder should not be made. Oppositional and negativistic behaviors can also be present in ADHD, cognitive disorders, and mental retardation. Whether a concomitant diagnosis of oppositional defiant disorder should be made depends on the severity, pervasiveness, and duration of such behavior. Some young children who receive a diagnosis of oppositional defiant disorder go on in several years to meet the criteria for conduct disorder. Some investigators believe that the two disorders may be developmental variants of each other, with conduct disorder being the natural progression of oppositional defiant behavior when a child matures. Most children with oppositional defiant disorder, however, do not later meet the criteria for conduct disorder, and up to one fourth of children with oppositional defiant disorder may not meet the diagnosis several years later. The subtype of oppositional defiant disorder that tends to progress to conduct disorder is one in which aggression is prominent, for example, the Angry/Irritable type and the Vindictive type. Many children who have ADHD and oppositional defiant disorder develop conduct disorder before the age of 12 years. Many children who develop conduct disorder have a history of oppositional defiant disorder. Overall, the current consensus is that two subtypes of oppositional defiant disorder may exist. One type, which is likely to progress to conduct disorder, includes certain symptoms of conduct disorder (e.g., fighting, bullying). The other type, which is characterized by less aggression and fewer antisocial traits, does not progress to conduct disorder. However, in either case, when both oppositional defiant disorder and conduct disorder are present, according to DSM-5, they may be diagnosed concurrently. Jackson, age 8 years, was brought to the clinic for evaluation of irritability, negativity and defiant behavior by his mother. She complained that he had frequent prolonged tantrums, triggered by not “getting his way.” Jackson’s mother described the tantrums as consisting of shouting, cursing, crying, slamming doors, and sometimes throwing books or objects on the floor. Jackson had been having troubles in school as well and his teacher had reported to the family that he seemed to have a habit of provoking other students as well as the teacher by making noises, rocking in his seat, and whistling in class. Recently, at home, Jackson was kicking his foot against his mother’s chair and she asked him to stop. He looked at her and continued to kick her chair until she became angry and sent him to his room. He then started yelling and stated that he wasn’t doing anything and that his mother was just picking on him. Jackson’s mother reports that she has given up on asking him to help with chores, because it inevitably results in an argument. Jackson appears sullen and irritable on interview. He insists that his problems are all his mother’s fault and she is always nagging him unfairly. During the interview with his mother, he interrupted her several times, to say that she was lying and to contradict her story. Despite Jackson’s behavioral problem he has been able to succeed academically and scores highly on standardized tests. His mother reports that Jackson used to have some friends in kindergarten, but as he has gotten older, he has lost almost all of his friends because he has difficulty sharing his things and tends to be bossy. Jackson’s mother reports that ever since his sister was born when he was 2 years old, he has been aggressive and rivalrous toward her. Jackson’s parents separated and divorced when he was 3. He has had no contact with his father since then. Jackson’s mother was depressed for a year after the divorce until she sought treatment. She has always felt guilty that his father is not in his life, and Jackson blames her for not having his father around. She believes his behaviors have become worse since she recently started dating again. COURSE AND PROGNOSIS The course of oppositional defiant disorder depends on the severity of the symptoms and the ability of the child to develop more adaptive responses to authority. The stability of oppositional defiant disorder varies over time, with approximately 25 percent of children with the disorder no longer meeting diagnostic criteria. Persistence of oppositional defiant symptoms poses an increased risk of additional disorders, such as mood disorders, conduct disorder and substance use disorders. Positive outcomes are more likely for intact families who can modify their own expression of demands and give less attention to the child’s argumentative behaviors. An association exists between oppositional defiant disorder and ADHD, as well as with mood disorders. In children who have a long history of aggression and oppositional defiant disorder, there is a greater risk of the development of conduct disorder and later substance use disorders. Parental psychopathology, such as antisocial personality disorder and substance abuse, appears to be more common in families with children who have oppositional defiant disorder than in the general population, which creates additional risks for chaotic and troubled home environments. The prognosis for oppositional defiant disorder in a child depends somewhat on family functioning and the development of comorbid psychopathology. TREATMENT The primary treatment of oppositional defiant disorder is family intervention using both direct training of the parents in child management skills and careful assessment of family interactions. The goals of this intervention are to reinforce more prosocial behaviors and to diminish undesired behaviors at the same time. Cognitive behavioral therapists emphasize teaching parents how to alter their behavior to discourage the child’s oppositional behavior by diminishing attention to it, and encourage appropriate therapy focuses on selectively reinforcing and praising appropriate behavior and ignoring or not reinforcing undesired behavior. Children with oppositional defiant behavior may also benefit from individual psychotherapy in which they role play and “practice” more adaptive responses. In the therapeutic relationship, the child can learn new strategies to develop a sense of mastery and success in social situations with peers and families. In the safety of a more “neutral” relationship, children may discover that they are capable of less provocative behavior. Often, self-esteem must be restored before a child with oppositional defiant disorder can make more positive responses to external control. Parent–child conflict strongly predicts conduct problems; patterns of harsh physical and verbal punishment particularly evoke the emergence of aggression in children. Replacing harsh, punitive parenting and increasing positive parent–child interactions may positively influence the course of oppositional and defiant behaviors. REFERENCES Boxer P, Huesmann LR, Bushman BJ, O’Brien M, Moceri D. The role of violent media preference in cumulative developmental risk for violence and general aggression. J Youth Adolesc. 2009;38:417–428. Canino G, Polanczyk G, Bauermeister JJ, Rhode LA, Frick P. Does the prevalence of CD and ODD vary across cultures? Soc Psychiatry Psychiatr Epidemiol. 2010;45:695–704. Correll CU, Kratochvil CJ, March J. Developments in pediatric psychopharmacology: Focus on stimulants, antidepressants, and antipsychotics. J Clin Psychiatry. 2011;72:655–670. Dodge KA & Conduct Problems Prevention Research Group. The effects of the Fast Track Preventive Intervention on the development of conduct disorder across childhood. Child Develop. 2011;82:331–345. Kim HW, Cho SC, Kim BN, Kim JW, Shin MS, Yeo JY. Does oppositional defiant disorder have temperament and psychopathological profiles independent of attention deficit/hyperactivity disorder? Compr Psychiatry. 2010;51:412– 418. LeBlanc JC, Binder CE, Armenteros JL, Aman MG, Want JS, Hew H, Kusumakar V. Risperidone reduces aggression in boys with a disruptive behavior disorder and below average intelligence quotient: Analysis of two placebo-controlled 33 - 31.12e Conduct Disorder 31.12e Conduct Disorder randomized trials. Int Clin Psychopharmacol. 2005;20:275. Lochman JE, Powell NP, Boxmeyer CL, Jimenez-Camargo L. Cognitive-behavioral therapy for externalizing disorders in children and adolescents. Child Adolesc Psychiatric Clin N Am. 2011;20:305–318. Patel NC, Crismon ML, Hoagwood K, Jensen PS. Unanswered questions regarding atypical antipsychotic use in aggressive children and adolescents. J Child Adolesc Psychopharmacol. 2005;15:270. Pelletier J, Collett B, Gimpel G, Crowley S. Assessment of disruptive behaviors in preschoolers: Psychometric Properties of the Disruptive Behavior Disorders Rating Scale and School Situations Questionnaire. J Psychoeduc Assess. 2006;24:3–18. Reyes M, Buitelaar J, Toren P, Augustyns I, Eerdekens M. A randomized, double-blind, placebo-controlled study of risperidone maintenance treatment in children and adolescents with disruptive behavior disorders. Am J Psychiatry. 2006;163:402–410. Rutter M. Research review: child psychiatric diagnosis and classification: Concepts, finding, challenges and potential. J Child Psychol and Psychiatry. 2011;52:647–660. Sasayam D, Hayashida A, Yamasue H, Yuzuru H, Kaneko T, Kasai K, Washizuka S, Amano N. Neuroanatomical correlates of attention-deficit-hyperactivity disorder accounting for comorbid oppositional defiant disorder and conduct disorder. Psychiatry Clin Neurosci. 2010:64:394–402. Santesso DL, Reker DL, Schmidt LA, Segalowitz SJ. Frontal electroencephalogram activation asymmetry, emotional intelligence, and externalizing behaviors in 10-year-old children. Child Psychiatr Hum Dev 2006;36:311–328. Van Huylle CA, Waldman ID, D’Onofrio BM, Rodgers JL, Rthouz PJ, Lahey BB. Developmental structure of genetic influences on antisocial behavior across childhood and adolescence. J Abnorm Psychol. 2009;118:711–734. Webster-Stratton C, Reid JM. The Incredible Years parents, teachers and children training series. In: Weisz JR, Kadin AE, eds. Evidence-based psychotherapies for children and adolescents. 2nd ed. New York: Guildford; 2010:194–210. Zuddas A, Zanni R, Usala T. Second generation antipsychotics (SGAs) for non-psychotic disorders in children and adolescents: A review of the randomized controlled studies. Eur Neuropsychopharmacol. 2011;21:600–620. 31.12e Conduct Disorder Aggressive patterns of behavior are among the most frequent reasons for children and adolescents to be referred for psychiatric intervention. Although demonstration of impulsive behaviors is developmentally normative in children, many youth who continue to display excessive patterns of aggression in middle childhood generally require intervention. Children who develop enduring patterns of aggressive behaviors that begin in early childhood and violate the basic rights of peers and family members, however, may be destined for an entrenched pattern of conduct disordered behaviors over time. Controversy remains as to whether a set of “voluntary” behaviors can constitute a valid psychiatric disorder, or may be better accounted for as maladaptive responses to adverse events, harsh or punitive parenting, or a threatening environment. Longitudinal studies have demonstrated that, for some youth, early patterns of disruptive behavior may become a lifelong pervasive repertoire culminating in adult antisocial personality disorder. The etiology of enduring patterns of aggressive behavior is widely accepted as a convergence of multiple contributing factors, including biological, temperamental, learned, and psychological conditions. Risk factors for the development of aggressive behavior in youth include childhood maltreatment such as physical or sexual abuse, neglect, emotional abuse, and overly harsh and punitive parenting. Chronic exposure to violence in the media including television, video games, and music videos has been shown to promote lower levels of empathy in children, which may add a risk factor for the development of aggressive behavior. Conduct disorder is an enduring set of behaviors in a child or adolescent that evolves over time, usually characterized by aggression and violation of the rights of others. Youth with conduct disorder often demonstrate behaviors in the following four categories: physical aggression or threats of harm to people, destruction of their own property or that of others, theft or acts of deceit, and frequent violation of ageappropriate rules. Conduct disorder is associated with many other psychiatric disorders including ADHD, depression, and learning disorders. It is also associated with certain psychosocial factors, including childhood maltreatment, harsh or punitive parenting, family discord, lack of appropriate parental supervision, lack of social competence, and low socioeconomic level. The American Psychiatric Association’s DSM-5 criteria require three persistent specific behaviors of 15 conduct disorder symptoms listed, over the past 12 months, with at least one of them present in the past 6 months (Table 31.12e-1). Conduct disorder symptoms include bullying, threatening, or intimidating others, and staying out at night despite parental prohibition. DSM-5 also specifies that when truancy from school is a symptom, it begins before 13 years of age. The disorder may be diagnosed in a person older than 18 years only if the criteria for antisocial personality disorder are not met. DSM-5 includes specifiers denoting the severity of the disorder, including “mild” in which there are few conduct problems in excess of those needed to make the diagnosis and behaviors cause only minor harm to others. In “moderate” cases, symptoms exceed the minimum; however, there is less confrontation that may cause harm to individuals than in “severe” cases. According to DSM-5, the “severe” level shows many conduct problems in excess of the minimal diagnostic criteria or conduct problems that cause considerable harm to others. DSM-5 has also added the following specifier: “With limited prosocial emotions.” To qualify for this specifier, the individual must show a persistent interpersonal and emotional pattern that can be characterized by at least two of the following: (1) Lack of remorse or guilt, (2) callous lack of empathy, (3) unconcerned about performance, (4) shallow or deficient affect. Individuals with conduct disorder who qualify for this specifier are more likely to have childhood-onset type and meet the criteria for a “severe” disorder. Children with conduct disorder engage in severe repeated acts of aggression that can cause physical harm to themselves and others and frequently violate the rights of others. Children with conduct disorder usually have behaviors characterized by aggression to persons or animals, destruction of property, deceitfulness or theft, and multiple violations of rules, such as truancy from school. These behavior patterns cause distinct difficulties in school life as well as in peer relationships. Conduct disorder has been divided into three subtypes, based on the age of onset of the disorder. Childhood-onset subtype, in which at least one symptom has emerged repeatedly before age 10 years; Adolescent-onset type, in which no characteristic persistent symptoms were seen until after age 10 years; and Unspecifiedonset, in which age of onset is unknown. Although some young children show persistent patterns of behavior consistent with violating the rights of others or destroying property, the diagnosis of conduct disorder in children appears to increase with age. Epidemiological surveys indicate that geographic locations representing a broad range of different cultures are not associated with significant variability in prevalence rates of either oppositional defiant disorder or conduct disorder. A longitudinal study of population density and antisocial behaviors in youth found no relationship in children 4 to 13 years of age between conduct problems and density of living area. However, higher rates of conduct problems were self-reported by youth 10 years to 17 years who lived in higher-density communities. Table 31.12e-1 DSM-5 Diagnostic Criteria for Conduct Disorder EPIDEMIOLOGY Estimated prevalence rates of conduct disorder in the United States range from 6 to 16 percent for males, and from 2 to 9 percent for females. Ratio of conduct disorder in males compared to females ranges from 4:1 to as much as 12:1. Conduct disorder occurs with greater frequency in the children of parents with antisocial personality disorder and alcohol dependence than in the general population. The prevalence of conduct disorder and antisocial behavior is associated with socioeconomic factors, as well as parental psychopathology. ETIOLOGY A meta-analysis of longitudinal studies indicates that the most important risk factors that predict conduct disorder include impulsivity, physical or sexual abuse or neglect, poor parental supervision and harsh and punitive parental discipline, low intelligence quotient (IQ), and poor school achievement. Parental Factors Harsh, punitive parenting characterized by severe physical and verbal aggression is associated with the development of children’s maladaptive aggressive behaviors. Chaotic home conditions are associated with conduct disorder and delinquency. Divorce itself is not necessarily a risk factor, but the persistence of hostility, resentment, and bitterness between divorced parents may be the more important contributor to maladaptive behavior. Parental psychopathology, child abuse, and negligence often contribute to conduct disorder. Sociopathy, alcohol dependence, and substance abuse in the parents are associated with conduct disorder in their children. Parents may be so negligent that a child’s care is shared by relatives or assumed by foster parents. Many such parents were scarred by their own upbringing and tend to be abusive, negligent, or engrossed in getting their own personal needs met. Studies indicate that parents of children with conduct disorder have high rates of serious psychopathology, including psychotic disorders. Data shows that children who exhibit a pattern of aggressive behavior have frequently been exposed to physically or emotionally harsh parenting. Genetic Factors A study of more than 6,000 male, female, and opposite sex twins found that genetic and environmental factors accounted for proportionally the same amount of variance in males and females. Genetic, and/or shared environmental factors exert different effects on males and females in childhood conduct disorder, but by adulthood, the genderspecific influences on antisocial behavior are no longer apparent. The sex-specific effects on antisocial behavior in youth along with the replicated finding of a potential role for the X-linked monoamine oxidase A gene in the etiology of antisocial behavior leads to the need for further genetic investigation of conduct disorder on the X chromosome and for analyses of these behaviors to be done separately by gender. Sociocultural Factors Youth residing in geographic areas with greater population density report increased rates of aggression and delinquency. Unemployed parents, lack of a supportive social network, and lack of positive participation in community activities seem to predict conduct disorder. Associated findings that may influence the development of conduct disorder in urban areas are increased exposure to and prevalence of substance use. A survey of alcohol use and mental health in adolescents found that weekly alcohol use among adolescents is associated with increased delinquent and aggressive behavior. Significant interactions between frequent alcohol use and age indicated that those adolescents with weekly alcohol use at younger ages were most likely to exhibit aggressive behaviors and mood disorders. Although drug and alcohol use does not cause conduct disorder, it increases the risks associated with it. Drug intoxication itself can also aggravate the symptoms. Thus, all factors that increase the likelihood of regular substance use may, in fact, promote and expand the disorder. Psychological Factors Poor emotion regulation among youth is associated with higher rates of aggression and conduct disorder. Emotion regulation is associated with social competence and can be observed even in children of preschool age. Those children with greater degrees of emotion dysregulation exhibit higher levels of aggression. Poor modeling of impulse control and the chronic lack of having their own needs met leads to a less welldeveloped sense of empathy. Neurobiological Factors Neuroimaging studies utilizing MRI have used voxel-based morphometry methods to compare structural brain differences between children with conduct disorder compared to normal controls. Studies have reported that children with conduct disorder had decreased gray matter in limbic brain structures, and in the bilateral anterior insula and left amygdala compared to healthy controls. A study investigated structural brain differences in children comorbid for oppositional defiant disorder or conduct disorder and ADHD compared to those with ADHD alone, and normal controls. Findings included decreased gray matter in ADHD and ADHD comorbid for oppositional defiant disorder or conduct disorder compared to controls in regions including bilateral temporal and occipital cortices, and the left amygdala. Neurotransmitter studies in children with conduct disorder, suggest low level of plasma dopamine β-hydroxylase, an enzyme that converts dopamine to norepinephrine, leading to a hypothesis of decreased noradrenergic functioning in conduct disorder. Other studies of conduct-disordered juvenile offenders have found high plasma serotonin levels in blood. Evidence indicates that blood serotonin levels correlate inversely with levels of 5-HIAA in the cerebrospinal fluid (CSF) and that low 5-HIAA levels in CSF correlate with aggression and violence. Neurologic Factors An electroencephalography (EEG) study investigating resting frontal brain electrical activity, emotional intelligence, aggression, and rule breaking in 10-year-old children found that aggressive children had significantly greater relative right frontal brain activity at rest compared with nonaggressive children. Frontal resting brain electrical activity has been hypothesized to reflect the ability to regulate emotion. Boys tended to show lower emotional intelligence than girls and greater aggressive behavior than girls. No relationship, however, was found between emotional intelligence and pattern of frontal EEG activation. Child Abuse and Maltreatment Evidence shows that children chronically exposed to violence, physical or sexual abuse, and neglect, particularly at a young age, are at high risk for demonstrating aggression. A study of female caregivers exposed to intimate partner violence revealed a strong association with offspring aggression and mood disturbance. Severely abused children and adolescents tend to be hypervigilant; in some cases, they misperceive benign situations as directly threatening and respond defensively with violence. Not all expressed aggressive behavior in adolescents is synonymous with conduct disorder; however, youth with a repetitive pattern of hypervigilance and violent responses are likely to violate the rights of others. Comorbid Factors ADHD and conduct disorder are often found to coexist, with ADHD often predating the development of conduct disorder, and not infrequently substance abuse. Central nervous system injury, dysfunction, or damage predispose a child to impulsivity and behavioral disturbances, which sometimes evolve into conduct disorder. DIAGNOSIS AND CLINICAL FEATURES Conduct disorder does not develop overnight, instead, many symptoms evolve over time until a consistent pattern develops that involves violating the rights of others. Very young children are unlikely to meet the criteria for the disorder, because they are not developmentally able to exhibit the symptoms typical of older children with conduct disorder. A 3-year-old does not break into someone’s home, steal with confrontation, force someone into sexual activity, or deliberately use a weapon that can cause serious harm. School-age children, however, can become bullies, initiate physical fights, destroy property, or set fires. The DSM-5 diagnostic criteria for conduct disorder are given in Table 31.12e-1. The average age of onset of conduct disorder is younger in boys than in girls. Boys most commonly meet the diagnostic criteria by 10 to 12 years of age, whereas girls often reach 14 to 16 years of age before the criteria are met. Children who meet the criteria for conduct disorder express their overt aggressive behavior in various forms. Aggressive antisocial behavior can take the form of bullying, physical aggression, and cruel behavior toward peers. Children may be hostile, verbally abusive, impudent, defiant, and negativistic toward adults. Persistent lying, frequent truancy, and vandalism are common. In severe cases, destructiveness, stealing, and physical violence often occur. Some adolescents with conduct disorder make little attempt to conceal their antisocial behavior. Sexual behavior and regular use of tobacco, liquor, or illicit psychoactive substances begin unusually early for such children and adolescents. Suicidal thoughts, gestures, and acts are frequent in children and adolescents with conduct disorder who are in conflict with peers, family members, or the law and are unable to problem solve their difficulties. Some children with aggressive behavioral patterns have impaired social attachments, as evinced by their difficulties with peer relationships. Some may befriend a much older or younger person or have superficial relationships with other antisocial youngsters. Many children with conduct problems have poor self-esteem, although they may project an image of toughness. They may lack the skills to communicate in socially acceptable ways and appear to have little regard for the feelings, wishes, and welfare of others. Children and adolescents with conduct disorders often feel guilt or remorse for some of their behaviors, but try to blame others to stay out of trouble. Many children and adolescents with conduct disorder suffer from the deprivation of having few of their dependency needs met and may have had either overly harsh parenting or a lack of appropriate supervision. The deficient socialization of many children and adolescents with conduct disorder can be expressed in physical violation of others and, for some, in sexual violation of others. Severe punishments for behavior in children with conduct disorder almost invariably increases their maladaptive expression of rage and frustration rather than ameliorating the problem. In evaluation interviews, children with aggressive conduct disorders are typically uncooperative, hostile, and provocative. Some have a superficial charm and compliance until they are urged to talk about their problem behaviors. Then, they often deny any problems. If the interviewer persists, the child may attempt to justify misbehavior or become suspicious and angry about the source of the examiner’s information and perhaps bolt from the room. Most often, the child becomes angry with the examiner and expresses resentment of the examination with open belligerence or sullen withdrawal. Their hostility is not limited to adult authority figures, but is expressed with equal venom toward their age-mates and younger children. In fact, they often bully those who are smaller and weaker. By boasting, lying, and expressing little interest in a listener’s responses, such children reveal their lack of trust in adults to understand their position. Evaluation of the family situation often reveals severe marital disharmony, which initially may center on disagreements about management of the child. Because of a tendency toward family instability, parent surrogates are often in the picture. Children with conduct disorder are more likely to be unplanned or unwanted babies. The parents of children with conduct disorder, especially the father, have higher rates of antisocial personality disorder or alcohol dependence. Aggressive children and their family show a stereotyped pattern of impulsive and unpredictable verbal and physical hostility. A child’s aggressive behavior rarely seems directed toward any definable goal and offers little pleasure, success, or even sustained advantages with peers or authority figures. In other cases, conduct disorder includes repeated truancy, vandalism, and serious physical aggression or assault against others by a gang, such as mugging, gang fighting, and beating. Children who become part of a gang usually have the skills for ageappropriate friendships. They are likely to show concern for the welfare of their friends or their own gang members and are unlikely to blame them or inform on them. In most cases, gang members have a history of adequate or even excessive conformity during early childhood that ended when the youngster became a member of the delinquent peer group, usually in preadolescence or during adolescence. Also present in the history is some evidence of early problems, such as marginal or poor school performance, mild behavior problems, anxiety, and depressive symptoms. Some family social or psychological pathology is usually evident. Patterns of paternal discipline are rarely ideal and can vary from harshness and excessive strictness to inconsistency or relative absence of supervision and control. The mother has often protected the child from the consequences of early mild misbehavior, but does not seem to encourage delinquency actively. Delinquency, also called juvenile delinquency, is most often associated with conduct disorder but can also result from other psychological or neurological disorders. Violent Video Games and Violent Behavior Longitudinal studies corroborate the contribution of media violence including video gaming in middle-school children with the expression of aggression in those adolescents. A review of the literature of the effect of violent video games on children and adolescents revealed that violent video game playing is related to aggressive affect, physiologic arousal, and aggressive behaviors. It stands to reason that the degree of exposure to violent games and the more restriction of activity would be related to a greater preoccupation with violent themes. PATHOLOGY AND LABORATORY EXAMINATION No specific laboratory test or neurological pathology helps make the diagnosis of conduct disorder. Some evidence indicates that amounts of certain neurotransmitters, such as serotonin in the CNS, are low in some persons with a history of violent or aggressive behavior toward others or themselves. Whether this association is related to the cause, or is the effect, of violence or is unrelated to the violence is not clear. DIFFERENTIAL DIAGNOSIS Disturbances of conduct, including impulsivity and aggression, may occur in many childhood psychiatric disorders, ranging from ADHD, to oppositional defiant disorder, to disruptive mood dysregulation disorder mood disorder, to major depression, to bipolar disorder, specific learning disorders, and psychotic disorders. Therefore, clinicians must obtain a comprehensive history of the chronology of the symptoms to determine whether the conduct disturbance is a transient or an enduring pattern. Isolated acts of aggressive behavior do not justify a diagnosis of conduct disorder; an entrenched pattern must be present. The relationship of conduct disorder to oppositional defiant disorder is still under debate. Historically, oppositional defiant disorder has been conceptualized as a mild precursor of conduct disorder, without the violation of rights, likely to be diagnosed in younger children who may be at risk for conduct disorder. Children who progress from oppositional defiant disorder to conduct disorder over time, maintain their oppositional characteristics, and some evidence indicates that the two disorders are independent. Currently, in the DSM-5, oppositional defiant disorder and conduct disorder are considered distinct, and they may be diagnosed comorbidly. Many children with oppositional defiant disorder do not develop conduct disorder, and conduct disorder emerging in adolescence is not necessarily preceded by oppositional defiant disorder. The main distinguishing clinical feature between these two disorders is that in conduct disorder, the basic rights of others are violated, whereas in oppositional defiant disorder, hostility and negativism fall short of seriously violating the rights of others. Mood disorders are often present in children who exhibit irritability and aggressive behavior. Both major depressive disorder and bipolar disorders must be ruled out, but the full syndrome of conduct disorder can occur and be diagnosed during the onset of a mood disorder. Substantial comorbidity exists of conduct disorder and depressive disorders. A recent report concludes that the high correlation between the two disorders arises from shared risk factors for both disorders rather than a causal relation. Thus, a series of factors, including family conflict, negative life events, early history of conduct disturbance, level of parental involvement, and affiliation with delinquent peers, contribute to the development of affective disorders and conduct disorder. This is not the case with oppositional defiant disorder, which cannot be diagnosed if it occurs exclusively during a mood disorder. ADHD and learning disorders are commonly associated with conduct disorder. Usually, the symptoms of these disorders predate the diagnosis of conduct disorder. Substance abuse disorders are also more common in adolescents with conduct disorder than in the general population. Evidence indicates an association between fighting behaviors as a child and substance use as an adolescent. Once a pattern of drug use is formed, this pattern may interfere with the development of positive mediators, such as social skills and problem-solving, which could enhance remission of the conduct disorder. Thus, once substance abuse develops, it may promote continuation of the conduct disorder. Obsessive-compulsive disorder also frequently seems to coexist with disruptive behavior disorders. All the disorders described here should be noted when they co-occur. Children with ADHD often exhibit impulsive and aggressive behaviors that may not meet the full criteria for conduct disorder. Damien, age 12 years, was referred for psychiatric evaluation after being picked up by police for truancy, and running away from home. Damien explained that he just wanted to get out of his house and go see his friends. He doesn’t like to be at home because his mother tries to tell him what to do. Damien’s mother says that he left and stayed out overnight multiple times in the past year, but that he usually returns the next morning. She complains that he is constantly in trouble. He has shoplifted on several occasions that she knows of, the first time at age 8 years. She suspects that he also steals from neighbors or school. The police have been involved on many occasions including truancy, staying out all night, stealing from a neighborhood store, and smoking marijuana. Damien has a quick temper, and his mother knows he was involved in several fights over the past year in the neighborhood. Damien is particularly cruel to his younger brother, constantly taunting and teasing him. Damien’s mother stated that he lies constantly, sometimes for no apparent reason. When he was 6 years of age, he was fascinated with fire and set several small fires at home, fortunately with no serious injury or damage. Damien’s mother was tearful when she disclosed that Damien is just like his no-good father and that she wished she never had him. Damien initially refused to answer questions, and turned away scowling, but gradually began to talk. Damien presented a tough image with an indifferent attitude toward the interviewer. Damien denied any abuse at home, saying that he ran off because he was bored. However, upon further questioning, Damien admitted that his mother’s previous boyfriend who was in the home when Damien was between 6 and 8 years of age used to hit him with a belt when he got out of line. Damien justified his own behaviors as just having fun. He explained the fights as being provoked by the others and denied the use of any weapons, although he bragged about breaking the nose of another youth. Damien’s school records indicate that an Individualized Educational Plan (IEP) was required when he was in the 2nd grade, and he was evaluated for symptoms of ADHD when he was in 1st grade. Methylphenidate (Ritalin) was prescribed; however, the family did not continue with treatment, and he is currently on no medication. Damien is currently in 6th grade special education classes, having failed and repeated 5th grade. Damien’s grades are failing, and he may have to repeat 6th grade. Damien admits to truancy on several occasions this year in addition to his problems with completing schoolwork. His previous evaluation indicates that child protective services evaluated the family for possible neglect when he was 5 years of age after he and his brother were found barefoot on the street late one evening without his mother in sight. Apparently, Damien’s family was referred for counseling and never attended. Both of Damien’s parents have a history of drug and alcohol abuse. Damien’s birth was unplanned, and his mother used drugs during pregnancy. His parents separated soon after his birth, and his mother returned to live with her parents briefly. Damien and his mother moved to live with her boyfriend when Damien was 1 year of age after she became pregnant with his younger sister. Damien’s mother’s relationship ended within a year, and only Damien, his mother, and his sister live in their apartment. Damien’s mother has worked several different jobs, and Damien wonders if she has a drinking problem. COURSE AND PROGNOSIS The course and prognosis for children with conduct disorder is most guarded in those who have symptoms at a young age, exhibit the greatest number of symptoms, and the most severe, and express them most frequently. This finding is true partly because those with severe conduct disorder seem to be most vulnerable to comorbid disorders later in life, such as mood disorders and substance use disorders. A longitudinal study found that, although assaultive behavior in childhood and parental criminality predict a high risk for incarceration later in life, the diagnosis of conduct disorder per se was not correlated with imprisonment. The best prognosis is predicted for mild conduct disorder in the absence of coexisting psychopathology and the presence of normal intellectual functioning. TREATMENT Psychosocial Interventions Early sustained preventive interventions can significantly alter the course and prognosis of aggressive behavior when it is administered starting at kindergarten age. A screening program used with kindergarteners predicted lifetime disruptive behavior disorder by age 18 years, with the highest risk group demonstrating an 82 percent chance of a disruptive behavior diagnosis without intervention. A prevention program, the Fast Track Preventive Intervention, randomized 891 kindergarteners to either a 10-year prevention program or a control condition. The 10-year intervention included parent behavior management, child social cognitive skills, reading, home visiting, mentoring, and classroom curricula. The children in the Fast Track Intervention were substantially prevented from the development of conduct disorder during the 10-year period and for 2 years thereafter. A meta-analysis of controlled trials of CBT programs indicates that CBT can result in significant reductions in conduct-disordered symptoms in children and adolescents. CBT treatment interventions that are proven to be efficacious include the following. Kazdin’s Problem-Solving Skills Training (PSST) in which a 12-week sequential program helps children develop problem-solving solutions when faced with conflictual situations. Assignments called “supersolvers” provide vignette situations in which children can practice these techniques. A companion program, Parent Management Training (PMT) can be added to the intervention, but PSST can be effective even without the parent component. Another CBT-based intervention, the Incredible Years (IY), targeting young children from 3 to 8 years, is administered over 22 weeks and delivers sessions to the child and has a parent training component and a teacher training. Another CBT-based intervention is the Anger Coping Program, an 18-session intervention for school-aged children in the grades 4 to 6 focused on a child’s increased development of emotion recognition and regulation, and managing anger. Anger coping strategies include distraction, self-talk, perspective taking, goal setting, and problem solving. Overall, treatment programs have been more successful in decreasing overt symptoms of conduct, such as aggression, than the covert symptoms, such as lying or stealing. Treatment strategies for young children that focus on increasing social behavior and social competence are believed to reduce aggressive behavior. A study of 548 third graders administered a school-based intervention instead of a regular health curriculum in several public schools in North Carolina, called Making Choices: Social Problem Solving Skills for Children (MC) program along with supplemental teacher and parent components. Compared with third graders receiving the routine health curriculum, children exposed to the MC program were rated lower on the posttest social and overt aggression, and higher on social competence. In addition, they scored higher on an information-processing skills posttest. These findings support the notion that schoolbased prevention programs have the potential to strengthen social and emotional skills and diminish aggressive behavior among normal populations of school-age children. School settings can also use behavioral techniques to promote socially acceptable behavior toward peers and to discourage covert antisocial incidents. Psychopharmacologic Interventions Efficacy of psychopharmacologic interventions includes several placebo-controlled studies of risperidone for aggression in youth associated with disruptive behavior disorders, and/or mental retardation. In addition, risperidone has been found to be superior to placebo in reducing aggressive behavior in a large 6-month placebosubstitution study. One randomized double-blind placebo-controlled trial with quetiapine also showed efficacy for aggressive behavior. Early studies of antipsychotics, most notably haloperidol (Haldol), reported decreased aggressive and assaultive behaviors in children with a variety of psychiatric disorders. Atypical antipsychotics risperidone (Risperdal), olanzapine (Zyprexa), quetiapine (Seroquel), ziprasidone (Geodon), and aripiprazole (Abilify) have generally replaced the older antipsychotics in clinical practice due to their comparable efficacy and improved side effect profiles. Side effects of second-generation antipsychotics include sedation, increased prolactin levels, (with risperidone use) and extrapyramidal symptoms, including akathisia. In general, however, the atypical antipsychotics appear to be well tolerated. A study of divalproex in youth with conduct disorder showed that those who responded most robustly exhibited aggression characterized by agitation, dysphoria, and distress. Although early trials suggested that carbamazepine (Tegretol) was useful to control aggression, a doubleblind, placebo-controlled study did not show superiority of carbamazepine over placebo in decreasing aggression. A pilot study found that clonidine (Catapres) may decrease aggression. The SSRIs, including fluoxetine (Prozac), sertraline (Zoloft), paroxetine (Paxil), and citalopram (Celexa), are used clinically to target symptoms of impulsivity, irritability, and mood lability, which frequently accompany conduct disorder. Conduct disorder often coexists with ADHD, learning disorders, and, over time, mood disorders and substance-related disorders; thus, the treatment of concurrent disorders must also be addressed. 34 - 31.13 Anxiety Disorders of Infancy, Childhood 31.13 Anxiety Disorders of Infancy, Childhood, and Adolescence REFERENCES Boxer P, Huesmann LR, Bushman BJ, O’Brien M, Moceri D. The role of violent media preference in cumulative developmental risk for violence and general aggression. J Youth Adolesc. 2009;38:417–428. Canino G, Polanczyk G, Bauermeister JJ, Rhode LA, Frick P. Does the prevalence of conduct disorder and ODD vary across cultures? Soc Psychiatry Psychiatr Epidemiol. 2010;45:695–704. Correll CU, Kratochvil CJ, March J. Developments in pediatric psychopharmacology: Focus on stimulants, antidepressants, and antipsychotics. J Clin Psychiatry. 2011;72:655–670. Dodge KA & Conduct Problems Prevention Research Group. The effects of the Fast Track Preventive Intervention on the development of conduct disorder across childhood. Child Develop. 2011;82:331–345. Harden KP, D’Onofrio BM, Van Hulle C, Turkheimer E, Rodgers JL, Waldman ID, Lahey BB. Population density and youth antisocial behavior. J Child Psychol and Psychiatry. 2009;50:999–1008. Huebner T, Vloet TD, Marx I, Konrad K, Fink GR, Herpetz SC, Herpetz-Dahlmann B. Morphometric brain abnormalities in boys with conduct disorder. J Am Acad Child Adolesc Psychiatry. 2008;47:540–547. Lochman JE, Powell NP, Boxmeyer CL, Jimenez-Camargo L. Cognitive-behavioral therapy for externalizing disorders in children and adolescents. Child Adolesc Psychiatr Clin N Am. 2011;20:305–318. Meier MH, Slutske WS, Heath AC, Martin NG. Sex differences in the genetic and environmental influences on childhood conduct disorder and adult antisocial behavior. J Abnorm Psychol. 2011;120:377–388. Murray J, Farrington DP. Risk factors for conduct disorder and delinquency: Key findings from longitudinal studies. Can J Psychiatry. 2010;55:633–642. Padhy R, Saxena K, Remsing L, Heumer J, Plattner B, Steiner H. Symptomatic response to divalproex in subtypes of conduct disorder. Child Psychiatry Hum Dev. 2011;42:584–593. Reyes M, Buitelaar J, Toren P, Augustyns I, Eerdekens M. A randomized, double-blind, placebo-controlled study of risperidone maintenance treatment in children and adolescents with disruptive behavior disorders. Am J Psychiatry. 2006;163:402–410. Rutter M. Research review: child psychiatric diagnosis and classification: Concepts, findings, challenges and potential. J Child Psychol and Psychiatry. 2011;52:647–660. Sasayam D, Hayashida A, Yamasue H, Yuzuru H, Kaneko T, Kasai K, Washizuka S, Amano N. Neuroanatomical correlates of attention-deficit-hyperactivity disorder accounting for comorbid oppositional defiant disorder and conduct disorder. Psychiatry Clin Neurosci. 2010:64:394–402. Santesso DL, Reker DL, Schmidt LA, Segalowitz SJ. Frontal electroencephalogram activation asymmetry, emotional intelligence, and externalizing behaviors in 10-year-old children. Child Psychiatr Hum Dev 2006;36:311–328. Van Huylle CA, Waldman ID, D’Onofrio BM, Rodgers JL, Rthouz PJ, Lahey BB. Developmental structure of genetic influences on antisocial behavior across childhood and adolescence. J Abnorm Psychol. 2009;118:711–734. Zahrt DM, Melzer-Lange MD. Aggressive behavior in children and adolescents. Pediatr Rev. 2011;32:325–331. Zuddas A, Zanni R, Usala T. Second generation antipsychotics (SGAs) for non-psychotic disorders in children and adolescents: A review of the randomized controlled studies. Eur Neuropsychopharmacol. 2011;21:600–620. 31.13 Anxiety Disorders of Infancy, Childhood, and Adolescence Anxiety disorders are among the most common disorders in youth, affecting 10 to 20 percent of children and adolescents. Although observable anxiety behaviors mark 35 - 31.13a Separation Anxiety Disorder, Generaliz 31.13a Separation Anxiety Disorder, Generalized Anxiety Disorder, and Social Anxiety Disorder (Social Phobia) normative development in infants, anxiety disorders in childhood predict a wide range of psychological difficulties in adolescence including additional anxiety disorders, panic attacks, and depressive disorders. Fear is an expected response to real or perceived threat; however, anxiety is the anticipation of future danger. Anxiety disorders are characterized by recurrent emotional and physiological arousal in response to excessive perceptions of perceived threat or danger. Anxiety disorders commonly found in youth include separation anxiety disorder, generalized anxiety disorder, social anxiety disorder, and selective mutism. Anxiety is classified into disorders based on how it is experienced, the situations that trigger it, and the course that it tends to follow. 31.13a Separation Anxiety Disorder, Generalized Anxiety Disorder, and Social Anxiety Disorder (Social Phobia) Separation anxiety disorder, generalized anxiety disorder, and social anxiety disorder in children are often considered together in the evaluation process and differential diagnosis, and in developing treatment strategies, because they are highly comorbid and have overlapping symptoms. A child with separation anxiety disorder, generalized anxiety disorder, or social anxiety disorder has a 60 percent chance of having at least one of the other two disorders as well. Of children with one of the above anxiety disorders, 30 percent have all three of them. Children and adolescents may also have additional comorbid anxiety disorders such as specific phobia or panic disorder. Separation anxiety disorder, generalized anxiety disorder, and social anxiety disorder are distinguished from each other by the types of situations that elicit the excessive anxiety and avoidance behaviors. SEPARATION ANXIETY DISORDER Separation anxiety is a universal human developmental phenomenon emerging in infants younger than 1 year of age and marking a child’s awareness of a separation from his or her mother or primary caregiver. Normative separation anxiety peaks between 9 months and 18 months and diminishes by about 2½ years of age, enabling young children to develop a sense of comfort away from their parents in preschool. Separation anxiety or stranger anxiety most likely evolved as a human response that has survival value. The expression of transient separation anxiety is also normal in young children entering school for the first time. Approximately 15 percent of young children display intense and persistent fear, shyness, and social withdrawal when faced with unfamiliar settings and people. Young children with this pattern of significant behavioral inhibition are at higher risk for the development of separation anxiety disorder, generalized anxiety disorder, and social phobia. Behaviorally inhibited children, as a group, exhibit characteristic physiological traits, including higher than average resting heart rates, higher morning cortisol levels than average, and low heart rate variability. Separation anxiety disorder is diagnosed when developmentally inappropriate and excessive anxiety emerges related to separation from the major attachment figure. According to the American Psychiatric Association’s Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5), separation anxiety disorder is characterized by a level of fear or anxiety regarding separation from their parents or primary caregiver, which is beyond developmental expectations. Furthermore, there may be a pervasive worry that harm will come to a parent upon separation, which leads to extreme distress, and sometimes nightmares. The DSM-5 requires the presence of at least three symptoms related to excessive worry about separation from a major attachment figure for a period of at least 4 weeks. The worries often take the form of refusal to go to school, fears and distress on separation, repeated complaints of physical symptoms such as headaches and stomachaches when separation is anticipated, and nightmares related to separation issues. GENERALIZED ANXIETY DISORDER Children with generalized anxiety disorder have significant distress in activities of daily life often focused on the child’s fears of incompetence in many areas, including school performance and in social settings. In addition, children with generalized anxiety disorder, according to DSM-5, experience at least one of the following symptoms: restlessness, being easily fatigued, “mind going blank,” irritability, muscle tension, or sleep disturbance. Children with generalized anxiety disorder tend to feel fearful in multiple settings and expect more negative outcomes when faced with academic or social challenges, compared with peers. Children and adolescents with generalized anxiety disorder may experience symptoms of autonomic hyperarousal such as tachycardia, shortness of breath, or dizziness, and are more likely than nonanxious youth to experience sweating, nausea, or diarrhea when they become anxious. Children and adolescents with generalized anxiety disorder tend to be overly concerned about potential natural disasters such as earthquakes or floods, and these worries can interfere with their daily activities. Finally, children and adolescents with generalized anxiety disorder are continuously worried about the quality of their performance in academics, sports, and other activities, and often seek excessive reassurance about their performance. SOCIAL ANXIETY DISORDER (SOCIAL PHOBIA) Children who experience intense discomfort and distress in social situations and are impaired by their fear of scrutiny or humiliation are given the diagnosis of social anxiety disorder. Their distress may be expressed in the form of crying, tantrums, avoidance, freezing, or even becoming “mute” in these situations. According to DSM-5, this disorder is characterized by consistent anxiety and distress in almost all social situations. Any situation in which the child feels exposed to possible scrutiny by others can provoke fear or anxiety, and the child will often try to avoid these feared social situations. Children must experience the anxiety in the presence of peers, not only with adults, in order to receive the diagnosis. A child or adolescent with social anxiety disorder may exhibit the performance only type, which targets a specific type of performing, such as fear of public speaking. The performance only type typically manifests in school or academic settings in which public presentations must be performed, such as in front of classmates in school. Social anxiety disorder has significant implications for future accomplishments, since it is associated with lower levels of satisfaction in leisure activities, increased rates of school dropout, less productivity in the workplace as adults, and increased rates of remaining single. Despite the significant impairment caused by social anxiety disorder, up to half of individuals with the disorder do not receive treatment. EPIDEMIOLOGY The prevalence of anxiety disorders has varied with the age group of the children surveyed and the diagnostic instruments used. Lifetime prevalence of any anxiety disorder in children and adolescents ranges from 10 percent to 27 percent. Anxiety disorders are common in preschoolers as well, and follow a similar epidemiologic profile as in older children. An epidemiologic survey using the Preschool Age Psychiatric Assessment (PAPA) found that 9.5 percent of preschoolers met criteria for any anxiety disorder, with 6.5 percent exhibiting generalized anxiety disorder, 2.4 percent meeting criteria for separation anxiety disorder, and 2.2 percent meeting criteria for social phobia. Separation anxiety disorder is estimated to be about 4 percent in children and young adolescents. Separation anxiety disorder is more common in young children than in adolescents and has been reported to occur equally in boys and girls. The onset may occur during preschool years, but is most common in children 7 to 8 years of age. The rate of generalized anxiety disorder in school-age children is estimated to be approximately 3 percent, the rate of social phobia is 1 percent, and the rate of simple phobias is 2.4 percent. In adolescents, lifetime prevalence for panic disorder was found to be 0.6 percent; the prevalence for generalized anxiety disorder was 3.7 percent. ETIOLOGY Biopsychosocial Factors Evidence for the influences of parental psychopathology and parenting styles on the emergence of anxiety disorders in childhood has been found in multiple investigations. Longitudinal studies have found that parental overprotection has been associated with an increased risk of the development of anxiety disorders in children, and insecure parent–child attachment is associated with higher than expected rates of anxiety disorders in childhood. It is also well known that maternal depression and anxiety have led to an increased risk for anxiety and depression in children. Psychosocial factors in conjunction with a child’s temperament influences the degree of separation anxiety evoked in situations of brief separation and exposure to unfamiliar environments. The temperamental trait of shyness and withdrawal in unfamiliar situations has been shown to be associated with a higher risk of developing separation anxiety disorder, generalized anxiety disorder, social anxiety disorder, or all three during childhood and adolescence. External life stresses often coincide with development of the disorder. The death of a relative, a child’s illness, a change in a child’s environment, or a move to a new neighborhood or school is frequently noted in the histories of children with separation anxiety disorder. In a vulnerable child, these changes probably intensify anxiety. Neurophysiological correlations are found with behavioral inhibition (extreme shyness); children with this constellation are shown to have a higher resting heart rate and an acceleration of heart rate with tasks requiring cognitive concentration. Additional physiological correlates of behavioral inhibition include elevated salivary cortisol levels, elevated urinary catecholamine levels, and greater papillary dilation during cognitive tasks. Neuroimaging studies of adolescents with anxiety show an increased activation of the amygdala compared to non-anxious adolescents when presented with anxiety-provoking stimuli. Furthermore, anxious adolescents maintain the hyperactivation of the amygdala over time, rather than showing an attenuation of the effect as in nonanxious adolescents. Structural studies of the amygdala in adolescents with anxiety have led to conflicting results, some studies finding increased amygdala volumes, whereas other studies finding decreased amygdala volumes. Social Learning Factors Fear, in response to a variety of unfamiliar or unexpected situations, may be unwittingly communicated from parents to children by direct modeling. If a parent is fearful, the child will probably have a phobic adaptation to new situations, especially to a school environment. There are much data to suggest that overprotective parenting promotes increased interpersonal sensitivity in healthy children, and increases the risk of social anxiety disorder in children with behavioral inhibition or other anxiety disorders such as separation anxiety disorder. Some parents appear to teach their children to be anxious by overprotecting them from expected dangers or by exaggerating the dangers. For example, a parent who cringes in a room during a lightning storm teaches a child to do the same. A parent who is afraid of mice or insects conveys the affect of fright to a child. Conversely, a parent who becomes angry with a child when the child expresses fear of a given situation, for example, when exposed to animals, may promote a phobic concern in the child by exposing the child to the intensity of the anger expressed by the parent. Social learning factors in the development of anxiety reactions are magnified when parents have anxiety disorders themselves. These factors may be pertinent in the development of separation anxiety disorder as well as in generalized anxiety disorder and social phobia. A recent study found no association between psychosocial hardships, such as ongoing family conflict, and behavioral inhibition among young children. It appears that temperamental predisposition to anxiety disorders emerges as a highly heritable constellation of traits, and is not created by psychosocial stressor. Genetic Factors Genetic studies suggest that genes account for at least one third of the variance in the development of anxiety disorders. Heritability for anxiety disorders in children and adolescents ranges from 36 percent to 65 percent, with the highest estimates found in younger children with anxiety disorders. Two heritable characteristics—behavioral inhibition (the tendency toward fear and withdrawal in new situations) and physiological hyperarousal—have both been found to impart significant risk factors for future development of an anxiety disorder. However, although the temperamental constellation of behavioral inhibition, excessive shyness, the tendency to withdraw from unfamiliar situations, and the eventual emergence of anxiety disorders have a genetic contribution, one third to two thirds of young children with behavioral inhibition do not appear to go on to develop anxiety disorders. Family studies have shown that the offspring of adults with anxiety disorders are at an increased risk of having an anxiety disorder themselves. Separation anxiety disorder and depression in children overlap, and the presence of an anxiety disorder increases the risk of a future episode of a depressive disorder. Current consensus on the genetics of anxiety disorders suggests that what is inherited is a general predisposition toward anxiety, causing heightened levels of arousal, emotional reactivity, and increased negative affect, all of which increase the risk of developing separation anxiety disorder, generalized anxiety disorder, and social phobia. DIAGNOSIS AND CLINICAL FEATURES Separation anxiety disorder, generalized anxiety disorder, and social phobia are highly related in children and adolescence because, in most children, overlapping symptoms as well as comorbid disorders emerge. Generalized anxiety disorder is the most common anxiety disorder among youth, more common in adolescents than in younger children; in almost one third of these cases, a child with generalized anxiety disorder also exhibits separation anxiety disorder and social anxiety disorder. Diagnostic criteria for separation anxiety disorder, according to the DSM-5, include three of the following symptoms for at least 4 weeks: persistent and excessive worry about losing, or possible harm befalling, major attachment figures; persistent and excessive worry that an untoward event can lead to separation from a major attachment figure; persistent reluctance or refusal to go to school or elsewhere because of fear of separation; persistent and excessive fear or reluctance to be alone or without major attachment figures at home or without significant adults in other settings; persistent reluctance or refusal to go to sleep without being near a major attachment figure or to sleep away from home; repeated nightmares involving the theme of separation; repeated complaints of physical symptoms, including headaches and stomachaches, when separation from major attachment figures is anticipated; and recurrent excessive distress when separation from home or major attachment figures is anticipated or involved. The following case history demonstrates separation anxiety disorder along with autonomic arousal symptoms. Jake was a 9-year-old boy who was referred for outpatient evaluation by his family physician. He refused to sleep in his room alone at night and exhibited violent tantrums each morning in order to avoid going to school. Jake expressed recurrent fears that something bad would happen to his mother. He worried that she would get into a car accident or that there would be a fire at home and his mother would be killed. Developmental history revealed that Jake was anxious and irritable as an infant and toddler. He had trouble adjusting to babysitters in the preschool years. There was a history of panic disorder, with agoraphobia in the mother and major depression in his father. Jake became more concerned and territorial over his mother when his father left the family, and his mother became depressed. Jake always kept track of his mother’s whereabouts and insisted that she stay at home. Nighttime was a particularly difficult time at home. When Jake’s mother tried to get Jake to remain in his room, Jake would whine and cry and insist that his mother lie in bed with him until he fell asleep. He also expected his mother to be in the master bedroom across the hall from his room throughout the evening. Jake’s mother reported that that each evening her son would get up and peek through the crack in the master bedroom door, as frequently as every 10 minutes, to be certain that she was still there. Jake reported frequent nightmares that his mother was killed and that monsters prevented him from rescuing his mother, taking him away from his family forever. During the daytime, Jake would shadow his mother around the house. Jake would agree to play a game with his sister in the lower level of the house only if his mother was close by. When Jake’s mother went upstairs, he would interrupt the game and follow her upstairs. He refused to sleep at a friend’s house. Frequently, at home as the evening progressed, Jake described a queasy sensation in his stomach mixed with feelings of sadness. On school days, Jake usually complained of stomachaches and tried to stay home. Jake appeared distressed and panicky and would become violent when his mother attempted to drop him off at school. Once at school, he seemed calmer and less distressed, but frequently was seen in the nurse’s office, complaining of nausea and seeking to be sent home. (Adapted from case material from Gail A. Bernstein, M.D. and Anne E. Layne, Ph.D.) The essential feature of separation anxiety disorder is extreme anxiety precipitated by separation from parents, home, or other familiar surroundings, whereas in generalized anxiety disorder, fears are extended to negative outcomes for all kinds of events, including academic, peer relationship, and family activities. In generalized anxiety disorder, a child or adolescent experiences at least one recurrent physiological symptom, such as restlessness, poor concentration, irritability, or muscle tension. In social phobia, the child’s fears peak during performance situations involving exposure to unfamiliar people or situations. Children and adolescents with social phobia have extreme concerns about being embarrassed, humiliated, or negatively judged. In each of the preceding anxiety disorders, the child’s experience can approach terror or panic. The distress is greater than that normally expected for the child’s developmental level and cannot be explained by any other disorder. Morbid fears, preoccupations, and ruminations characterize separation anxiety disorder. Children with anxiety disorders overestimate the probability of danger and the likelihood of negative outcome. Children with separation anxiety disorder and generalized anxiety disorder become overly fearful that someone close to them will be hurt or that something terrible will happen to them or their families, especially when they are away from important caring figures. Many children with anxiety disorders are preoccupied with health and worry that their families or friends will become ill. Fears of getting lost, being kidnapped, and losing the ability to be in contact with their families is predominant among children with separation anxiety disorder. Adolescents with anxiety disorders may not directly express their worries; however, their behavior patterns often reflect either separation anxiety or other anxiety if they exhibit discomfort about leaving home, engage in solitary activities because of fears about how they will perform in front of peers, or have distress when away from their families. Separation anxiety disorder in children is often manifested at the thought of travel or in the course of travel away from home. Children may refuse to go to camp, a new school, or even a friend’s house. Frequently, a continuum exists between mild anticipatory anxiety before separation from an important figure and pervasive anxiety after the separation has occurred. Premonitory signs include irritability, difficulty eating, whining, staying in a room alone, clinging to parents, and following a parent everywhere. Often, when a family moves, a child displays separation anxiety by intense clinging to the mother figure. Sometimes, geographical relocation anxiety is expressed in feelings of acute homesickness or psychophysiological symptoms that break out when the child is away from home or is going to a new country. The child yearns to return home and becomes preoccupied with fantasies of how much better the old home was. Integration into the new life situation may become extremely difficult. Children with anxiety disorders may retreat from social or group activities and express feelings of loneliness because of their self-imposed isolation. Sleep difficulties are frequent in children and adolescents with any anxiety disorder or in severe separation anxiety; a child or adolescent may require having someone remain with him or her until he or she falls asleep. An anxious child may awaken and go to a parent’s bed or even sleep at the parents’ door in an effort to diminish anxiety. Nightmares and morbid fears may be expressions of anxiety (Fig. 31.13a-1). FIGURE 31.13a-1 This surrealistic photograph symbolically represents the anxiety in a childhood nightmare. (Courtesy of Arthur Tress for Magnum Photos, Inc.) Associated features of most anxiety disorders include fear of the dark and imaginary worries. Children may have the feeling that eyes are staring at them and monsters are reaching out for them in their bedrooms. Children with separation anxiety disorder, generalized anxiety disorder, and social anxiety disorder often complain of somatic symptoms and may be more sensitive to changes in their bodies compared to youth without anxiety disorders. Children with separation anxiety disorder, generalized anxiety disorder, or social anxiety disorder are often more emotionally sensitive than peers and more easily brought to tears. Frequent somatic complaints accompanying anxiety disorders include gastrointestinal symptoms, nausea, vomiting, and stomachaches; unexplained pain in various parts of the body; sore throats; and flu-like symptoms. Older children and adolescents typically complain of somatic experiences classically reported by adults with anxiety, such as cardiovascular and respiratory symptoms—palpitations, dizziness, faintness, and feelings of strangulation. Physiological signs of anxiety are a part of the diagnostic criteria for generalized anxiety disorder, but they are more often also experienced by children with separation anxiety and social phobia than the general population. The following case history demonstrates a young adolescent with generalized anxiety disorder. Rachel was a 13-year-old girl referred for an evaluation by her pediatrician based on her chronic gastrointestinal complaints without any organic illness. On interview, Rachel appeared withdrawn and meek but responsive to questions. She endorsed a number of worries that included concerns about her health, her parents’ safety, her school performance, and her peer relationships. Rachel’s greatest worries were related to her health and safety. Rachel’s mother reported that Julie had recently been very reluctant to play outside, because she feared she would contract Lyme disease from a tick bite or West Nile virus from a mosquito bite. Rachel was also very distressed by news reports about catastrophic events locally and around the world (e.g., kidnapping, crime, terrorism). Rachel was described by her family and teachers as overly conscientious about her schoolwork and as often being concerned about adult matters (e.g., finances, parents’ job security). Symptoms that accompanied Rachel’s worries primarily involved stomach pain and problems falling asleep. Rachel tended to be quite perseverative; repetitively verbalizing her worries even after reassurance was given. Rachel admitted that she worried for hours each day and could not “turn off” her worried thoughts. Rachel was the product of a normal pregnancy and delivery. Her medical history was unremarkable, with the exception of frequent gastrointestinal pain since kindergarten. Julie was described as irritable and difficult to soothe as an infant. Developmental milestones were met within normal limits. She was described as very obedient and had no history of externalizing behavior problems. She was very concerned about her academic performance from an early age and earned A’s with an occasional B. Rachel was somewhat shy in social situations but well-liked by her peers. Family history included depression in her maternal grandmother and a maternal history of generalized anxiety disorder, social anxiety, and separation anxiety disorder as a child. Rachel had two younger siblings who were high achievers and without notable problems. (Adapted from case material from Gail A. Bernstein, M.D., and Ann E. Layne, Ph.D.) The next case history demonstrates an adolescent with multiple anxiety and depressive disorders. Kate is a 15-year-old 10th grader who lives with her biological parents and two sisters, age 9 and 14 years. Kate is a very articulate teen who has always been a good student, although she never volunteers answers in school unless she is called on by her teachers. She gets along well with her sisters when at home, but ever since she entered high school in the 9th grade year, she declines invitations to go to friends’ homes, has turned down opportunities to go to parties, and has even stopped going on outings with her sisters to the neighborhood mall and the movies. Kate reports that she gets too nervous, and blushes when she is with friends outside of the classroom at school because she can’t think of anything to say to them. She reports that she is embarrassed to go shopping or to the movies with her sisters because they often run into neighborhood peers along the way, stop to chat, and this makes her feel “stupid,” because even though she is the oldest, she does not say anything, and believes that her sisters’ friends will laugh at her shyness. Recently, one of her former best friends confronted her about why she had stopped “hanging out” with her friends. Kate had stopped eating lunch with her friends in school because she felt humiliated when they would talk about their weekend plans and even when they invited her to join, she would just look the other way and ignore the conversation. Kate had become isolated, even in school, and admitted to her sister that she was lonely. Kate was brought for an evaluation after her younger sister commented to her mother that Kate spent all of her time alone whenever her sisters saw their friends, and that she looked sad and stressed out whenever she was around peers. Kate was down, always in poor spirits and had stopped interacting with her sisters even at home, and her sisters were often out with their own friends. On rare occasions Kate’s younger sister had invited Kate to parties or to friend’s homes, but Kate had declined and burst into tears. Kate was evaluated by a child psychiatrist who made the diagnoses of social anxiety disorder, generalized anxiety disorder, and major depression and recommended a combination of treatment options, including cognitive-behavioral therapy (CBT) and a trial of a selective serotonin reuptake inhibitor (SSRI), fluoxetine. Kate and her family decided to try the medication first. Kate was started on 10 mg of fluoxetine and over the next month was titrated to a dose of 20 mg. By the third week of the medication trial, Kate was noticeably less resistant to going out with her sisters to places where they were likely to encounter peers. Her sisters noticed that she did not seem as stressed and started to occasionally sit with peers at lunch in the school cafeteria. She stated that she did not feel as self-conscious as she used to in class and was willing to go to a friend’s house. She still declined to go to a birthday party of a peer that she didn’t know very well. Kate continued on the same medication and within 2 months, she was significantly less anxious in social situations. She complained occasionally of a stomachache, but tolerated the medication well. Her family was impressed when she requested they plan a birthday party for her 16th birthday and decided to invite 10 friends. Pathology and Laboratory Examination No specific laboratory measures help in the diagnosis of separation anxiety disorder, generalized anxiety disorder, or social anxiety disorder. DIFFERENTIAL DIAGNOSIS The presence of separation anxiety is a developmentally expected feature in a young child and often does not represent an impairing condition, thus clinical judgment must be used in distinguishing normal anxiety from separation anxiety disorder in this age group. In older school-age children, a child experiencing more than normal distress is apparent when school is refused on a regular basis. For children who resist school, it is important to distinguish whether fear of separation, general worry about performance, or more specific fears of humiliation in front of peers or the teacher are driving the resistance. In many cases in which anxiety is the primary symptom, all three of the above-feared scenarios come into play. In generalized anxiety disorder, anxiety is not primarily focused on separation. When depressive disorders occur in children, possible comorbidities such as separation anxiety disorder should be evaluated as well. A comorbid diagnosis of separation anxiety disorder and depressive disorder should be made when the criteria for both disorders are met; the two diagnoses often coexist. Panic disorder with agoraphobia is uncommon before 18 years of age; the fear is of being incapacitated by a panic attack rather than of separation from parental figures. School refusal is a frequent symptom in separation anxiety disorder, but is not pathognomonic of it. Children with other diagnoses, such as specific phobias, or social anxiety disorder, or fear of failure in school because of learning disorder, may also lead to school refusal. When school refusal occurs in an adolescent, the severity of the dysfunction is generally greater than when it emerges in a young child. Similar and distinguishing characteristics of childhood separation anxiety disorder, generalized anxiety disorder, and social anxiety disorder are presented in Table 31.13a-1. Table 31.13a-1 Common Characteristics in Childhood Anxiety Disorders COURSE AND PROGNOSIS The course and the prognosis of separation anxiety disorder, generalized anxiety disorder, and social anxiety disorder are varied and are related to the age of onset, the duration of the symptoms, and the development of comorbid anxiety and depressive disorders. Young children who can maintain attendance in school, after-school activities, and peer relationships generally have a better prognosis than children or adolescents who refuse to attend school and withdraw from social activities. The large multisite randomized clinical trial Child/Adolescent Anxiety Multimodal Study (CAMS) provided acute treatment for children and adolescents with one or more anxiety disorders with sertraline medication alone, cognitive-behavior therapy (CBT) alone, or both together, and found that predictors of future remission included younger age of initiation of treatment, lower severity of anxiety, absence of a comorbid depressive or anxiety disorder, and the absence of social anxiety disorder as the primary anxiety disorder being treated. A follow-up study of children and adolescents with mixed anxiety disorders over a 3-year period reported that up to 82 percent no longer met criteria for the anxiety disorder at follow-up. Of the group followed, 96 percent of those with separation anxiety disorder were remitted at follow-up. Most children who recovered did so within the first year. Early age of onset and later age at diagnosis were factors in this study that predicted slower recovery. Close to one third of the group studied, however, had developed another psychiatric disorder within the follow-up period, and 50 percent of these children developed another anxiety disorder. Studies have shown a significant overlap between separation anxiety disorder and depressive disorders. In cases with multiple comorbidities, the prognosis is more guarded. Longitudinal data indicate that some children with severe school refusal continue to resist attending school into adolescence and remain impaired for many years. TREATMENT The treatment of child and adolescent separation anxiety disorder, generalized anxiety disorder, and social anxiety disorder are often considered together, given the frequent comorbidity and overlapping symptomatology of these disorders. A multimodal comprehensive treatment approach usually includes psychotherapy, most often CBT, family education, family psychosocial intervention, and pharmacological interventions, such as SSRIs. The best evidence-based treatments for childhood anxiety disorders include CBT and SSRIs. The comparative efficacy of CBT, SSRI medication, and their combination (CBT + SSRI) in the treatment of childhood anxiety disorders was investigated in the National Institute of Mental Health (NIMH)–funded Child/Adolescent Anxiety Multimodal Study (CAMS). This double-blind, placebo-controlled, multi-site study included 488 children and adolescents with separation anxiety disorder, generalized anxiety disorder, or social anxiety disorder, who were randomly assigned to be treated with either CBT alone, SSRI medication (sertraline) alone, both CBT and sertraline, or placebo. After an acute treatment phase of 12 weeks, those in the combined CBT + sertraline group had an 80.7 percent response rate of much or very much improved on the clinical global improvement (CGI) rating. Response rates for the CBT–only and sertraline-only groups were 59.7 percent and 54.9 percent, respectively. Placebo response was 23.7 percent. Over time, during open follow-up, the combination of CBT plus sertraline continued to provide the most efficacy. All three treatments— CBT, sertraline, and their combination—were superior to placebo, and thus effective treatments in childhood anxiety, but combined treatment was most likely to help children and adolescents with anxiety disorders. A trial of CBT may be applied first, if available, when a child is able to function sufficiently to engage in daily activities while obtaining this treatment. For a child with severe impairment, however, a combination of treatments is recommended. BT is widely accepted as first-line evidence-based treatment for childhood anxiety disorders. A meta-analysis reviewed 16 randomized controlled trials of CBT for childhood anxiety disorders and found CBT to be consistently superior to a wait-list control group or a psychological placebo group. Exposure-based CBT has received the most empiric support among psychotherapeutic interventions for anxiety disorders in youth and has been shown to be superior to wait-list control groups in reducing impairment and symptoms of anxiety. Several psychosocial interventions have been designed specifically for anxiety disorders in young children. A randomized clinical trial of CBT for 4- to 7-year-old children was administered via a manualized intervention called “Being Brave: A Program for Coping with Anxiety for Young Children and their Parents.” This manual was loosely modeled after the manualized Coping Cat program. The intervention utilized a combination of parent-only sessions and child-and-parent sessions. Response rate, measured as much or very much improved on the Clinical Global Improvement Scale for Anxiety, was 69 percent among completers versus 32 percent of the wait-list controls. The treated children showed significantly better CGI improvement on social anxiety disorder, separation anxiety disorder, and specific phobia, but not on generalized anxiety disorder. This treatment, a developmentally modified parent–child CBT, shows promise in young children. Coaching Approach behavior and Leading by Modeling (the CALM program) is an intervention aimed at treating anxiety disorders in children younger than 7 years of age, who are too young to effectively engage in traditional CBT. The CALM program draws on previous work with children aged 2 to 7 years through interventions that target a child’s undesired behavior by modifying parents’ behavior, called Parent-Child Interaction Therapy (PCIT). The CALM program is a 12-session manual-based intervention that provides live, individualized coaching via a bug-in-the-ear receiver worn by the parent during sessions. It incorporates exposure tasks and promotes “brave” behavior with parent coaching. A pilot study using the CALM program with nine patients with a mean age of 5.4 years found that all treatment completers (seven patients and families) were rated as global responders, and all but one showed functional improvement. Adapting the PCIT model for anxiety disorders in young children appears to be a promising approach to treating anxiety in early childhood. A meta-analysis of randomized controlled trials of antidepressant agents for childhood anxiety provides evidence that multiple SSRIs, including fluvoxamine (Luvox), fluoxetine (Prozac), sertraline (Zoloft), and paroxetine (Paxil) are efficacious in the treatment of childhood anxiety. Based on this evidence, SSRIs are the first choice of medication in the treatment of anxiety disorders in children and adolescents. A large, multisite investigation by the National Institute of Mental Health (Research Units in Pediatric Psychopharmacology [RUPP]) confirmed the safety and efficacy of fluvoxamine in the treatment of childhood separation anxiety disorder, generalized anxiety disorder, and social phobia. This double-blind, placebo controlled study of 128 children and adolescents revealed that 76 percent of children in the group treated with fluvoxamine showed significant improvement compared with 29 percent of those in the placebo group. Response to medication was noticeable after only two weeks of treatment. Fluvoxamine dosages ranged from 50 mg to 250 mg per day in children and up to 300 mg per day in adolescents. Children and adolescents with less comorbid depressive symptoms had the best response. Children and adolescents who responded to this medication were continued on fluvoxamine for a period of 6 months, and almost all of them continued to be responders at the 6-month mark. Several other randomized clinical trials have also supported the efficacy of SSRIs in the treatment of child and adolescent anxiety disorders. A randomized, controlled trial found fluoxetine, at a dose of 20 mg per day, to be safe and effective for children with these disorders, with minor side effects including gastrointestinal distress, headache, and drowsiness. In addition, a randomized clinical trial for the treatment of generalized anxiety disorder in children lends support for the efficacy of sertraline (Zoloft). Finally, a large industry randomized clinical trial of paroxetine (Paxil) in the treatment of children with social phobia found that paroxetine was associated with response in 78 percent of children treated. Paroxetine was utilized at a dosage range of 10 to 50 mg per day. The Food and Drug Administration (FDA) has placed a “black box” warning on antidepressants, including all of the SSRI agents, used in the treatment of any childhood disorder, because of concerns about increased suicidality; however, no individual childhood anxiety study has found a statistically significant increase in suicidal thoughts or behaviors. Tricyclic drugs are not currently recommended due to their potentially serious cardiac adverse effects. β-Adrenergic receptor antagonists, such as propranolol (Inderal), and buspirone (BuSpar) have been used clinically in children with anxiety disorders, but currently no data support their efficacy. Diphenhydramine (Benadryl) may be used in the short term to control sleep disturbances in children with anxiety disorders. Open trials and one double-blind, placebo-controlled study suggested that alprazolam (Xanax), a benzodiazepine, may help to control anxiety symptoms in separation anxiety disorder. Clonazepam (Klonopin) has been studied in open trials and may be useful in controlling symptoms of panic and other anxiety symptoms. Although SSRIs and CBT alone and in combination have demonstrated efficacy in the treatment of anxiety disorders in youth, approximately 20 to 35 percent of children and adolescents with anxiety disorders do not appear to benefit. Several novel agents have been suggested as potential treatments, some based on their effect on the N-methyl-daspartate (NMDA) system. For example, d-cycloserine (DCS), currently FDA approved in the treatment of pediatric tuberculosis, is a partial receptor agonist of the NMDA system and is hypothesized to augment the benefits of exposure treatment for phobias. Some evidence suggests that DCS may increase the speed of exposure interventions; however, long-term gains have not been proven. Riluzole is an antiglutamatergic agent that decreases glutamatergic transmission by inhibiting glutamate release and inactivation of sodium channels in cortical neurons, and blocking γ-aminobutyric acid (GABA) reuptake. Due to its antiglutamatergic effects, Riluzole has been postulated to provide augmentation in the treatment of obsessive-compulsive disorder and generalized anxiety disorder. Another agent, memantine, an NMDA receptor antagonist, with FDA approval in the treatment of Alzheimer’s disease, has been hypothesized to decrease anxiety due to its influence on the glutamatergic system. Published case reports have provided mixed results. Although most childhood anxiety disorders wax and wane over time, school refusal associated with separation anxiety disorder can be viewed as a psychiatric emergency. A comprehensive treatment plan involves the child, the parents, and the child’s peers and school. Family interventions are critical in the management of separation anxiety disorder, especially in children who refuse to attend school, so that firm encouragement of school attendance is maintained while appropriate support is also provided. When a return to a full school day is overwhelming, a program should be arranged so the child can progressively increase the time spent at school. Graded contact with an object of anxiety is a form of behavior modification that can be applied to any type of separation anxiety. Some severe cases of school refusal require hospitalization. Cognitivebehavioral modalities include exposure to feared separations and cognitive strategies, such as coping self-statements aimed at increasing a sense of autonomy and mastery. In summary, evidence-based treatments for anxiety disorders have focused SSRIs and CBT. SSRIs have been shown to be both safe and efficacious in the treatment of childhood anxiety disorders; however, in severe disorders, the evidence suggests that optimal treatment is to provide both CBT and SSRI antidepressant agents simultaneously. REFERENCES Bittner A, Egger HL, Erkanli A. What do childhood anxiety disorders predict? J Child Psychol Psychiatry. 2007;48:1174– 1183. Comer JS, Puliafico AC, Ascenbrand SG, McKnight K, Robin JA, Goldfine ME, Albano AM. A pilot feasibility evaluation of the CALM Program for anxiety disorders in early childhood. J Anxiety Disord. 2012;26:40–49. Compton SN, Walkup JT, Albano AM, Piacentini JC, Birmaher B, Sherrill JT, Ginsburg GS, Rynn MA, McCracken JT, Waslick BD, Iyengar S, Kendall PC, March JS. Child/Adolescent Anxiety Multimodal Study (CAMS): Rationale, design, and methods. Child Adolesc Psychiatry Ment Health. 2010;4:1. Connolly SC, Suarez L, Sylvester C. Assessment and treatment of anxiety disorders in children and adolescents. Curr Psychiatry Rep. 2011;13:99–110. Davis III TE, May A, Whiting SE. Evidence-based treatment of anxiety and phobia in children and adolescents: Current status and effects on the emotional response. Clin Psychol Rev. 2011;31:592–602. Ginsburg GS, Kendall PC, Sakolsky D, Compton SN, Piacentini J, Albano AM, Walkup JT, Sherrill J, Coffey KA, Rynn MA, Keeton CP, McCracken JT, Bergman L, Iyengar S, Birmaher B, March J. Remission after acute treatment in children and adolescents with anxiety disorders: findings from The CAMS. J Consult Clin Psychol. 2011;79: 806–813. 36 - 31.13b Selective Mutism 31.13b Selective Mutism Hanna GL, Fischer DJ, Fluent TE. Separation anxiety disorder and school refusal in children and adolescents. Pediatr Rev. 2006;27:56–63. Hirshfeld-Becker DR, Masek B, Henin A, Blakely LR, Pollock-Wurman RA, McQuade J, DePetrillo L, Briesch J, Ollendick TH, Rosenbaum JF, Biederman J. Cognitive behavioral therapy for 4- to 7-year-old children with anxiety disorders: A randomized clinical trial. J Consult Clin Psychol. 2010;78:498–510. Otani K, Suzuki A, Matsumoto Y, Kamata. Parental overprotection increases interpersonal sensitivity in healthy subjects. Comp Psychiatry. 2009;50:54–57. Reinblatt SP, Walkup JT. Psychopharmacologic treatment of pediatric anxiety disorders. Child Adolesc Psychiatric Clin N Am. 2005;14:877. Rockhill C, Kodish I, DiBassisto C, Macias M, Varley C, Ryan S. Anxiety disorders in children and adolescents. Curr Prob Pediatr Adolesc Health Care. 2010;0:66–99. Rynn M, Puliafico A, Heleniak C, Rikhi P, Ghalib K, Vidair H. Advances in pharmacotherapy for pediatric anxiety disorders. Depress Anxiety. 2011;28:76–87. Schneider S, Blatter-Meunier J, Herren C, Adornetto C, In-Albon T, Lavallee K. Disorder-specific cognitive-behavioral therapy for separation anxiety disorder in young children: A randomized waiting-list–controlled group. Psychother Psychosom. 2011;80:206–215. Vanderwerker LC, Jacobs SC, Parkes CM, Prigerson HG. An exploration of associations between separation anxiety in childhood and complicated grief in later life. J Nerv Ment Dis. 2006;194(2):121–123. Walkup JT, Albano AM, Piacentini J. Cognitive behavioral therapy, sertraline, or a combination in childhood anxiety. N Engl J Med. 2008;359:2753–2766. 31.13b Selective Mutism Selective mutism, believed to be related to social anxiety disorder, although an independent disorder, is characterized in a child by persistent lack of speaking in one or more specific social situations, most typically, the school setting. A child with selective mutism may remain completely silent or near silent, in some cases only whispering in a school setting. Although selective mutism often begins before age 5 years, it may not be apparent until the child is expected to speak or read aloud in school. Current conceptualization of selective mutism highlights a convergence of underlying social anxiety, along with an increased likelihood of speech and language problems leading to the failure to speak in certain situations. Typically, children with the disorder are silent during stressful situations, whereas some may verbalize almost inaudibly single-syllable words. Despite an increased risk for delayed speech and language acquisition in children with selective mutism, children with this disorder are fully capable of speaking competently when not in a socially anxiety-producing situation. Some children with the disorder will communicate with eye contact or nonverbal gestures but not verbally when at school. Otherwise, children with selective mutism speak fluently at home and in many familiar settings. Selective mutism is believed to be related to social anxiety disorder because of its expression primarily in selective social situations. EPIDEMIOLOGY The prevalence of selective mutism varies with age, with younger children at increased risk for the disorder. According to the DSM-5, the point prevalence of selective mutism using clinic or school samples has been found to range between 0.03 percent and 1 percent, depending on whether a clinical or community sample is studied. A large epidemiologic survey in the United Kingdom reported a prevalence rate of selective mutism to be 0.69 percent in children 4 to 5 years of age, which dropped to 0.8 percent near the end of the same academic year. Another survey in the United Kingdom identified 0.06 percent of 7-year-olds as having selective mutism. Young children are more vulnerable to the disorder than older ones. Selective mutism appears to be more common in girls than in boys. Clinical reports suggest that many young children spontaneously “outgrow” this disorder as they get older; the longitudinal course of the disorder remains to be studied. ETIOLOGY Genetic Contribution Selective mutism may have many of the same etiologic factors leading to the emergence of social anxiety disorder. In contrast to other childhood anxiety disorders, however, children with selective mutism are at greater risk for delayed onset of speech or speech abnormalities that may be contributory. However, in addition to the speech and language factor, one survey found that 90 percent of children with selective mutism met diagnostic criteria for social phobia. These children showed high levels of social anxiety without notable psychopathology in other areas, according to parent and teacher ratings. Thus, selective mutism may not represent a distinct disorder, but may be better conceptualized as a subtype of social phobia. Maternal anxiety, depression, and heightened dependence needs are often noted in families of children with selective mutism, similar to families with children who exhibit other anxiety disorders. Parental Interactions Maternal overprotection and anxiety disorders in parents may exacerbate interactions that unwittingly reinforce selective mutism behaviors. Children with selective mutism usually speak freely at home, and only exhibit symptoms when under social pressure either in school or other social situations. Some children seem predisposed to selective mutism after early emotional or physical trauma; thus, some clinicians refer to the phenomenon as traumatic mutism rather than selective mutism. Speech and Language Factors Selective mutism is conceptualized as an anxiety-based refusal to speak; however, a higher than expected proportion of children with the disorder have a history of speech delay. An interesting finding suggests that children with selective mutism are at higher risk for a disturbance in auditory processing, which may interfere with efficient processing of incoming sounds. For the most part, however, speech and language problems in children with selective mutism are subtle and cannot account for the diagnosis of selective mutism. DIAGNOSIS AND CLINICAL FEATURES The diagnosis of selective mutism is not difficult to make after it is clear that a child has adequate language skills in some environments but not in others. The mutism may have developed gradually or suddenly after a disturbing experience. The age of onset can range from 4 to 8 years. Mute periods are most commonly manifested in school or outside the home; in rare cases, a child is mute at home but not in school. Children who exhibit selective mutism may also have symptoms of separation anxiety disorder, school refusal, and delayed language acquisition. Because social anxiety is almost always present in children with selective mutism, behavioral disturbances, such as temper tantrums and oppositional behaviors, may also occur in the home. Compared to children with other anxiety disorders, except social anxiety disorder, children with selective mutism tend to have less social competence and more social anxiety. Janine is a 6-year-old Chinese-American first-grade girl who lives with her biological mother, father, and siblings. Janine’s parents reported a 2-year history of not speaking at school, beginning in kindergarten, or to any children or adults outside of her family, despite speaking normally at home. At home, she reportedly is animated and quite talkative with her immediate family and a few young cousins as well. Although she speaks to adult relatives outside of her immediate family, her communication is often limited to one-word responses to their questions. By her parents’ report, Janine also exhibits extreme social anxiety, to the point of “freezing” in certain situations when attention is focused on her. At the time of her evaluation, Janine had not received prior treatment. Janine speaks fluent English, as well as Mandarin, and, according to her parents, met all developmental milestones on time and appears to have above-average intelligence. They also reported that Janine enjoys dancing, singing, and imaginative play with her sisters. During initial evaluation, Janine failed to make eye contact or respond verbally to the intake clinician. Janine’s parents reported that this behavior is typical of her when in a new situation but that she communicates nonverbally and makes eye contact with most people once she “gets to know them.” On request, Janine’s parents provided a videotaped recording of Janine playing at home with her sisters. In the video, Janine was animated and was speaking spontaneously and fluently without obvious impairment. Janine received diagnoses of selective mutism and social anxiety disorder. CBT was recommended at this time. CBT was initiated and the therapist instructed Janine and her mother to come up with lists of easy, medium, and most difficult “speaking” situations and lists of small, medium, and large rewards. These lists then became the basis for assignments for exposures and reinforcement for speaking tasks that gradually increased in difficulty. BT sessions included time with Janine and her mother together to review past and future assignments and time with Janine and the therapist alone. When treatment began, Janine did not communicate at all verbally or nonverbally with the therapist. The therapist gradually developed a rapport with Janine utilizing less stressful tasks such as whispering to her mother with the therapist in the corner, then nodding yes or no, pointing, whispering to a stuffed animal, whispering to her mother while facing the therapist, and eventually responding to the therapist directly. The therapist used animal puppets to enable Janine to “warm up” without talking directly to the therapist. After three sessions, Janine began to speak to the therapist in a quiet whisper. Janine received stickers for completing each speaking assignment, and, after filling up the sticker charts, she received rewards (a small toy or treat from reward list). Janine was also given assignments that involved her teacher and classmates. These were implemented in gradual fashion and included waving to the teacher, playing an audiotape of her saying “hello” to the teacher, whispering “hello” to the teacher, speaking “hello” to the teacher in a regular voice, and so on. After approximately 14 sessions, Janine succeeded in speaking a complete sentence in front of the class when called on and spoke to her teacher in front of several other students. During the last few sessions, Janine’s mother took an increasingly active role in assigning and following up on speaking assignments. When Janine entered the 2nd grade it took only a few days for her to speak to her teacher and to most peers in class. After completion of therapy, Janine’s mother continued to monitor Janine’s speaking behaviors and to promote speaking in new situations by encouraging (and rewarding) Janine’s gradual successes with novel people and situations. (Adapted from case material from. Lindsey Bergman, Ph.D. and John Piacentini, Ph.D.) Pathology and Laboratory Examination No specific laboratory measures are useful in the diagnosis or treatment of selective mutism. DIFFERENTIAL DIAGNOSIS Differential diagnosis of children who are silent in social situations emphasizes ruling out communications disorder, autism spectrum disorder, and social anxiety disorder, which may be diagnosed comorbidly. Once it is confirmed that the child is fully capable of speaking in certain situations, which are comfortable, but not in school and other social situations, an anxiety-related disorder comes to mind. Shy children may exhibit a transient muteness in new, anxiety-provoking situations. These children often have histories of not speaking in the presence of strangers and of clinging to their mothers. Most children who are mute on entering school improve spontaneously and may be described as having transient adaptation shyness. Selective mutism must also be distinguished from mental retardation, pervasive developmental disorders, and expressive language disorder. In these disorders, the symptoms are widespread, and no one situation exists in which the child communicates normally; the child may have an inability, rather than a refusal, to speak. In mutism secondary to conversion disorder, the mutism is pervasive. Children introduced into an environment in which a different language is spoken may be reticent to begin using the new language. Selective mutism should be diagnosed only when children also refuse to converse in their native language and when they have gained communicative competence in the new language but refuse to speak it. COURSE AND PROGNOSIS Children with selective mutism are often excessively shy during preschool years, but the onset of the full disorder is usually not evident until age 5 or 6 years. Many very young children with early symptoms of selective mutism in a transitional period when entering preschool have a spontaneous improvement over a number of months and never fulfill criteria for the disorder. A common pattern for a child with selective mutism is to speak almost exclusively at home with the nuclear family but not elsewhere, especially not at school. Consequently, a child with selective mutism may have academic difficulties, or even failure due to a lack of participation. Children with selective mutism are typically shy, anxious, and at increased risk for a depressive disorder. Many children with early onset selective mutism remit with or without treatment. Recent data suggest that fluoxetine (Prozac) may influence the course of selective mutism, and treatment enhances recovery. Children in whom the disorder persists often have difficulty forming social relationships. Teasing and scapegoating by peers may cause them to refuse to go to school. Some children with any form of severe social anxiety are characterized by rigidity, compulsive traits, negativism, temper tantrums, and oppositional and aggressive behavior at home. Other children with the disorder tolerate the feared situation by communicating with gestures, such as nodding, shaking the head, and saying “Uh-huh” or “No.” In one follow-up study, about one half of children with selective mutism improved within 5 to 10 years. Children who do not improve by age 10 years appear to have a long-term course and a worse prognosis. As many as one third of children with selective mutism, with or without treatment, may develop other psychiatric disorders, particularly other anxiety disorders and depression. TREATMENT A multimodal approach using psychoeducation for the family, CBT, and SSRIs as needed is recommended. Preschool children may also benefit from a therapeutic nursery. For school-age children, individual CBT is recommended as a first-line treatment. Family education and cooperation are beneficial. Published data on the successful treatment of children with selective mutism is scant, yet solid evidence indicates that children with social anxiety disorder respond to various SSRIs and, currently, CBT treatments are under investigation in a multisite, randomized placebo-controlled trial of children with 37 - 31.14 Obsessive Compulsive Disorder in Childh 31.14 Obsessive-Compulsive Disorder in Childhood and Adolescence anxiety disorders. A recent report of 21 children with selective mutism treated in an open trial with fluoxetine suggested that this medication may be effective for childhood selective mutism. Reports have confirmed the efficacy of fluoxetine in the treatment of adult social phobia and in at least one double-blind, placebo-controlled study using fluoxetine with children with mutism. A large NIMH-funded study of anxiety disorders in children and adolescents called Research Units in Pediatric Psychopharmacology (RUPP), has shown distinct superiority of fluvoxamine over placebo in the treatment of a variety of childhood anxiety disorders. Children with selective mutism may benefit similarly to those with social phobia given the current belief that it is a subgroup of social phobia. SSRI medications that have been shown in randomized, placebo-controlled trials to have benefit in the treatment of children with social phobia include fluoxetine (20 mg to 60 mg per day), fluvoxamine (Luvox; 50 mg to 300 mg per day), sertraline (Zoloft; 25 mg to 200 mg per day), and paroxetine (Paxil; 10 mg to 50 mg per day). REFERENCES Bergman RL, Lee JC. Selective mutism. In: Sadock BJ, Sadock VA, Ruiz P, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Vol. 2. Philadelphia: Lippincott Williams & Wilkins; 2009:3694. Carbone D, Schmidt LA, Cunningham CC, McHolm AE, Edison S, St. Pierre J, Boyle JH. Behavioral and socio-emotional functioning in children with selective mutism: A comparison with anxious and typically developing children across multiple informants. J Abnorm Child Psychol. 2010;38:1057–1067. Davis TE III, May A, Whiting SE. Evidence-based treatment of anxiety and phobia in children and adolescents: Current status and effects on the emotional response. Clin Psychol Rev. 2011;31:592–602. Kehle TJ, Bray MA, Theodore LA. Selective mutism. In: Bear GG, Minke KM, eds. Children’s Needs III: Development, Prevention, and Intervention. Washington DC: National Association of School Psychologists; 2006:293. Rynn M, Puliafico A, Heleniak C, Rikhi P, Ghalib K, Vidair H. Advances in pharmacotherapy for pediatric anxiety disorder. Depress Anxiety. 2011;28:76–87. Schwartz RH, Freedy AS, Sheridan MJ. Selective mutism: Are primary care physicians missing the silence? Clin Pediatr (Phila). 2006;45:43–48. Scott S, Beidel DC. Selective mutism: An update and suggestions for future research. Curr Psychiatry Rep. 2011;13:251–257. Toppelberg CO, Tabors P, Coggins A, Lum K, Burger C. Differential diagnosis of selective mutism in bilingual children. J Am Acad Child Adolesc Psychiatry. 2005;44(6):592–595. Wagner KD, Berard R, Stein MB, Wetherhold E, Carpenter DJ, Perera P, Gee M, Davy K, Machin A. A multicenter, randomized, double-blind, placebo controlled trial of paroxetine in children and adolescents with social anxiety disorder. Arch Gen Psychiatry. 2004;61:1153. Waslick B. Psychopharmacology intervention for pediatric anxiety disorders: A research update. Child Adolesc Psychiatr Clin N Am. 2006;1:51. Yeganeh R, Beidel DC, Turner SM. Selective mutism: More than social anxiety? Depress Anxiety. 2006;23(3):117. 31.14 Obsessive-Compulsive Disorder in Childhood and Adolescence Childhood obsessive-compulsive disorder (OCD) is characterized by recurrent intrusive thoughts associated with anxiety or fear and/or repetitive purposeful mental or behavioral actions aimed at reducing fears and tensions caused by obsessions. Data suggest that up to 25 percent of cases of OCD have their onset by 14 years of age. The overall clinical presentation of OCD in youth is similar to that in adults; however, compared to adults, children and adolescents with OCD more often do not consider their obsessional thoughts or repetitive behaviors to be unreasonable. In milder cases of OCD, a trial of cognitive-behavioral therapy (CBT) is recommended as an initial intervention. OCD in youth is often treated successfully with selective serotonin reuptake inhibitors (SSRIs) or CBT alone, or in combination. The results of a large-scale, randomized, placebo-controlled study called the Pediatric OCD Treatment Study (POTS), demonstrated that the greatest rates of remission in pediatric OCD are achieved with a combination of both serotonergic agents and CBT treatment. The American Psychiatric Association’s Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5) removed OCD from its former category of Anxiety Disorders and placed it in a new category called Obsessive-Compulsive and Related Disorders, with related disorders such as trichotillomania (hair pulling disorder), hoarding disorder, body dysmorphic disorder, and excoriation (skin picking) disorder. Nevertheless the relationship between OCD and other anxiety disorders remains significant and supported by research. EPIDEMIOLOGY OCD is common among children and adolescents, with a point prevalence of about 0.5 percent and a lifetime prevalence of 2 to 4 percent. The rate of OCD among youth rises exponentially with increasing age, with rates of 0.3 percent in children between the ages of 5 and 7 years, rising to rates between 0.6 percent and 1 percent among teens. According to the DSM-5, the prevalence of OCD in the United States is 1.2 percent, with a slightly higher rate in females. Rates of OCD among adolescents are greater than those for schizophrenia or bipolar disorder. Among young children with OCD there appears to be a slight male predominance, which diminishes with age. ETIOLOGY Genetic Factors Genetic factors have been estimated to contribute significantly to the development of OCD in early onset illness. The rate of OCD among first-degree relatives of children and adolescents who develop OCD is ten times greater than for the general population. Twin studies have shown that the concordance rates for OCD is higher for monozygotic twins (0.57) than for dizygotic twins (0.22); however, nongenetic factors play a role that may be equal to or greater than genetic contributions in some cases. OCD is a heterogeneous disorder that has been recognized for decades to run in families. In addition, the presence of subclinical symptom constellations in family members appears to breed true. Genetic linkage studies have revealed evidence of susceptibility loci on chromosomes 1q, 3q, 6q, 7p, 9p, 10p, and 15q. The OCD collaborative genetics study found that the Sapap3 gene was associated with grooming disorders and may be a promising candidate gene for OCD. There is evidence that the glutamate receptor– modulating genes may also be associated with and play a role in the emergence of OCD. Family studies have suggested a relationship between OCD and tic disorders such as Tourette’s syndrome. OCD and tic disorders are believed to share susceptibility factors, which may include both genetic and nongenetic factors. Neuroimmunology Immunological contributions to the emergence of OCD have been hypothesized to be related to an inflammatory process in the basal ganglia associated with an immune response to a systemic infection that may trigger OCD and tics. A prototype of this hypothesis has been the controversial association of OCD symptoms in a small subgroup of children and adolescents following documented exposure to or infection with group A β-hemolytic streptococcus (GABHS). Under this hypothesis, cases of infection-triggered OCD have been termed Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcus (PANDAS), and are believed to parallel an autoimmune process leading to a movement disorder much like Sydenham’s chorea following rheumatic fever. Some evidence from magnetic resonance imaging (MRI) studies has documented a proportional relationship between the size of the basal ganglia and the severity of OCD symptoms in a small sample. GABHS may be one of many physiological stressors that can lead to an increase or emergence of OCD or tics; however, a prospective longitudinal study of youth with PANDAS followed over a 2-year period found no evidence of a temporal association between GABHS infections and OCD symptom exacerbations in children who met the criteria for PANDAS. The presentation of OCD in children and adolescents due to acute exposure to GABHS represents a minority of OCD cases in youth and remains controversial. Neurochemistry The evidence that SSRIs diminish symptoms of OCD, along with findings of altered sensitivity to the acute administration of 5-hydroxytryptamine (5-HT) agonists in individuals with OCD, supports the probability of serotonin’s role in OCD. In addition, the dopamine system is believed to be influential in OCD, especially in light of the frequent comorbidity of OCD with tic disorders in childhood. Clinical observations have indicated that obsessions and compulsions may be exacerbated during treatment of ADHD (another frequent OCD comorbidity) with stimulant agents. Dopamine antagonists administered along with SSRIs may augment effectiveness of SSRIs in the treatment of OCD. Evidence suggests that multiple neurotransmitter systems may play a role in OCD. Neuroimaging Both computed tomography (CT) and MRI of untreated children and adults with OCD have revealed smaller volumes of basal ganglia segments compared to normal controls. A meta-analysis of voxel-based morphometry (VBM) to assess gray matter density compared 343 OCD patients with 318 healthy controls, and found that gray matter density in OCD patients was smaller in parietofrontal cortical regions (including the supramarginal gyrus, the dorsolateral prefrontal cortex, and the orbitofrontal cortex), but larger in the basal ganglia (the putamen) and anterior prefrontal cortex compared to healthy controls. Increased gray matter volume in the basal ganglia of patients with OCD has been reported in other studies as well. These structural abnormalities in the prefrontal-basal ganglia are likely to be integrally involved in the pathophysiology of OCD. It is not clear whether the increases in gray matter in individuals with OCD occur before or after the symptoms emerge. In children, evidence suggests that thalamic volume is increased. Adult studies have provided evidence of hypermetabolism of frontal cortical-striatal-thalamocortical networks in untreated individuals with OCD. Of interest, imaging studies of before and after treatment have revealed that both medication and behavioral interventions lead to a reduction of orbit frontal and caudate metabolic rates in children and adults with OCD. DIAGNOSIS AND CLINICAL FEATURES Children and adolescents with obsessions or compulsions are often referred for treatment due to the excessive time that they devote to their intrusive thoughts and repetitive rituals. For some children, their compulsive rituals are perceived as reasonable responses to their extreme fears and anxieties. Nevertheless, they are aware of their discomfort and inability to carry out usual daily activities in a timely manner due to the compulsions, such as getting ready to leave their homes to go to school each morning. The most commonly reported obsessions in children and adolescents include extreme fears of contamination—exposure to dirt, germs, or disease—followed by worries related to harm befalling themselves, family members, or fear of harming others due to losing control over aggressive impulses. Also commonly reported are obsessional needs for symmetry or exactness, hoarding, and excessive religious or moral concerns. Typical compulsive rituals among children and adolescents involve cleaning, checking, counting, repeating behaviors, or arranging items. Associated features in children and adolescents with OCD include avoidance, indecision, doubt, and a slowness to complete tasks. In most cases of OCD among youth, obsessions and compulsions are present. According to the DSM-5, diagnosis of OCD is identical to that of adults, with the note that young children may not be able to articulate the aims of their compulsions in diminishing their anxiety. The DSM-5 has also added the following specifiers: with good, fair, poor, or absent insight; that is, the greater the belief in the OCD obsessions and compulsions, the poorer the insight. An additional specifier indicates whether the individual has a current or past history of a tic disorder. Table 10.1-1 designates the DSM-5 diagnostic criteria for OCD. Many children and adolescents who develop OCD have an insidious onset and may hide their symptoms as long as possible so that their rituals will not be challenged or disrupted. A minority of children, particularly males with early onset may have a rapid unfolding of multiple symptoms within a few months. OCD is commonly found to be comorbid with anxiety disorders, attention-deficit/hyperactivity disorder (ADHD), and tic disorders, especially Tourette’s syndrome. Children with comorbid OCD and tic disorders are more likely to exhibit counting, arranging, or ordering compulsions and less likely to manifest excessive washing and cleaning compulsions. The high comorbidity of OCD, Tourette’s syndrome, and ADHD has led investigators to postulate a common genetic vulnerability to all three of these disorders. It is important to search for comorbidity in children and adolescents with OCD so that optimal treatments can be administered. Jason, a 12-year-old boy in the sixth grade, was brought for evaluation by his parents, who expressed concerns over his repeated questions and anxiety regarding developing acquired immunodeficiency syndrome (AIDS). Jason was a highfunctioning and well-adjusted boy who abruptly began to exhibit extremely disruptive behaviors related to his fears of AIDS approximately 2 to 3 months before the evaluation. Jason’s new behaviors included relentless concerns about contracting illness, washing rituals, repeated expressions of uncertainty over his own behavior, seeking reassurance, repeating rituals, and avoidance. Specifically, Jason repeatedly expressed his fear and belief that he was exposed to human immunodeficiency virus (HIV) through exposure to multiple strangers who were infected. For example, while riding in the car, if Jason saw a stranger from the window who appeared to him to be poor or ill kempt, he experienced a surge of extreme anxiety and obsessively agonized about whether the stranger could have AIDS and had exposed him to it. Despite his parents’ reassurances about his safety and lack of exposure to illness, Jason insisted on vigorously washing himself for approximately one hour each time he reached home after being out. Jason continually expressed doubts about his own behavior. He often asked his parents, “Did I use the s___ word? Did I use the f___ word?” Reassurance was only slightly calming. Jason, previously an excellent student, began to lose the ability to focus on schoolwork. While reading passages from assigned materials, Jason frequently experienced severe anxiety, wondering if he had missed a word or misunderstood the sentence, and proceeded to reread the material. Completing a page of written material began to take Jason 30 to 60 minutes. Over several weeks, he was less and less able to complete assignments, following which, he became very distressed over his deteriorating grades. During Jason’s evaluation, his family history suggested that Jason’s older sister had experienced a period in which she too had similar but milder anxieties, with less interference in functioning, and she had never received any treatment for those symptoms. At the intake interview, Jason presented as a preoccupied and sad boy who was cooperative with questioning. He did not volunteer much information, and he allowed his parents to recount the extent of his symptoms. Jason believed that his relentless concerns were well founded, and that he required repeated reassurance from his parents in order to continue his daily activities. Jason met full diagnostic criteria for OCD. Symptoms of depression were present but not sufficient for major depressive disorder. CBT was initiated; however, Jason was so fearful of deviating from his rituals that he was unable to participate fully in his treatment, and he became despondent about his future. Jason refused to go to school due to his increasing distress associated with reading and his shame regarding his diminishing academic performance. Given his limited progress during the first 2 months of CBT, fluoxetine (Prozac) was added and increased up to 40 mg per day. Over a 3-week period some improvement was noted, and Jason was more amenable to cooperating with his CBT treatment. CBT and SSRI treatment was continued over the next 3 months on a regular basis. Over time, Jason finally began to show some flexibility with his rituals, and he was able to decrease the amount of time he spent with rituals. Once he had found some relief from his symptoms, Jason was able to focus more on his schoolwork and his family life. Follow-up over the next year was positive; Jason had maintained his gains from treatment, with only minimal interference from residual OCD symptoms. Jason’s academic achievement improved, he was able to engage in activities with friends, and he spent almost no time preoccupied with obsessional thoughts of illness and cleansing rituals. (Adapted from a case courtesy of James T. McCracken, M.D.) Pathology and Laboratory Examination No specific laboratory measures are useful in the diagnosis of obsessive-compulsive disorder. Even when the onset of obsessions or compulsions appears to be associated with a recent infection with GABHS, antigens and antibodies to the bacteria do not indicate a causal relationship between GABHS and OCD. DIFFERENTIAL DIAGNOSIS Developmentally appropriate rituals in the play and behavior of young children should not be confused with OCD in that age group. Preschoolers often engage in ritualistic play and request a predictable routine such as bathing, reading stories, or selecting the same stuffed animal at bedtime, to promote a sense of security and comfort. These routines allay developmentally normal fears and lead to reasonable completion of daily activities. On the other hand, obsessions or compulsions are driven by extreme fears, and they significantly interfere with daily function because of the excessive time that they consume and the extreme distress that ensues when they are interrupted. The rituals of preschoolers generally become less rigid by the time they enter grade school, and school-age children do not typically experience a surge of anxiety when they encounter small changes in their routine. Children and adolescents with generalized anxiety disorder, separation anxiety disorder, and social phobia experience intense worries that are often expressed repeatedly; however, these are mundane compared to obsessions, which are often so extreme that they appear bizarre. A child with generalized anxiety disorder typically worries repeatedly about performance on academic examinations, whereas a child with OCD may experience repeated intrusive thoughts that he may harm someone he loves. The compulsions of OCD are not present in other anxiety disorders; however, children with autism spectrum disorders often display repetitive behaviors that may resemble OCD. In contrast with the rituals of OCD, children with autism spectrum disorder are not responding to anxiety, but are more often exhibiting stereotyped behaviors that are selfstimulating or self-comforting. Children and adolescents with tic disorders such as Tourette’s syndrome may display complex repetitive compulsive behaviors similar to the compulsions seen in OCD. Children and adolescents with tic disorders, in fact, are at higher risk for the development of concurrent OCD. Severe OCD symptoms may be difficult to distinguish from delusional symptoms, especially when the obsessions and compulsions are bizarre in nature. In most adults, and often in youth with OCD, despite an inability to control their obsessions or resist completing compulsions, insight into their lack of reasonableness is preserved. That is, an individual’s conviction in their beliefs often does not reach delusional intensity. When insight is present, and underlying anxiety can be described, even in the face of significant dysfunction due to bizarre obsessions and compulsion, the diagnosis of OCD is suspect. COURSE AND PROGNOSIS OCD with an onset in childhood and adolescence is most often a chronic, waxing and waning disorder with variability in severity and outcome. Follow-up studies suggest that up to 40 to 50 percent of children and adolescents recover from OCD with minimal residual symptoms. A study of childhood OCD treatment with sertraline resulted in close to 50 percent of participants experiencing complete remission, and partial remission in another 25 percent with a follow-up time of one year. Predictors of the best outcome were in those children and adolescents without comorbid disorders, including tic disorders and ADHD. A study of 142 children and adolescents with OCD followed over a period of 9 years at the Maudsley Hospital in England found 41 percent to have a persistence of OCD, with 40 percent exhibiting an additional psychiatric diagnosis at follow-up. The main predictor for persistent OCD was duration of illness at the time of initial assessment. Approximately half of the follow-up group was still receiving treatment, and half believed that they needed continued treatment. Neuropsychological functioning may also play a role in outcome and prognosis. A study of 63 youth with OCD who completed the Rey-Osterrieth Complex Figure (ROCF) along with specific subtests of the Wechsler Intelligence Scale for Children, Third Edition (WISC-III), found that 5-minute recall accuracy from the ROCF was positively correlated with response to treatment, particularly CBT. These findings imply that poorer performance on the ROCF and poor response to CFBT may be in part due to executive functioning difficulties and that treatment may need to be modified to account for these obstacles. Overall, the prognosis is hopeful for most children and adolescents with mild to moderate OCD. In about 10 percent of cases, OCD may represent a prodrome of a psychotic disorder in children and adolescents. In youth with subthreshold OCD symptoms, there is a high risk of developing of the full OCD disorder within a period of 2 years. Childhood OCD has been shown to be responsive to available treatments, resulting in improvement, if not complete remission, in the majority of cases. TREATMENT CBT and SSRIs have both been shown to be efficacious treatments for OCD in youth. CBT geared toward children of varying ages is based on the principle of developmentally appropriate exposure to the feared stimuli coupled with response prevention, leading to diminishing anxiety over time on exposure to feared situations. CBT manuals have been developed to ensure that developmentally appropriate interventions are made and that comprehensive education is provided to the child and parents. Treatment guidelines for children and adolescents with mild to moderate OCD recommend a trial of CBT prior to initiating medication. However, the Pediatric OCD Treatment Study (POTS), a multi-site National Institute of Health (NIH)–funded investigation of sertraline and CBT each alone, and in combination, for the treatment of childhood-onset OCD, revealed that the combination was superior to either treatment alone. Each treatment alone also provided encouraging levels of response. Mean daily dose of sertraline was 133 mg/day in the group administered the combination treatment, and 170 mg/day for the sertraline alone group. Improvement with pharmacologic intervention of childhood OCD usually occurs within 8 to 12 weeks of treatment. Most children and adolescents who experienced a remission with acute treatment using SSRIs were still responsive over a period of a year. Among youth with OCD who obtain partial response to a therapeutic trial of SSRI treatment, augmentation with a short-term OCD-specific CBT leads to a significantly greater response. Evidence shows that higher treatment expectations by patients and families are linked to better treatment response, greater compliance with home-based CBT assignments, less drop out of treatment, and reduced impairment. In addition to individual CBT, both family and group CBT interventions have been shown to be efficacious in the treatment of childhood OCD. Family CBT (FCBT) intervention in the treatment of OCD in youth has been shown to increase response rates. A controlled comparison of family CBT and psychoeducation and relaxation (PRT) in 71 families of children with OCD showed that clinical remission rates in the FCBT group were significantly higher than those in the PRT group. The FCBT treatment reduced parent involvement and accommodation in their affected child’s symptoms, which led to decreased symptomatology. A randomized controlled study investigating web-camera delivered FCBT (W-CBT) compared to a waitlist condition assigned 31 families to one of the above conditions. Assessments were conducted immediately before and after treatment and at 3-month follow-up for the W-CBT group. The W-CBT group was superior to the waitlist control group on all primary outcome measures, with large effect sizes. Eighty-one percent of the W-CBT group responded compared to 13% of the waitlist group. The gains were maintained at the 3-month follow-up assessment. The authors conclude that W-CBT may be efficacious in the treatment of OCD in youth and may be a promising tool for future dissemination. Exposure and response prevention (ERP), a common strategy within CBT already shown to be effective on an individual basis for OCD, was studied in a group format in youth with OCD in a community-based program. Group-based ERP was found to be effective in reducing OCD symptom severity and depressive symptoms, but not anxiety symptoms, in a naturalistic treatment setting for children with OCD and comorbid anxiety and/or depressive features. Robust evidence of SSRI efficacy for OCD in youth has been shown through multiple randomized clinical trials. A meta-analysis of 13 studies of SSRIs, including sertraline, fluvoxamine, fluoxetine, and paroxetine have provided evidence of efficacy of SSRIs with a moderate effect size. A randomized controlled clinical trial of citalopram versus fluoxetine in youth with OCD found that citalopram was as safe and effective as fluoxetine for the treatment of OCD in children and adolescents. There have been no apparent differences in the rate of response for the individual SSRIs. Currently, three SSRIs: sertraline (at least 6 years), fluoxetine (at least 7 years), and fluvoxamine (at least 8 years), as well another as clomipramine (at least 10 years), have received Food and Drug Administration (FDA) approval for the treatment of OCD in youth. The black box warning for antidepressants used in children for any disorder, including OCD is applicable, so that close monitoring for suicidal ideation or behavior is mandated when these agents are used in the treatment of childhood OCD. Typical side effects that emerge with the use of SSRIs include insomnia, nausea, agitation, tremor, and fatigue. Dosage ranges for the various SSRIs found to have efficacy in randomized clinical trials are the following: fluoxetine (20 mg to 60 mg), sertraline (50 mg to 200 mg), fluvoxamine (up to 200 mg), and paroxetine (up to 50 mg). Clomipramine was the first antidepressant studied in the treatment of OCD in childhood and the only tricyclic antidepressant that has FDA approval for the treatment of anxiety disorders in childhood. Clomipramine was found to be efficacious in doses up to 200 mg, or 3 mg/kg, whichever is less, and may be chosen for children or adolescents who cannot tolerate other SSRIs due to insomnia, significant appetite suppression, or activation. Nevertheless, clomipramine is not recommended as a first-line treatment due to its greater potential risks compared to other SSRIs, including cardiovascular risk of hypotension and arrhythmia, and seizure risk. Pediatric patients with OCD who respond only partially to medications tend to have at least moderate to severe OCD symptoms, high ratings of global impairment and significant comorbidity even after their partial response to an adequate trial of medication. Augmentation strategies with medications to enhance serotonergic effects, such as with atypical antipsychotics (e.g., risperidone) have demonstrated increased response when partial response has been achieved with SSRIs. Aripiprazole augmentation in 39 adolescents with OCD who did not respond to two trials of monotherapy with SSRIs led to 59 percent of patients being rated as improved or very much improved. Patients who responded to aripiprazole were less impaired at baseline in functional impairment but not in clinical severity of their OCD. Aripiprazole final mean dose was 12.2 mg per day. This agent may be effective for pediatric OCD and warrants further controlled trials. Given the lack of data on discontinuation, recommendations for maintaining medication such as stabilization, education about relapse risk, and tapering medication during the summer are likely in order to minimize academic compromise in case of relapse. For children and adolescents with more severe or multiple episodes of significant exacerbation of symptoms, treatment for more than a year is recommended. Overall, efficacy of treatment for children and adolescents with OCD is high with choices of SSRIs and CBT. 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Child Adolesc Psychiatr Clin N Am. 2006;1:51. 31.15 Early-Onset Schizophrenia Early-onset schizophrenia comprises childhood-onset and adolescent-onset schizophrenia. Childhood-onset schizophrenia is a very rare and virulent form of schizophrenia now recognized as a progressive neurodevelopmental disorder. Childhood onset is characterized by a more chronic course, with severe social and cognitive consequences and increased negative symptoms compared to adult-onset schizophrenia. Childhood-onset schizophrenia is defined by an onset of psychotic symptoms before the age of 13 years, believed to represent a subgroup of patients with schizophrenia with an increased heritable etiology, and evidence of widespread abnormalities in the development of brain structures including the cerebral cortex, white matter, hippocampus and cerebellum. Children diagnosed with childhood-onset schizophrenia have higher than normal rates of premorbid developmental abnormalities that appear to be nonspecific markers of abnormal brain development. Early-onset schizophrenia is defined as an onset of disease before the age of 18 years, including childhood-onset as well as adolescent-onset schizophrenia. Early-onset schizophrenia is associated with severe clinical course, poor psychosocial functioning, and increased severity of brain abnormality. Despite the more severe course, current evidence supports the efficacy of both psychosocial and pharmacological interventions in the management of childhoodonset and, particularly, adolescent-onset schizophrenia. Children with childhood-onset schizophrenia have been shown to have more significant deficits in measures of intelligence quotient (IQ), memory, and tests of perceptuomotor skills compared with adolescent-onset schizophrenia. Increased impairment in childhood-onset schizophrenia of cognitive measures such as IQ, working memory, and perceptuomotor skills such deficits may be premorbid markers of illness, rather than sequelae, of the disorder. Although cognitive impairments are greater in younger patients with schizophrenia, clinical presentation of schizophrenia remains remarkably similar across the ages, and the diagnosis of childhood-onset schizophrenia is continuous with that in adolescents and adults, with one exception: in childhood-onset schizophrenia a failure to achieve expected social and academic functioning may replace a deterioration in functioning. According to the American Psychiatric Association’s Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5), the diagnosis of schizophrenia includes an “active phase” of the illness, consisting of at least one of the following three symptoms: delusions, hallucinations, or disorganized speech, and at least one additional symptom present most of the time for a month. The additional symptom may be another one of the preceding three, or one of the following two symptoms: grossly disorganized or catatonic behavior, or negative symptoms (i.e., diminished emotional expression or avolition). In the active phase, symptoms are present for a significant amount of time during a single month and cause impairment. To meet full criteria for schizophrenia, continuous signs of disturbance must persist for at least 6 months. Social, academic, or occupational impairment must be present. In contrast to previous diagnostic criteria, the subtypes of schizophrenia (paranoid, disorganized, catatonic, undifferentiated, and residual) have been eliminated due to their lack of diagnostic validity and reliability. Instead, an eight-symptom “ClinicianRated Dimensions of Psychosis Symptom Severity” scale for determining severity of psychosis across many psychotic illnesses is included in Section III of the DSM-5. Symptom domains rated in this scale include the following: hallucinations, delusions, disorganized speech, abnormal psychomotor behavior, negative symptoms (restricted emotional expression or avolition), impaired cognition, depression, and mania. HISTORICAL PERSPECTIVE Before the 1960s, the term childhood psychosis was applied to a heterogeneous group of children, many of whom exhibited autism spectrum disorder symptoms without hallucinations and delusions. In the late 1960s and 1970s, reports of children with evidence of a profound psychotic disturbance very early in life included observations of intellectual disabilities, social deficits, and severe communication and language impairments, and no family history of schizophrenia. Children whose psychoses emerged after the age of 5 years, however, more often exhibited auditory hallucinations, delusions, inappropriate affect, thought disorder, normal intellectual function, and a positive family history of schizophrenia. In the 1980s, schizophrenia with childhood onset was formally separated from what was then termed autistic disorder, and currently termed autism spectrum disorder. The distinction of childhood schizophrenia from autism spectrum disorder reflected evidence accrued during the 1960s and 1970s showing a divergent clinical picture, family history, age of onset, and course between the two disorders. However, even after the separation of the disorders, controversy and confusion remained as to the distinctiveness in the long-term courses of these disorders. First, research documented a small group of children with autism spectrum disorder who developed schizophrenia in later childhood or adolescence. Second, many children with childhood-onset schizophrenia exhibit neurodevelopmental abnormalities, some of which are also evident in children with autism spectrum disorder. Children with autism spectrum disorder and those with childhood-onset schizophrenia are typically impaired in multiple areas of adaptive functioning from relatively early in life. However, in autism spectrum disorder, the onset is almost always before 3 years of age, whereas the onset of childhood-onset schizophrenia occurs before the age of 13 years, but most often is not recognizable in children until after the age of 3 years. Childhood-onset schizophrenia is significantly less frequent than adolescent-onset or onset in young adulthood, and few reports document cases of schizophrenia onset before 5 years of age. According to the DSM-5, schizophrenia can be diagnosed in the presence of autism spectrum disorder, provided that the diagnosis of schizophrenia is specifically differentiated from autism spectrum disorder. EPIDEMIOLOGY The frequency of childhood-onset schizophrenia is reported to be less than one case in about 40,000 children, whereas among adolescents between the ages of 13 and 18 years, the frequency of schizophrenia is increased by a factor of at least 50. Schizophrenia with childhood onset resembles the more severe, chronic, and treatment-refractory adultonset schizophrenic subgroups, in that the same core phenomenological features are present; however, in childhood-onset schizophrenia, extremely high rates of comorbidities are present, including attention-deficit/hyperactivity disorder (ADHD), depressive disorders, anxiety disorders, speech and language disorders, and motor disturbances. In adolescents, the prevalence of schizophrenia is estimated to be 50 times that in younger children, with probable rates of 1 to 2 per 1,000. Boys seem to have a slight preponderance among children diagnosed with schizophrenia, with an estimated ratio of about 1.67 boys to 1 girl. Boys often become identified at a younger age than girls do. Schizophrenia rarely is diagnosed in children younger than 5 years of age. The prevalence of schizophrenia among the parents of children with schizophrenia is about 8 percent, which is about twice the prevalence in the parents of patients with adult-onset schizophrenia. ETIOLOGY Childhood-onset schizophrenia is a neurodevelopmental disorder in which complex interactions between genes and the environment are presumed to result in abnormal early brain development. The consequences of the aberrant brain development in schizophrenia may not be fully evident until adolescence or early adulthood; however, data support the hypothesis that white matter abnormalities and disturbances in myelination in childhood, lead to abnormal connectivity between brain regions. The aberrant connectivity in various regions of the brain is believed to be an important contributing factor in the psychotic symptoms and cognitive deficits in childhood-onset schizophrenia. Genetic Factors Estimates of heritability for childhood-onset schizophrenia have been as high as 80 percent. The precise mechanisms of transmission of schizophrenia are still not well understood. Schizophrenia is known to be up to eight times more prevalent among firstdegree relatives of those with schizophrenia than in the general population. Adoption studies of patients with adult-onset schizophrenia have shown that schizophrenia occurs in the biological relatives, not the adoptive relatives. Additional genetic evidence is supported by higher concordance rates for schizophrenia in monozygotic twins than in dizygotic twins. Higher rates of schizophrenia have been established among relatives of those with childhood-onset schizophrenia than in the relatives of those with adult-onset schizophrenia. Endophenotype Markers for Childhood-Onset Schizophrenia. Currently, no reliable method can identify persons at the highest risk for schizophrenia in a given family. Neurodevelopmental abnormalities and higher-than-expected rates of neurological soft signs and impairments in sustaining attention and in strategies for information processing appear among children at high risk. Increased rates of disturbed communication styles are found in family members of individuals with schizophrenia. Reports have documented higher than expected neuropsychological deficits in attention, working memory, and premorbid IQ among children who later develop schizophrenia and its spectrum disorders. Magnetic Resonance Imaging (MRI) Studies A National Institute of Mental Health (NIMH) prospective study of more than 100 patients with childhood-onset schizophrenia and their typically developing siblings has demonstrated progressive loss of gray matter, delayed and disrupted white matter growth, and a decline in cerebellar volume in those with childhood-onset schizophrenia. Although siblings of children with childhood-onset schizophrenia also showed some of these brain disruptions, the gray matter abnormalities were normalized over time in the siblings, indicating a protective mechanism in siblings that was not present in those children with childhood-onset schizophrenia. Furthermore, the hippocampal volume loss across the age span appears to be static among children with childhood-onset schizophrenia. An MRI NIMH study of more than 100 children with childhood-onset schizophrenia and their typically developing siblings, studied for about two decades, documented that in childhood-onset schizophrenia, progressive brain gray matter loss occurs continuously over time. This gray matter shrinkage occurs with ventricular increases, with a pattern of loss originating in the parietal region and proceeding frontally to dorsolateral prefrontal and temporal cortices, including superior temporal gyri. Studies of childhood-onset schizophrenia at the NIMH provided evidence that early loss of parietal gray matter followed by frontal and parietal gray matter loss is more pronounced in childhood-onset schizophrenia than in schizophrenia with later onset. Other research utilized diffusion tensor images from children with childhood-onset schizophrenia versus controls and found increased diffusivities in the posterior corona radiata in children with childhood-onset schizophrenia, which implicated abnormal connectivity with the parietal lobes. These results contrasted with findings among subjects with later onset of schizophrenia in whom there were more abnormalities in the frontal lobes. DIAGNOSIS AND CLINICAL FEATURES All of the symptoms included in adult-onset schizophrenia may be manifest in children and adolescents with the disorder. However, youth with schizophrenia are more likely to have a premorbid history of social rejection, poor peer relationships, clingy withdrawn behavior, and academic trouble than those with adult-onset schizophrenia. Some children with schizophrenia evaluated in middle childhood have early histories of delayed motor milestones and language acquisition similar to some symptoms of autism spectrum disorder. The onset of schizophrenia in childhood is frequently insidious, starting with inappropriate affect or unusual behavior; it may take months or years for a child to meet all of the diagnostic criteria for schizophrenia. Auditory hallucinations commonly occur in children with schizophrenia. The voices may reflect an ongoing critical commentary, or command hallucinations may instruct children to harm or kill themselves or others. Hallucinatory voices may sound human or animal, or “bizarre,” for example, identified as “a computer in my head,” martians, or the voice of someone familiar, such as a relative. The childhood-onset schizophrenia project at the NIMH found high rates across all hallucination modalities. However, there were unexpectedly high rates of tactile, olfactory, and visual hallucinations among this study group of patients with childhood-onset schizophrenia. Visual hallucinations were associated with lower IQ and earlier age at onset of disease. Visual hallucinations are often frightening; affected children may “see” images of the devil, skeletons, scary faces, or space creatures. Transient phobic visual hallucinations occur in severely anxious or traumatized children who do not develop major psychotic disorders. Visual, tactile, and olfactory hallucinations may be a marker of more severe psychosis. Delusions occur in up to half of children and adolescents with schizophrenia, in various forms, including persecutory, grandiose, and religious. Delusions increase in frequency with increased age. Blunted or inappropriate affect appears almost universally in children with schizophrenia. Children with schizophrenia may giggle inappropriately or cry without being able to explain why. Formal thought disorders, including loosening of associations and thought blocking, are common features among youth with schizophrenia. Illogical thinking and poverty of thought are also often present. Unlike adults with schizophrenia, children with schizophrenia do not have poverty of speech content, but they speak less than other children of the same intelligence and are ambiguous in the way they refer to persons, objects, and events. The communication deficits observable in children with schizophrenia include unpredictably changing the topic of conversation without introducing the new topic to the listener (loose associations). Children with schizophrenia also exhibit illogical thinking and speaking and tend to underuse self-initiated repair strategies to aid in their communication. When an utterance is unclear or vague, normal children attempt to clarify their communication with repetitions, revision, and more detail. Children with schizophrenia, on the other hand, fail to aid communication with revision, fillers, or starting over. These deficits may be conceptualized as negative symptoms in childhood schizophrenia. Although core phenomena for schizophrenia seem to be universal across the age span, a child’s developmental level significantly influences the presentation of the symptoms. Delusions of young children are less complex, therefore, than those of older children, for example, age-appropriate content, such as animal imagery and monsters, is likely to be a source of delusional fear in young children. According to the DSM-5, a child with schizophrenia may experience deterioration of function, along with the emergence of psychotic symptoms, or the child may never achieve the expected level of functioning. A 12-year-old 6th grade boy named Ian, with a longstanding history of social isolation, academic problems, and temper outbursts began to develop concerns that his parents might be poisoning his food. Over the next year, his symptoms progressed with increased suspiciousness and fearfulness, preoccupation with food, and beliefs that Satan was trying to communicate with him. Ian also appeared to be responding to auditory hallucinations that he believed were coming from the radio and television, which he found frightening and commanded him to harm his parents. Ian had also been informing his mother that their food had a strange smell and that’s why he thought it was poisoned, and at night, he would see frightening figures in his room. During this time, his parents also observed bizarre behaviors, including talking and yelling to himself, perseverating about devils and demons, and finally, assaulting family members because he thought they were evil. On one occasion, Ian was found to be scratching himself with a kitchen knife in an effort to “please God.” No predominant mood symptoms emerged, and there was no history of substance abuse found. Developmentally, Ian was the product of a full-term pregnancy complicated by a difficult labor and forceps delivery. His early motor and speech milestones were each delayed by about 6 months; however, his pediatrician reassured his parents that this was within the limits of normal development. As a younger child, Ian tended to be quiet and socially awkward. His intellectual function was tested and was found to be in the average range; however, academic achievement testing was consistently below grade level. Ian remained lonely and isolated, and he had great difficulty making friends. Ian has had no medical problems and his immunizations were up to date. Ian’s family psychiatric history was significant for depression in a maternal aunt and a completed suicide in a maternal great-grandparent. Ian was sent by ambulance to the hospital for the first time from school when he tried to jump off a balcony on the second story of his school, in response to auditory hallucinations commanding him to kill himself. During his hospitalization his parents reluctantly consented to a trial of risperidone for him, and he was titrated up to 3 mg per day. His auditory hallucinations were moderately improved after 2 weeks of treatment; however, he continued to be suspicious and mistrustful of his physicians and family. Ian’s family was very confused as to what had caused Ian’s serious symptoms, and the hospital treatment team met with his parents multiple times during his hospitalization to reassure them that they had not caused his illness and that; their continued support might improve his chances of improvement. After discharge from the hospital, 30 days later, Ian was placed in a special education program, in a nonpublic school, and he was assigned a psychotherapist who met regularly with him individually and with his family. At the time of discharge from the hospital, Ian’s symptoms had moderately improved, although he still had auditory hallucinations intermittently. Over the next 5 years subsequent to the onset of his illness, Ian had many exacerbations of his psychosis and he was hospitalized nine times, including placement in a long-term residential program. Ian had received trials of olanzapine, quetiapine, and aripiprazole, each of which seemed to lead to improvement for a period of time, after which he was no longer responsive to the medications. Ian continued to receive individual cognitive behavioral therapy and family therapy, and his family was very supportive. Even with these interventions, Ian’s mental status continued to display tangential and disorganized thinking, paranoid delusions, loose associations, perseverative speech patterns, and a flat, at times inappropriate, affect. He had periods of time in which he resorted to pacing and muttering to himself, with no social interaction with others unless initiated by adults. Finally, Ian achieved significant improvement after being placed on clozapine (Clozaril) therapy, although he remained mildly symptomatic. (Adapted from a case by Jon M. McClellan, M.D.) PATHOLOGY AND LABORATORY EXAMINATIONS No specific laboratory tests are diagnostically specific for childhood-onset schizophrenia. Electroencephalography (EEG) studies have not been helpful in distinguishing children with schizophrenia from other children. Although data exist to suggest that hypoprolinemia is associated with the risk of schizoaffective disorder due to an alteration on chromosome 22q11, no association of hyperprolinemia with childhoodonset schizophrenia has been identified. DIFFERENTIAL DIAGNOSIS One of the significant challenges in making a diagnosis of childhood-onset schizophrenia is that very young children who report hallucinations, apparent thought disorders, language delays, and poor ability to differentiate reality from fantasy may be manifesting phenomena better accounted for by other disorders such as posttraumatic stress disorder, or sometimes developmental immaturity, none of which evolve into a major psychotic illness. Nevertheless, the differential diagnosis of childhood-onset schizophrenia includes autism spectrum disorder, bipolar disorders, depressive psychotic disorders, multicomplex developmental syndromes, drug-induced psychosis, and psychosis caused by organic disease states. Children with childhood-onset schizophrenia have been shown to have frequent comorbidities, including ADHD, oppositional defiant disorder, and major depression. Children with schizotypal personality disorder have some traits in common with children who meet diagnostic criteria for schizophrenia. Blunted affect, social isolation, eccentric thoughts, ideas of reference, and bizarre behavior can be seen in both disorders; however, in schizophrenia, overt psychotic symptoms, such as hallucinations, delusions, and incoherence, must be present at some point. Hallucinations alone, however, are not evidence of schizophrenia; patients must show either a deterioration of function or an inability to meet an expected developmental level to warrant the diagnosis of schizophrenia. Auditory and visual hallucinations can appear as self-limited events in nonpsychotic young children who are experiencing extreme stress or anxiety related to unstable home lives, abuse, or neglect or in children experiencing a major loss. Psychotic phenomena are common among children with major depressive disorder, in which both hallucinations and, less commonly, delusions may occur. The congruence of mood with psychotic features is most pronounced in depressed children, although children with schizophrenia may also seem sad. The hallucinations and delusions of schizophrenia are more likely to have a bizarre quality than those of children with depressive disorders. In children and adolescents with bipolar I disorder, it often is difficult to distinguish a first episode of mania with psychotic features from schizophrenia if the child has no history of previous depressions. Grandiose delusions and hallucinations are typical of manic episodes, but clinicians often must follow the natural history of the disorder to confirm the presence of a mood disorder. Autism spectrum disorders share some features with schizophrenia, most notably, difficulty with social relationships, an early history of delayed language acquisition, and ongoing communication deficits. However, hallucinations, delusions, and formal thought disorder are core features of schizophrenia and are not expected features of autism spectrum disorder. Autism spectrum disorder is usually diagnosed by 3 years of age, whereas schizophrenia with childhood onset can rarely be diagnosed before 5 years of age. Among adolescents, alcohol and other substance abuse sometimes can result in a deterioration of function, psychotic symptoms, and paranoid delusions. Amphetamines, lysergic acid diethylamide (LSD), and phencyclidine (PCP) may lead to a psychotic state. A sudden, flagrant onset of paranoid psychosis may suggest substance-induced psychotic disorder. Medical conditions that can induce psychotic features include thyroid disease, systemic lupus erythematosus, and temporal lobe disease. COURSE AND PROGNOSIS Important predictors of the course and outcome of childhood and early-onset schizophrenia include the child’s premorbid level of functioning, the age of onset, IQ, response to psychosocial and pharmacological interventions, degree of remission after the first psychotic episode, and degree of family support. Early age at onset, and children with comorbid developmental delays, learning disorders, lower IQ, and premorbid behavioral disorders, such as ADHD and conduct disorder, are less treatment responsive and likely to have the most guarded prognoses. Predictors of a poorer course of childhood-onset schizophrenia include family history of schizophrenia, young age and insidious onset, developmental delays and lower level of premorbid function, and chronic or length of first psychotic episode. Psychosocial and family stressors are known to influence the relapse rate in adults with schizophrenia, and high expression of negative emotion (EE) likely affects children with childhood-onset schizophrenia as well. An important factor in outcome is the accuracy and stability of the diagnosis of schizophrenia. One study reported that one third of children who received an initial diagnosis of schizophrenia were later diagnosed with bipolar disorder in adolescence. Children and adolescents with bipolar I disorder may have a better long-term prognosis than those with schizophrenia. The NIMH-funded Treatment of Early-Onset Schizophrenia reported outcome of neurocognitive functioning in 8- to 19-year-old youth with schizophrenia or schizoaffective disorders, who participated in a randomized double-blind clinical trial comparing molindone, olanzapine, and risperidone. The three medication groups yielded no group differences in neurocognitive functioning over a year; however, when data from the three groups were combined, a significant modest improvement was observed in several domains of neurocognitive functioning. The authors concluded that antipsychotic intervention in youth with early-onset schizophrenia spectrum disorders led to modest improvement in neurocognitive function. TREATMENT The treatment of childhood-onset schizophrenia requires a multimodal approach, including psychoeducation for families, pharmacological interventions, psychotherapeutic interventions, social skills interventions, and appropriate educational placement. A recent randomized controlled trial investigated the effectiveness of several psychosocial interventions on youth in an early prodromal stage, characterized by changes in cognitive and social behavior. The interventions, termed integrated psychological interventions, specifically included cognitive-behavioral therapy, group skills training, cognitive remediation therapy, multifamily psychoeducation, and supportive counseling on the prevention of psychosis. Of interest, the integrated psychological intervention was shown to be more effective than standard treatments in delaying the onset of psychosis over a 2-year follow-up period. These results sparked interest in the potential utility of psychosocial interventions to mediate psychosis, and to alter relapse rate and severity of illness over time. Children with childhood-onset schizophrenia may have less robust responses to antipsychotic medications than adolescents and adults. Family education and ongoing therapeutic family interventions are critical to maintain the maximum level of support for the patient. Monitoring the most appropriate educational setting for a child with childhood-onset schizophrenia is essential, especially in view of the frequent social skill deficits, attention deficits, and academic difficulties that often accompany childhood-onset schizophrenia. Pharmacotherapy Second-generation antipsychotics, serotonin-dopamine antagonists, are current mainstay pharmacological treatments for children and adolescents with schizophrenia, having largely replaced the conventional antipsychotics, that is, dopamine receptor antagonists, due to their more favorable side-effect profiles. Current data include six randomized clinical trials in youth investigating the efficacy of second-generation antipsychotics for early-onset schizophrenia, with limited support for one agent over the others. Although clozapine, a serotonin receptor antagonist with some dopamine (D2) antagonism, which is hypothesized to be more effective in reducing positive and negative symptoms, has been shown to be highly effective in adults with treatmentrefractory schizophrenia, it remains a choice of last resort in youth, based on its serious side effects. To date, however, evidence from multisite randomized clinical trials supports some efficacy of risperidone, olanzapine, aripiprazole, and clozapine in the treatment of childhood- and adolescent-onset schizophrenia. Two randomized clinical trials using risperidone in adolescents with schizophrenia found risperidone at doses up to 3 mg per day to be superior to placebo. A multisite randomized 6-week controlled trial of olanzapine in adolescents with schizophrenia found that it was more efficacious than placebo. A randomized controlled trial of aripiprazole at two fixed doses found that it was superior to placebo in the treatment of positive symptoms of adolescent schizophrenia; however, more than 40 percent of subjects in the active medication group did not achieve remission. Finally, clozapine has been demonstrated to be more effective than haloperidol in improving both positive and negative symptoms in treatment-resistant schizophrenia in youth. More recently, a study compared clozapine to high doses of olanzapine and found that response rates were about twice as great for clozapine as olanzapine (66% vs. 33%) when response was defined by a 30% or greater reduction in symptoms on the Brief Psychiatric Rating Scale and improvement on the Clinical Global Impression Scale. The Treatment of Early Onset Schizophrenia Spectrum Disorders Study compared the efficacy of risperidone and olanzapine with those of molindone, a mid-potency conventional antipsychotic. In this study, lacking a placebo group, each of these agents provided a similar therapeutic effect; however, fewer than half of the patients responded optimally. Despite the limited randomized controlled studies of second-generation antipsychotics for the treatment of schizophrenia in youth, the Food and Drug Administration (FDA) is progressively approving the use of these agents for pediatric schizophrenia and bipolar illness. In 2007, the FDA approved the use of risperidone and aripiprazole for the treatment of schizophrenia in 13- to 17-yearolds. The use of olanzapine and quetiapine were approved by the FDA in 2009 in the treatment of schizophrenia in 13 to 17 year olds. A double-blind, randomized 8-week controlled trial compared the efficacy and safety of olanzapine to clozapine in childhood-onset schizophrenia. Children with childhoodonset schizophrenia who were resistant to at least two previous treatments with antipsychotics were randomized to treatment for 8 weeks with either olanzapine or clozapine followed by a 2-year open-label follow-up. Using the Clinical Global Impression of Severity of Symptoms Scale and Schedule for the Assessment of Negative/Positive Symptoms, clozapine was found to be associated with a significant reduction in all outcome measures, whereas olanzapine showed improvement on some measures but not on all. The only statistically significant measure in which clozapine was superior to olanzapine was in alleviating negative symptoms, compared with baseline. Clozapine was associated with more adverse events, such as lipid abnormalities and a seizure in one patient. Several studies have provided evidence that risperidone, a benzisoxazole derivative, is as effective as the older high-potency conventional antipsychotics, such as haloperidol (Haldol), and causes less frequent severe side effects, in the treatment of schizophrenia in older adolescents and adults. Published case reports and limited larger controlled studies have supported the efficacy of risperidone in the treatment of psyhosis in children and adolescents. Risperidone has been reported to cause weight gain and dystonic reactions and other extrapyramidal adverse effects in children and adolescents. Olanzapine is generally well tolerated with respect to extrapyramidal adverse effects compared with conventional antipsychotics and risperidone, but it is associated with moderate sedation and significant weight gain. Psychosocial Interventions Psychosocial interventions aimed at family education and patient and family support are recognized as critical components of the treatment plan for childhood-onset schizophrenia. Although there are not yet randomized controlled trials of psychosocial interventions in children and adolescents with schizophrenia, family therapy, psychoeducation, and social skills training have been shown to lead to improved clinical symptoms in young adults with a first episode of schizophrenia, and reviews of the adult literature support the benefit of cognitive behavioral therapy, and cognitive remediation as adjunctive treatments to pharmacologic agents in adults. 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Hyperprolinemia is not associated with childhood onset schizophrenia. Am J Med Genet B Neuropsychiatr Genet. 2006;141:192. Kryshanovskaya L, Schulz C, McDougle C et al. Olanzapine versus placebo in adolescents with schizophrenia: a 6-week, randomized, double-blind, placebo-controlled trial. J Am Acad Child Adolesc Psychiatry. 2009;48:60–70. Kumra S, Kranzler H, Gerbine-Rosen G, Kester H,M, De Thomas C, Kafantaris V, Correll C, Kane J. Clozapine and ‘highdose’ olanzapine in refractory early-onset schizophrenia: A 12-week randomized and double-blind comparison. Biol Psychiatry. 2008;63:524–529. Sikich L. Early onset psychotic disorders. In: Sadock BJ, Sadock VA, Ruiz P, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Vol. 2. Philadelphia: Lippincott Williams & Wilkins; 2009:3699. McGurk SR, Twamlety EW, Sitezer DL, McHugo JG, Mueser KT. A meta-analysis of cognitive remediation in schizophrenia. Am J Psychiatry. 2007;164:1791–1802. 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Neurobiology and phenotypic expression in early-onset schizophrenia. Early Interv Psychiatry. 2011;5:3–14. 31.16 Adolescent Substance Abuse Substance use is a public health concern among American youth. The most common substances used by adolescents in the United States are tobacco, alcohol, and marijuana. Adolescent substance use and abuse, however, includes a wider range of substances, including cocaine, heroin, inhalants, phencyclidine (PCP), lysergic acid diethylamide (LSD), dextromorphan, anabolic steroids and various club drugs, 3,4methylenedioxymethamphetamine (MDMA or Ecstasy), flunitrazepam (Rohypnol), gamma-hydroxybutyrate (GHB), and ketamine (Ketalar). It is estimated that approximately 20 percent of 8th graders in the United States have tried illicit drugs and about 30 percent of 10th through 12th graders have used an illicit substance. Alcohol remains the most common substance used and abused by adolescents. Binge drinking occurs in about 6 percent of adolescents, and teens with alcohol use disorders are at greater risk of problems with other substances as well. The American Psychiatric Association’s Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5), in contrast to the previous Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition, Text Revision (DSM-IV-TR), does not separate the diagnoses of substance abuse from substance dependence. Instead, the DSM-5 provides criteria for substance use disorder, accompanied by criteria for intoxication, withdrawal, and substance-induced disorders. The previous DSM-IV-TR criterion of recurrent substance-related legal problems has been deleted in the DSM-5, and a new criterion, craving, or a strong desire or urge to use a substance, has been added. In the DSM-5, a threshold of two or more criteria must be present. Cannabis withdrawal and caffeine withdrawal are new disorders in the DSM-5. The combined substance use criteria including both abuse and withdrawal phenomena may strengthen the validity of the disorder in adolescents, and the elimination of the criterion for “legal problems” is also an appropriate change for adolescents, since this is less common for younger adolescents and for adolescent females who use substances. Two recent commentaries raise concerns regarding the application of DSM-5 criteria to adolescents with respect to the symptom of tolerance, particularly to alcohol, that may occur across the board, and may be developmentally normal for adolescents who use alcohol but for whom there is no clinical impairment, and for withdrawal symptoms, which may have clinical significance but is only moderately associated with level of severity of substance use. Many risk and protective factors influence the age of onset and severity of substance use among adolescents. Psychosocial risk factors mediating the development of substance use disorders include parent modeling of substance use, family conflict, lack of parental supervision, peer relationships, and individual stressful life events. Protective factors that mitigate substance use among adolescents include variables such as a stable family life, strong parent–child bond, consistent parental supervision, investment in academic achievement, and a peer group that models prosocial family and school behaviors. Interventions that diminish risk factors are likely to mitigate substance use. Approximately one of five adolescents has used marijuana or hashish. Approximately one third of adolescents have used cigarettes by age 17 years. Studies of alcohol use among adolescents in the United States have shown that by 13 years of age, one third of boys and almost one fourth of girls have tried alcohol. By 18 years of age, 92 percent of males and 73 percent of females reported trying alcohol, and 4 percent reported using alcohol daily. Of high school seniors, 41 percent reported using marijuana; 2 percent reported using the drug daily. Drinking among adolescents follows adult demographic drinking patterns: The highest proportion of alcohol use occurs among adolescents in the northeast; whites are more likely to drink than are other groups; among whites, Roman Catholics are the least likely nondrinkers. The four most common causes of death in persons between the ages of 10 and 24 years are motor vehicle accidents (37 percent), homicide (14 percent), suicide (12 percent), and other injuries or accidents (12 percent). Of adolescents treated in pediatric trauma centers, more than one third are treated for alcohol or drug use. Studies considering alcohol and illicit drug use by adolescents as psychiatric disorders have demonstrated a greater prevalence of substance use, particularly alcoholism, among biological children of alcoholics than among adopted youth. This finding is supported by family studies of genetic contributions, by adoption studies, and by observing children of substance users reared outside the biological home. Numerous risk factors influence the emergence of adolescent substance abuse. These include parental belief in the harmlessness of substances, lack of anger control in families of substance abusers, lack of closeness and involvement of parents with children’s activities, maternal passivity, academic difficulties, comorbid psychiatric disorders such as conduct disorder and depression, parental and peer substance use, impulsivity, and early onset of cigarette smoking. The greater the number of risk factors, the more likely it is that an adolescent will be a substance user. EPIDEMIOLOGY Alcohol The Centers for Disease Control and Prevention Youth Risk Behavior Survey found that 72.5 percent of high school students had tried at least one alcoholic drink, and 24.2 percent reported an episode of heavy drinking in the month preceding the survey. Findings from the Monitoring the Future Survey suggest that about 39 percent of adolescents have used alcohol before the 8th grade. Another survey found that drinking was a significant problem for 10 to 20 percent of adolescents. Drinking was reported by 70 percent of 8th grade students: 54 percent reported drinking within the past year, 27 percent reported having gotten drunk at least once, and 13 percent reported binge drinking in the 2 weeks before the survey. By the 12th grade, 88 percent of high school students reported drinking, and 77 percent drank within the past year; 5 percent of 8th grade students, 1.3 percent of 10th grade students, and 3.6 percent of 12th grade students reported daily alcohol use. In the age range of 13 to 17 years, in the United States, reports indicate there are 3 million problem drinkers and 300,000 adolescents with alcohol dependence. The gap between male and female alcohol consumers is narrowing. Marijuana For the last two decades, marijuana has been one of the most widely used drugs by young people in developed countries, and recently it has become highly used globally. The United Nations Office on Drugs and Crime estimated that marijuana was used by 3.9 percent of people worldwide between ages 15 years and 64 years. Marijuana is the most commonly used illicit drug among high school students in the United States. It is estimated that about 10 percent of those who try marijuana become daily users, and 20 to 30 percent become weekly users. Marijuana has been termed a “gateway drug,” because the strongest predictor of future cocaine use is frequent marijuana use during adolescence. Of 8th grade, 10th grade, and 12th grade students, 10, 23, and 36 percent, respectively, report using marijuana, a slight decrease from the year preceding the survey. Of 8th grade, 10th grade, and 12th grade students, 0.2, 0.8, and 2 percent, respectively, report daily marijuana use. Prevalence rates for marijuana are highest among Native American males and females; these rates are nearly as high in white males and females and Mexican American males. The lowest annual rates are reported by Latin American females, African American females, and Asian American males and females. Cocaine The annual cocaine use reported by high school seniors decreased more than 30 percent between 1990 and 2000. Currently, about 0.5 percent of 8th grade students, 1 percent of 10th grade students, and 2 percent of 12th grade students are estimated to have used cocaine. The prevalence rates for crack cocaine use, however, is increasing and is most common among those between the ages of 18 and 25. Crystal Methamphetamine Crystal methamphetamine, or “ice,” was at a relative low level of use in adolescence about one decade ago of 0.5 percent, and has steadily increased to a recent rate of 1.5 percent among 12th graders. Opioids A survey of 7,374 high school seniors found that 12.9 percent reported nonmedical use of opioids. Of users, more than 37 percent reported intranasal administration of prescription opioids. Lysergic Acid Diethylamide (LSD) Lysergic acid diethylamide is reportedly used by 2.7 percent of 8th grade students, 5.6 percent of 10th grade students, and 8.8 percent of 12th grade students. Of 12th grade students, 0.1 percent report daily use. The current LSD rates are lower than rates of LSD use during the past two decades. 3,4-Methylenedioxymethamphetamine (MDMA) The popularity of MDMA has increased over the last decade, and current rates of use in the United States are in the range of about 5 percent for 10th graders and 8 percent of 12th graders, despite that the perceived harmfulness of this drug has increased over the last decade to almost 50 percent among 12th graders. Accidental adolescent deaths have been associated with the use of MDMA. Gamma-Hydroxybutyrate (GHB) Gamma-hydroxybutyrate, a club drug, has been found in surveys to have an annual prevalence rate of 1.1 percent for 8th graders, 1.0 percent rate for 10th graders, and a 1.6 percent rate of use for 12th graders. Ketamine (Ketalar) Ketamine, another club drug, was found recently to have a rate of 1.3 percent annual prevalence for 8th graders, 2.1 percent for 10th graders, and 2.5 percent rate for 12th graders. Flunitrazepam (Rohypnol) Flunitrazepam (Rohypnol), a third club drug, has been found to have an annual prevalence rate of about 1 percent for all high school grades combined. Anabolic Steroids Despite reported knowledge of the risks of anabolic steroids among high school students, surveys over the last 5 years found rates of anabolic steroid use to be 1.6 percent among 8th graders and 2.1 percent among 10th graders. Up to 45 percent of 10th and 12th graders reported knowledge of the risks of anabolic steroids; however, over the last decade it appears that high school seniors reported less disapproval of their use. Inhalants The use of inhalants in the form of glue, aerosols, and gasoline is relatively more common among younger than older adolescents. Among 8th grade, 10th grade, and 12th grade students, 17.6, 15.7, and 17.6 percent, respectively, report using inhalants; 0.2 percent of 8th grade students, 0.1 percent of 10th grade students, and 0.2 percent of 12th grade students report daily use of inhalants. Multiple Substance Use Among adolescents enrolled in substance abuse treatment programs, 96 percent are polydrug users; 97 percent of adolescents who abuse drugs also use alcohol. ETIOLOGY Genetic Factors The concordance for alcoholism is reportedly higher among monozygotic than dizygotic twins. Considerably fewer studies have been conducted of families of drug abusers. One twin study of drug users showed that the drug abuse concordance for male monozygotic twins was twice that for dizygotic twins. Studies of children of alcoholics reared away from their biological homes have shown that these children have about a 25 percent chance of becoming alcoholics. Psychosocial Factors Among adolescents, substance use, particularly marijuana use, is strongly influenced by peers, and especially for those adolescents who report using marijuana for relaxation, the drug is used to escape from stress, and as a social activity. There are data to suggest, however, that marijuana use is also associated with both social anxiety disorder and depressive symptoms. Among young adolescents who start using alcohol, tobacco, and marijuana at an early age, data suggest that they often come from families with low parental supervision. The risk of early initiation of substances is greatest for children below 11 years of age. Increased parental supervision during middle childhood years may diminish drug and alcohol sampling and ultimately diminish the risk of using marijuana, cocaine, or inhalants in the future. Comorbidity Rates of alcohol and marijuana use are reportedly higher in relatives of youth with depression and anxiety disorders. On the other hand, mood disorders are common among those with alcoholism. Evidence indicates another strong link between early antisocial behavior, conduct disorder, and substance abuse. Substance abuse can be viewed as one form of behavioral deviance that, unsurprisingly, is associated with other forms of social and behavioral deviance. Early intervention with children who show early signs of social deviance and antisocial behavior may conceivably impede the processes that contribute to later substance abuse. Comorbidity, the occurrence of more than one substance use disorder or the combination of a substance use disorder and another psychiatric disorder, is common. It is important to know about all comorbid disorders, which may show differential responses to treatment. Surveys of adolescents with alcoholism show rates of 50 percent or higher for additional psychiatric disorders, especially mood disorders. A recent survey of adolescents who used alcohol found that more than 80 percent met criteria for another disorder. The disorders most frequently present were depressive disorders, disruptive behavior disorders, and drug use disorders. These rates of comorbidity are even higher than those for adults. The diagnosis of alcohol abuse or dependence was likely to follow, rather than precede, other disorders; that a large proportion of adolescents with alcoholism have a previous childhood disorder may have both etiological and treatment implications. In this survey, the onset of alcohol disorders did not systematically precede drug abuse or dependence. In 50 percent of cases, alcohol use followed drug use. Alcohol use may be a gateway to drug use, but is not in most cases. The presence of other psychiatric disorders was associated with an earlier onset of alcohol disorder, but it did not seem to indicate a more protracted course of alcoholism. DIAGNOSIS AND CLINICAL FEATURES According to the DSM-5, substance-related disorders include the following three categories: substance use, substance intoxication, and substance withdrawal disorder. Whereas in DSM-IV-TR, substance abuse and dependence were two separate categories, in DSM-5, they are combined in one diagnosis called substance use disorder. Substance use refers to a maladaptive pattern of substance use leading to clinically significant impairment or distress, manifest by one or more of the following symptoms within a 12-month period: recurrent substance use in situations that causes physical danger to the user, recurrent substance use in the face of obvious impairment in school or work situations, recurrent substance use despite resulting legal problems, or recurrent substance use despite social or interpersonal problems. Substance intoxication refers to the development of a reversible, substance-specific syndrome caused by use of a substance. Clinically significant maladaptive behavioral or psychological changes must be present. Substance withdrawal refers to a substance-specific syndrome caused by the cessation of, or reduction in, prolonged substance use. The substance-specific syndrome causes clinically significant distress or impairs social or occupational functioning. Two new disorders in DSM-5 include Cannabis withdrawal disorder and caffeine withdrawal disorder. The diagnosis of alcohol or drug use in adolescents is made through careful interview, observations, laboratory findings, and history provided by reliable sources. Many nonspecific signs may point to alcohol or drug use, and clinicians must be careful to corroborate hunches before jumping to conclusions. Substance use can be viewed on a continuum with experimentation (the mildest use), regular use without obvious impairment, abuse, and finally, dependence. Changes in academic performance, nonspecific physical ailments, and changes in relationships with family members, changes in peer group, unexplained phone calls, or changes in personal hygiene may indicate substance use in an adolescent. Many of these indicators, however, also can be consistent with the onset of depression, adjustment to school, or the prodrome of a psychotic illness. It is important, therefore, to keep the channels of communication with an adolescent open when substance use is suspected. Nicotine Nicotine is one of the most addictive substances known; it involves cholinergic receptors, and enhancing acetylcholine, serotonin, and β-endorphin release. Young teens who smoke cigarettes are also exposed to other drugs more frequently than nonsmoking peers. Alcohol Alcohol use in adolescents rarely results in the sequelae observed in adults with chronic use of alcohol, such as withdrawal seizures, Korsakoff’s syndrome, Wernicke’s aphasia, or cirrhosis of the liver. One report, however, has stated that adolescent exposure to alcohol may result in diminished hippocampal brain volume. Because the hippocampus is involved with attention, it is conceivable that adolescent alcohol use could result in compromised cognitive function, especially with respect to attention. Marijuana The short-term effects of the active ingredient in marijuana, tetrahydrocannabinol (THC), include impairment in memory and learning, distorted perception, diminished problem-solving ability, loss of coordination, increased heart rate, anxiety, and panic attacks. Abrupt cessation of heavy marijuana use by adolescents has been reported to result in a withdrawal syndrome characterized by insomnia, irritability, restlessness, drug craving, depressed mood, and nervousness followed by anxiety, tremors, nausea, muscle twitches, increased sweating, myalgia, and general malaise. Typically, the withdrawal syndrome begins 24 hours after the last use, peaks at 2 to 4 days, and diminishes after 2 weeks. Marijuana use has been associated with increased risk of psychiatric disorders. Poor cognitive functioning has been associated with chronic marijuana use, although it is not clear whether marijuana impairs cognitive function. Deficits in verbal learning, memory, and attention have been reported in chronic marijuana users, and both acute and chronic marijuana use is associated with changes in cerebral blood flow to certain brain regions, which can be detected by positron emission tomography. Functional imaging studies suggest that there is less activity in brain regions involved with attention and memory in chronic marijuana users. A 15-year follow-up of 50,465 Swedish males in the military reported that participants who had used marijuana by 18 years of age were 2.4 times more likely to develop schizophrenia. Risks associated with chronic marijuana use include higher rates of motor vehicle accidents, impaired respiratory function, increased risk of cardiovascular disease, and potential increased risk for psychotic symptoms and disorders. Cocaine Cocaine can be sniffed or snorted, injected, or smoked. Crack is the term given to cocaine after it has been changed to a free base for smoking. Cocaine’s effects include constriction of peripheral blood vessels, dilated pupils, hyperthermia, increased heart rate, and hypertension. High doses or prolonged use of cocaine can induce paranoid thinking. There is immediate risk of death secondary to cardiac arrest or from seizures followed by respiratory arrest. In contrast to stimulants used to treat attentiondeficit/hyperactivity disorder (ADHD), such as methylphenidate, cocaine quickly crosses the blood–brain barrier and moves off the dopamine transporter within 20 minutes; methylphenidate remains bound to dopamine for long periods. Heroin Heroin, a derivative of morphine, is produced from a poppy plant. Heroin usually appears as a white or brown powder that can be snorted, but more commonly, it is used intravenously. Withdrawal symptoms include restlessness, muscle and bone pain, insomnia, diarrhea and vomiting, cold flashes with goose bumps, and kicking movements. Withdrawal occurs within a few hours after use; symptoms peak between 48 and 72 hours later and remit within about a week. Club Drugs Adolescents who frequent nightclubs, raves, bars, or music clubs also frequently use MDMA, GHB, Rohypnol, and ketamine. GHB, Rohypnol (a benzodiazepine), and ketamine (an anesthetic) are primarily depressants and can be added to drinks without detection because they are often colorless, tasteless, and odorless. The Drug-Induced Rape Prevention and Punishment Act was passed after these drugs were found to be associated with date rape. MDMA is a derivative of methamphetamine, a synthetic with both stimulant and hallucinogenic properties. MDMA can inhibit serotonin and dopamine reuptake. MDMA can result in dry mouth, increased heart rate, fatigue, muscle spasm, and hyperthermia. Lysergic Acid Diethylamide LSD is odorless, colorless, and has a slightly bitter taste. Higher doses of LSD can produce visual hallucinations and delusions and, in some cases, panic. The sensations experienced after ingestion of LSD usually diminish after 12 hours. Flashbacks can occur up to 1 year after use. LSD can produce tolerance; that is, after multiple uses, more is needed to provide the same degree of intoxication. Substance use is related to a variety of high-risk behaviors, including early sexual experimentation, risky driving, destruction of property, stealing, “heavy metal” or alternative music, and, occasionally, preoccupation with cults or Satanism. Although none of these behaviors necessarily predicts substance use, at the extreme, these behaviors reflect alienation from the mainstream of developmentally expected social behavior. Adolescents with inadequate social skills may use a substance as a modality to join a peer group. In some cases, adolescents begin their substance use at home with their parents, who also use substances to enhance their social interactions. Although no evidence indicates what determines a typical adolescent user of alcohol or drugs, many substance users seem to have underlying social skills deficits, academic difficulties, and less than optimal peer relationships. TREATMENT Interventions for substance use disorders in adolescents first require effective screening and identification of those teens in need of treatment. Once a substance use disorder has been identified in a teen, a variety of treatment options can be sought. In accordance with the goals of the U.S Substance Abuse Mental Health Services Administration (SAMHSA), a school-based alcohol and drug Screening, Brief Intervention, and Referral to Treatment (SBIRT) has been initiated in a study with 629 adolescents ages 14 to 17 years in 13 participating high schools in New Mexico. Initially, school-based health centers provided substance use screenings for all students who were seen in the clinic for any reason. Once identified, substance using adolescents were offered either brief intervention by clinic staff (85.1 percent of those identified), whereas 14.9 percent received brief treatment or referral to treatment. The brief intervention was based on motivational interviewing, with the goal of helping the student to gain motivation for behavioral change, and being referred for more intensive treatment if needed. Students who received the intervention, regardless of the severity of their substance use, reported decreases in self-reported drinking to intoxication at the 6-month follow-up. Furthermore, students who reported drug use, self-reported decreased use at follow-up. Alcohol use was reported by 42 percent of the student participants, and alcohol intoxication was reported by 37 percent. Eighty-five percent of study participants who reported drug use, reported only marijuana use in the month prior to entering the study. The frequency of alcohol and marijuana as the most predominant substances in this age group is consistent with epidemiological data. Overall, this school-based intervention had the advantage of being easily accessible to adolescents and provided a graded option for treatment according to the severity of the substance use. This study suggests that school-based programs for identifying and providing brief interventions for high school students is viable and merits further study. Treatment of substance use disorders in adolescents is designed to directly prevent the substance use behaviors and to provide education for the patient and family and to address cognitive, emotional, and psychiatric factors that influence the substance use in a variety of settings such as a residential milieu, group, and individual psychosocial session. One validated instrument used as a guide for clinicians in the treatment of adolescent substance use designates levels of care appropriate for the symptoms. This instrument called the Child and Adolescent Levels of Care Utilization Services (CALOCUS) outlines six levels of care: Level 0: Basic services (prevention) Level 1: Recovery maintenance (relapse prevention) Level 2: Outpatient (once per week visits) Level 3: Intensive outpatient (2 or more visits per week) Level 4: Intensive integrated services (day treatment, partial hospitalization, wraparound services) Level 5: Nonsecure, 24-hour medically monitored service (group home, residential treatment facility) Level 6: Secure 24-hour medical management (inpatient psychiatric or highly programmed residential facility) Treatment settings that serve adolescents with alcohol or drug use disorders include inpatient units, residential treatment facilities, halfway houses, group homes, partial hospital programs, and outpatient settings. Basic components of adolescent alcohol or drug use treatment include individual psychotherapy, drug-specific counseling, self-help groups (Alcoholics Anonymous [AA], Narcotics Anonymous [NA], Alateen, Al-Anon), substance abuse education and relapse prevention programs, and random urine drug testing. Family therapy and psychopharmacological intervention may be added. Before deciding on the most appropriate treatment setting for a particular adolescent, a screening process must take place in which structured and unstructured interviews help to determine the types of substances being used and their quantities and frequencies. Determining coexisting psychiatric disorders is also critical. Rating scales are typically used to document pretreatment and posttreatment severity of abuse. The Teen Addiction Severity Index (T-ASI), the Adolescent Drug and Alcohol Diagnostic Assessment (ADAD), and the Adolescent Problem Severity Index (APSI) are several severity-oriented rating scales. The T-ASI is broken down into dimensions that include a family function, school or employment status, psychiatric status, peer social relationships, and legal status. After most of the information about substance use and the patient’s overall psychiatric status has been obtained, a treatment strategy must be chosen and an appropriate setting must be determined. Two very different approaches to the treatment of substance abuse are embodied in the Minnesota model and the multidisciplinary professional model. The Minnesota model is based on the premise of AA; it is an intensive 12-step program with a counselor who functions as the primary therapist. The program uses self-help participation and group processes. Inherent in this treatment strategy is the need for adolescents to admit that substance use is problematic and that help is necessary. Furthermore, they must be willing to work toward altering their lifestyle to eradicate substance use. The multidisciplinary professional model consists of a team of mental health professionals that usually is led by a physician. Following a case-management model, each member of the team has specific areas of treatment for which he or she is responsible. Interventions may include cognitive-behavioral therapy, family therapy, and pharmacological intervention. This approach usually is suited for adolescents with comorbid psychiatric diagnoses. Cognitive-behavioral approaches to psychotherapy for adolescents with substance use generally require that adolescents be motivated to participate in treatment and refrain from further substance use. The therapy focuses on relapse prevention and maintaining abstinence. Psychopharmacological interventions for adolescent alcohol and drug users are still in their early stages. The presence of mood disorders clearly indicates the need for antidepressants, and generally, the selective serotonin reuptake inhibitors are the first line of treatment. Occasionally, an intervention is made to substitute the illicit drug with another drug that is more amenable to the treatment situation; for example, using methadone instead of heroin. Adolescents are required to have documented attempts at detoxification and consent from an adult before they can enter such a treatment program. Peter, a 16-year-old 11th grader, was admitted to substance abuse treatment for the second time, following a relapse and threats of suicide. He was initially admitted to an adolescent psychiatric inpatient unit following a serious suicide attempt. Peter reported a longstanding history of ADHD, but he had been a good student and not had any difficulties until middle school. Peter reported an onset of substance use at age 13 years, rapid progression in substance involvement since age 14 years, and then current use of marijuana on a daily basis, drinking alcohol up to five times each week, and experimentation with a variety of substances, such as LSD and Ecstasy. After being discharged from the psychiatric hospital, Peter attended teen group sessions focusing on his substance use problems. Family sessions led to the realization that Peter’s mother had been depressed for some time, and she entered into her own treatment. Peter was improving with respect to his substance use; however, his depressive symptoms increased following 4 weeks of abstinence. Peter was started on fluoxetine (Prozac). After the medication was titrated to 30 mg, he remained on it for a month at which time he showed improvement in mood and treatment compliance. Peter continued to attend the teen AA meetings and outpatient therapy. Family conflict soon recurred, however, and Peter became noncompliant with outpatient treatment, medication, and meetings. He resumed old relationships with substance using peers and relapsed into daily marijuana use and occasional alcohol use. (Courtesy of Oscar G. Bukstein, M.D.) Efficacious treatments for cigarette smoking cessation include nicotine-containing gum, patches, or nasal spray or inhaler. Bupropion (Zyban) aids in diminishing cravings for nicotine and is beneficial in the treatment of smoking cessation. Because comorbidity influences treatment outcome, it is important to pay attention to other disorders, such as mood disorders, anxiety disorders, conduct disorder, or ADHD during the treatment of substance use disorders. REFERENCES Buckner JD, Heimberg RG, Schneier FR, Liu SM, Want S, Blanco C. The relationship between cannabis use disorder and social anxiety disorder in the National Epidemiologic Study of Alcohol and Related Conditions (NESARC). Drug Alcohol Depend. 2012;124:128–134. Bukstein O. Adolescent substance abuse. In: Sadock BJ, Sadock VA, Ruiz P, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Vol. II. Philadelphia: Lippincott Williams & Wilkins; 2009:3818. Centers for Disease Control and Prevention, 2009. Youth Risk Behavior Survey. Updated February 22, 2011. Fiorentini A, Volunteri LS, Draogna F, Rovera C, Maffini M, et al. Substance-induced psychoses: A critical review of the literature. Curr Drug Abuse Rev. 2011;4:228–240. Fraser S, Hides L, Philips L, Proctor D, Lubman DI. Differentiating first episode substance induced primary psychotic disorders with concurrent substance use in young people. Schizophr Res. 2012;136:110–115. Giedd J, Stocvkman M Weele C. Anatomic magnetic resonance imaging of the developing child and adolescent brain. In: Reyna VF; Chapman SB, Dougherty MR, Copnfrey J., eds. The Adolescent Brain: Learning, Reasoning, and Decision Making. Washington, D.C: American Psychological Association; 2012. Harrow BS, Tompkins CP, Mitchell PD, Smith KW, Soldz S, Kasten L, Fleming K. The impact of publicly funded managed care on adolescent substance abuse treatment outcomes. Am J Drug Alcohol Abuse. 2006;32(3):379. Johnston LD, O’Malley PM, Bachman JG, Schulenberg JE. Monitoring the Future: National Survey Results on Drug Use. 1975–2007. Vol 3 Secondary School Students. Bethesda, MD. National Institute on Drug Abuse; 2008. Kaminer Y, Winters KC. Proposed DSM-5 substance use disorders for adolescents: If you build it, will they come? Am J Addict. 2012;21:280–281. Lenk KM, Erickson DJ, Wonters KC, Nelson TF, Toomey TL. Screening services for alcohol misuse and abuse at four-year colleges in the U.S. J Subst Abuse Treat. 2012;43:352–358. McCabe SE, West BT, Teter CJ, Boyd CJ. Medical and nonmedical use of prescription opioids among high school seniors in the United States. Arch Pediatr Adolesc Med. 2012;166:797–802. Mitchell SG, Gryczynski J, Gonzales A, Moseley A, Peterson T, et al. Screening, brief intervention, and referral to treatment (SBIRT) for substance use in a school-based program: Services and outcomes. Am J Addict. 2010;21:S5–S13. Tavolacci MP, Ladner J, Grigioni S, Richard L, Villet H, Dechelotte P. Prevalence and association of perceived stress, substance use and behavioral addictions: A cross-sectional study among university students in France, 2009–2011. BMC 40 - 31.17 Child Psychiatry Other Conditions 31.17 Child Psychiatry: Other Conditions 41 - 31.17a Attenuated Psychosis Syndrome 31.17a Attenuated Psychosis Syndrome Pub Health. 2013;13:724–732. Winters K. Advances in the science of adolescent drug involvement: Implications for assessment and diagnosis. Curr Opin Psychiatry. 2012;318–324. Winters KC, Martim CS, Chung T. Substance use disorders in DSM-V. When applied to adolescents. Addiction. 2011;106:882–884. Yuma-Guerrero PJ, Lawson KA, Velasquez MM, von Sternberg K, Maxson T, et al. Screening, brief intervention, and referral for alcohol use in adolescents: A systematic review. Pediatrics. 2012;130:115–122. 31.17 Child Psychiatry: Other Conditions 31.17a Attenuated Psychosis Syndrome Attenuated Psychosis Syndrome (APS) is a new diagnostic category included in the American Psychiatric Association’s Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5) as a condition for further study. It is a syndrome characterized by subthreshold psychotic symptoms, less severe than those found in psychotic disorders, but which are often present in prodromal psychotic states. Debate and controversy among clinicians and researchers have surrounded the inclusion of APS in the DSM-5. There are those who believe that the identification and treatment of a prodromal syndrome of a psychotic disorder would either delay or diminish the severity of the future psychotic illness. And, there are others who believe that identification of a prodromal syndrome, which may rarely if ever progress to a full psychotic illness, would lead to unnecessary exposure to antipsychotic agents with unpredictable and possibly harmful effects. There is agreement, however, that patients with subthreshold prodromal psychotic symptoms are often impaired and are in need of psychological and psychiatric intervention. A recent meta-analysis reported that the rate of onset of psychotic disorders in those patients with prodromal psychotic symptoms was 18 percent at 6 months, 22 percent at 1 year, 29 percent at 2 years, and 36 percent at 3 years. In a follow up study it was found that of those with prodromal symptoms who went on to develop a threshold psychotic illness, 73 percent met criteria for schizophrenia. In children and adolescents psychotic symptoms are not necessarily a hallmark of a threshold psychotic disorder compared to adults. For example, in 50 percent of children with major depressive episodes, psychotic symptoms were present. In addition, epidemiological studies have found that globally, auditory hallucinations occur in 9 percent to 21 percent of children and in 8.4 percent of adolescents. Thus, in youth, the association between subthreshold psychotic symptoms and the emergence of future psychotic illness may not be a reliable predictor. Nevertheless, identification and follow up of youth with APS may provide an increased understanding of the longitudinal significance of these symptoms. ETIOLOGY Genetic Factors Family studies have demonstrated that genetic factors influence vulnerability for schizophrenia spectrum disorders and other psychotic disorders. To the extent that APS and schizophrenia are related, genetic contributions are likely to be significant. Adoption and twin studies have confirmed that monozygotic twins have about a 50 percent concordance rate for schizophrenia compared to dizygotic twins who have a concordance rate of about 10 percent. In addition, adopted children of parents with schizophrenia do not have higher rates of schizophrenia; but biological children of schizophrenic parents do. However, genetic factors do not account fully for the emergence of schizophrenia spectrum disorders, since there is only a 50 percent concordance of exhibiting these disorders among monozygotic twins. Environmental factors also play an important role. Environmental Factors Early environmental factors that increase the risk of developing schizophrenia include fetal malnutrition, hypoxia at birth, and possibly prenatal infections. Other environmental factors include trauma, stress, social adversity and isolation. Finally, gene–environment interactions may influence an individual’s sensitivity to adverse environmental events. DIAGNOSIS Attenuated psychosis syndrome, according to DSM-5, is based on the presence of at least one of the following: delusions, hallucinations, or disorganized speech, which causes functional impairment. Although the symptoms may not have progressed to full psychotic severity, they must have been present at least once per week for one month, and must have emerged or worsened in the past year. The symptoms must cause impairment and warrant clinical attention. Attenuated delusions are described as either suspiciousness, persecutory, or grandiose, resulting in a lack of trust in others, and a sense of danger. Attenuated delusions, in contrast to delusions of threshold illness, may lead to loosely organized beliefs about hostile intentions of others, or danger; however, the delusions are not as fixed as they become in full blown psychotic illness. Attenuated hallucinations include altered sensory perceptions such as perception of murmurs, rumblings, or shadows that are disturbing; but they can be challenged, and skepticism about their reality is likely to be present. Disorganized communication or speech may be displayed as vague, or confused explanations, or circumstantial or tangential communication. When severe, but still in the attenuated range, thought blocking or loose associations may emerge; however, in contrast to psychotic illness, redirection is possible, and a logical conversation is typically achieved. Although impairment is present in APS, the individual retains an awareness and insight into the mental changes that are occurring. TREATMENT A recent review of the literature on treatment trials with patients at ultra-high-risk for psychosis found that early intervention with both psychological interventions and pharmacological agents can reduce symptoms and either delay or prevent the onset of a full psychotic illness. Other studies, however, found mixed results for early psychological or pharmacological interventions to prevent the onset of psychotic illness. One study found that most patients who became frankly psychotic did so within a few months after joining the study, making it difficult to determine if these patients were already exhibiting early signs of onset of schizophrenia when identified as prodromal. A variety of treatment approaches have been used including treatment with risperidone, olanzapine, omega-3 polyunsaturated fatty acid (w-3PUFA), cognitivebehavioral therapy (CBT), cognitive therapy (CT), and one using an integrated psychological intervention (IPI) including cognitive approaches, psychoeducation, and social skills intervention. A review of treatment effectiveness in APS found that receiving treatment was associated with lower risk of psychotic illness at 1 year, 2 years, and 3 years. Given the limited data, however, it is not clear which interventions are most efficacious. Therefore, until additional treatment trials provide efficacy data, the safest choices for treatment of APS includes psychological interventions rather than the use of antipsychotic agents. In summary, APS identifies a group of patients with psychotic-like phenomena that warrant interventions in order to improve their distress and functional levels. Further study is needed, however, to determine the relationship between APS and the development of schizophrenia and other psychotic illnesses. REFERENCES Amminger GP, Schafer MR, Papageorgiou K, Klier CM, Cotton SM. Long-chain w-3 fatty acids for indicated prevention of psychotic disorder: A randomized placebo-controlled trial. Arch Gen Psychiatry. 2010;67:146–14. Addington J, Epstein I, Liu L, French P, Boydell KM. A randomized controlled trial of cognitive behavioral therapy for individuals at clinical high risk of psychosis. Schizophr Res. 2011;125:54–61. Arango C. Attenuated psychotic symptoms syndrome: How it may affect child and adolescent psychiatry. Eur Child Adolesc Psychiatry. 2011;20:67–70. Bechdolf A, Wagner M, Ruhrman S, Harrigan S, Veith V, et al. Preventing progression to first episode psychosis in early initial prodromal states. Br J Psychiatry. 2012;200:22–29. Fusar-Poli P, Borgwardt S, Bechdolf A, Addington J, Riecher-Rossler A, et al. The psychosis high-risk state. A comprehensive state-of-the-art review. JAMA. 2013;70:107–120. Fusar-Poli P, Bechdolf A, Taylor M, Carpenter W, Yung A, McGuire P. At risk for schizophrenia or affective psychosis? A meta-analysis of DSM/ICD diagnostic outcomes in individuals at high clinical risk. [Published online May 15, 2012]. Schizophr Bull. Doi: 10.1093/schbul/sbs060. Fusar-Poli P, Bonoldi I, Yung AR. Predicting psychosis: A meta-analysis of transition outcomes in individuals at high clinical risk. Arch Gen Psychiatry. 2012;69:220–229. Jacobs E, Kline E, Schiffman J. Defining treatment as usual for attenuated psychosis syndrome: A survey of community practitioners. Psychiatr Serv. 2012;63:1252–1256. McGlashan TH, Zipursky RB, Perkins D, Addington J, Miller T, et al. Randomized, double-blind trial of olanzapine versus 42 - 31.17b Academic Problem 31.17b Academic Problem placebo in patients prodromally symptomatic for psychosis. Am J Psychiatry. 2006;163:790–799. McGorry PD, Nelson B, Amminger GP, Bechdolf A, Francey SM, et al. Intervention in individuals at ultra-high risk for psychosis: A review and future directions. J Clin Psychiatry. 2009;70:1206–1212. McGorry PD, Yung AR, Phillips LJ, Yuen HP, Francey S, et al. Randomized controlled trial of interventions designed to reduce the risk of progression to first episode psychosis in a clinical sample with subthreshold symptoms. Arch Gen Psychiatry. 2002;59:921–928. Morrison A, French P, Walford L, Lewis SW, Kilcommons A, et al. Cognitive therapy for the prevention of psychosis in people at ultra-high risk: Randomised controlled trial. Br J Psychiatry. 2004;185:291–297. Phillips LJ, Nelson B, Yuen HP, Francey SM, Simmons M. Randomized controlled trial of interventions for young people at ultra-high risk of psychosis; study design and baseline characteristics. Aust N Z J Psychiatry. 2009;43:818–829. Preti A. Cella M. Randomized-controlled trails in people at ultra high risk of psychosis: A review of treatment effectiveness. Schizophr Res. 2010;123:30–36. Shrivastava A, McGorry P, Tsuang M, Woods SW, Cornblatt BA, et al. “Attenuated psychotic symptoms syndrome” as a risk syndrome of psychosis, diagnosis in DSM-V: The debate. Indian J Psychiatry. 2011;53:57–65. Yung AR, Woods SW, Ruhrmann S, Addington J, Schultze-Lutter F. Wither the attenuated psychosis syndrome? Schizophr Bull. 2012;38:1130–1134. Yung AR, Phillips JL, Nelson B, Francey S, Panyuen H, et al. Randomized controlled trial of interventions for young people at ultra-high risk for psychosis: 6-month analysis. J Clin Psychiatry. 2011;72:430–440. 31.17b Academic Problem Academic underachievement or failure is a major public health concern in youth, affecting between 10 percent and 20 percent of youth, with long-ranging associations with high-risk behaviors and poorer adjustment in early adulthood. The DSM-5 includes the category Academic or Educational Problem in the section “other conditions that may be a focus of clinical attention,” since school failure requires clinical intervention and influences a child’s level of overall functioning. An investigation of the effects of students’ perception of support from parents, teachers, and peers showed a correlation with adolescent academic achievement. That is, adolescents’ perception of support from their teachers and parents was directly related to their academic achievement, whereas perceived peer support was indirectly related to actual academic achievement, it contributed to an adolescent’s overall perception of support, which was correlated to achievement. Academic difficulties and externalizing behavior problems have been found to coexist at higher rates than would be expected by chance. This association has been found in both clinical and epidemiological samples. A longitudinal study of academic underachievement and behavior problems in school-aged children from 1st grade to 6th grade found that the combination of academic and behavior problems in the 1st grade predicted continued academic difficulties and behavioral problems 5 years later. This combination was more frequently seen in boys than in girls, beginning with the 1st grade. This is also true for children with reading difficulties, attentional problems, and behavioral problems. Behavioral choices and life events can exacerbate academic problems in the absence of learning disorder and can interfere with lessening academic failures. For example, once a student perceives that he or she is falling behind academically, a greater temptation is to replace academic pursuits with other activities, such as drug use. A recent study assessed the level of, and deterioration in, academic achievement in relation to initiation of marijuana use among young teens. In a sample of rural teens, 36 percent of boys and 23 percent of girls initiated use of marijuana by the end of the 9th grade and that deteriorating academic performance was a significant predictor of initiating marijuana use. The hypothesis remaining to be tested is whether timely intervention to improve academic standing would lower the risk of beginning drug use. The DSM-5 Academic and Educational problem category is used when a child or adolescent is having significant academic difficulties that are not caused by a specific learning disorder or communication disorder or directly related to a psychiatric disorder. Nevertheless, intervention is necessary because the child’s achievement in school is significantly impaired, and this has an impact on the well-being of the child and may negatively influence concurrent psychiatric disorders. ETIOLOGY Many risk factors may play a role in academic underachievement or failure, including genetic factors, and developmental factors such as premature birth, as well as environmental factors such as level of maternal education. Very preterm children exhibit difficulties in working memory, which is a crucial ability and skill in learning new information and developing academic skills. Children and adolescents troubled by social isolation, identity issues, or extreme shyness may withdraw from full participation in academic activities. Academic problems may be the result of a confluence of multiple contributing factors and may occur in adolescents who were previously high academic achievers. School is the main social and educational venue for children and adolescents. Success and acceptance in the school setting depend on children’s physical, cognitive, social, and emotional adjustment. Children and adolescents’ competency in general coping with developmental tasks are reflected in their academic and social success in school. Anxiety can play a major role in interfering with children’s academic performance. Anxiety can hamper their ability to perform well on tests, to speak in public, and to ask questions when they do not understand something. Depressed youth also may withdraw from academic pursuits; they require specific interventions to improve their academic performance and to treat their depression. Youth consumed by family problems, such as financial troubles, marital discord in their parents, and mental illness in family members, may be distracted and unable to attend to academic tasks. Cultural and economic background can play a role in how well accepted a child feels in school and can affect the child’s academic achievement. Familial socioeconomic level, parental education, race, religion, and family functioning can influence a child’s sense of fitting in and can affect preparation to meet school demands. Schools, teachers, and clinicians can share insights about how to foster productive and cooperative environments for all students in a classroom. Teachers’ expectations about their students’ performance influence these performances. Teachers serve as agents whose varying expectations can shape the differential development of students’ skills and abilities. Such conditioning early in school, especially when negative, can disturb academic performance. A teacher’s affective response to a child, therefore, can prompt the appearance of an academic problem. Most important is a teacher’s humane approach to students at all levels of education, including medical school. DIAGNOSIS The DSM-5 contains the following statement about academic or educational problem: This category can be used when an academic or educational problem is the focus of clinical attention or has an impact on the individual’s diagnosis, treatment, or prognosis. Problems to be considered include illiteracy or low-level literacy; lack of access to schooling owing to unavailability or unattainability; problems with academic performance (e.g., failing school examinations, receiving failing marks, or grades) or underachievement (below what would be expected given the individual’s intellectual capacity); discord with teachers, school staff, or other students; and any other problems related to education and/or literacy. A 15-year-old 10th grade boy, Greg, with a history of prematurity and ADHD, was called to a meeting with his parents and school counselor due to his 12-week report card reflecting failure in two classes, and Cs and Ds in the rest. Until the end of 9th grade, Greg was a B and C student, and he had been stabilized for many years on his treatment for ADHD. In the 10th grade, however, since the beginning of the semester, Greg had not been able to keep up. His counselor had also noticed insidiously increasing isolative behavior for the past 2 months; previous evaluation of ADHD had included a full intellectual evaluation, which showed his full-scale intelligence quotient (IQ) to be 100 and revealed no specific areas of academic weakness. Discussion with his parents and school counselor revealed that Greg had become upset when his parents had announced that they would be separating. Greg had not been doing his homework, and felt that school was no longer relevant for his social life or future. After getting behind in his classes in the first 6 weeks of the semester, Greg stopped trying, feeling overwhelmed and demoralized. It was decided that Greg would given accommodations from his teachers so that he could pass his classes without having to hand in every assignment that had long passed. Greg would receive daily tutoring, and was referred for a psychiatric evaluation to determine the severity of his mood disorder. TREATMENT The initial step in determining a useful intervention for an academic problem is an evaluation of educational problems and psychosocial issues. Identifying and addressing family-, school-, and peer-related stressors are critical. An individualized evaluation may be indicated so that specific educational accommodations can be applied. In children with poor working memory, that is, a poor ability to store and retrieve information, learning and academic achievement is often impeded. Children with attention-deficit/hyperactivity disorder, as well as children born prematurely, often exhibit difficulties in working memory. In an effort to improve working memory in very preterm children, a computerized working memory training program (Cogmed) is being evaluated, consisting of 25 sessions of 35 minutes each, to be administered at home. Participants will undergo a baseline cognitive assessment, and then be randomized to either an adaptive or placebo version of Cogmed. Psychosocial intervention may be applied successfully for scholastic difficulties related to poor motivation, poor self-concept, and underachievement. In some cases, on the other hand, excessive hours spent in extracurricular activities, such as mandatory practices for multiple high school sports can result in compromised academic achievement. Early efforts to relieve academic problems are critical: Sustained problems in learning and school performance frequently are compounded and precipitate severe difficulties. Feelings of anger, frustration, shame, loss of self-respect, and helplessness— emotions that most often accompany school failures—damage self-esteem emotionally and cognitively, disabling future performance and clouding expectations for success. Generally, children with academic problems require either school-based intervention or individual attention. Tutoring on an individual and frequent basis is an effective technique for increasing academic production and is typically included in a comprehensive educational program. Tutoring has proved of value in preparing for standardized multiple choice examinations, such as the Scholastic Aptitude Test (SAT), as well as for increasing academic achievement in daily school subjects. Taking examinations, either school-based or standardized exams repetitively and using relaxation skills are techniques of great value in diminishing interference of test anxiety. REFERENCES Chen JJ. Relation of academic support from parents, teachers and peers to Hong Kong adolescents’ academic achievement: The mediating role of academic engagement. Genet Soc Gen Psychol Monogr. 2005;131:77. Henry KL, Smith EA, Caldwell LL. Deterioration of academic achievement and marijuana use onset among rural adolescents. Health Educ Res. 2007;22:372–384. Ingesson SG. Stability of IQ measures in teenagers and young adults with developmental dyslexia. Dyslexia. 2006;12:81. Ivanovic DM, Leiva BP, Perez HT, Olivares MG, Diaz NS, Urrutia MS, Almagia AF, Toro TD, Miller PT, Bosch EO, Larrain CG. Head size and intelligence, learning, nutritional status and brain development. Head, IQ, learning, nutrition and brain. Neuropsychologica. 2004;42:1118. Kempe C, Gustafson S, Samuelsson S. A longitudinal study of early reading difficulties and subsequent problem behaviors. Scand J Psychol. 2011;52:242–250. Knifsend CA, Graham S. Too much of a good thing? How breadth of extracurricular participation relates to school-related 43 - 31.17c Identity Problem 31.17c Identity Problem affect and academic outcomes during adolescence. J Youth Adolescence. 2012;41:379–389. Lucio R, Hunt E, Bornovalova M. Identifying the necessary and sufficient number of risk factors for predicting academic failure. Dev Psychol. 2012;48:422–428. Pascoe L, Roberts G, Doyle LW, Lee KJ, Thompson DK, et al. Preventing academic difficulties in preterm children: A randomised controlled trial of an adaptive working memory training intervention-IMPRINT study. BMC Pediatr. 2013;13:144–156. Reinke WM, Herman KC, Petras H, Ialongo NS. Empirically derived subtypes of child academic and behavior problems: Cooccurrence and distal outcomes. J Abnorm Psychol. 2008;36:759–770. Roberts G, Quach J, Gold L, Anderson P, Richards F, Mensah F, et al. Can improving working memory prevent academic difficulties? A school-based randomised controlled trial. BMC Pediatr. 2011;11:57–66. Williams BL, Dunlop AL, Kramer M, Dever BV, Hogue C, et al. Perinatal origins of first-grade academic failure: Role of prematurity and maternal factors. Pediatrics. 2013;131:693–700. 31.17c Identity Problem The normative developmental process for an adolescent was conceptualized by the developmentalist Erik Erikson as an adolescent “crisis of identity.” The transition between a childhood identity and the process of accepting a more mature sense of self is the resolution of the “crisis.” Consolidation of identity encompasses cognitive, psychodynamic, psychosexual, neurobiological, and cultural development. As identity is confirmed in adolescence, a sense of self-sameness and continuity over time unfolds. The notion of an identity crisis in adolescence gained widespread attention by clinicians and the popular media during the late 1960s and early 1970s, when many adolescents displayed rejection of mainstream cultural values and ideas and demonstrated alternative lifestyles. The concept of identity disorder as a psychiatric diagnosis was embraced in the 1980s when the DMS-III was devised, as a disorder usually first evident in childhood. It was meant to include adolescents who presented with “severe subjective distress regarding uncertainty about a variety of issues relating to identity” to the point where they became impaired. Identity problem is not currently conceptualized as a psychiatric disorder, rather it refers to uncertainty about issues, such as goals, career choice, friendships, sexual behavior, moral values, and group loyalties. An identity problem can cause severe distress for a young person and can lead a person to seek psychotherapy or guidance; however, it is not included in the DSM-5. It sometimes occurs in the context of such mental disorders as mood disorders, psychotic disorders, and borderline personality disorder. A study examining Intolerance of Uncertainty (IU), that is, the tendency to react negatively to uncertain situations, in 191 adolescents found that IU is correlated with adolescent social anxiety, worry, and to a lesser extent, depression. EPIDEMIOLOGY No reliable information is available regarding overall prevalence; however, factors increasing risk for identity problems include psychiatric disorders, psychosocial difficulties, and the pressures of assimilation as an ethnic minority into mainstream society. ETIOLOGY The causes of identity problems often are multifactorial and include the pressures of a dysfunctional families, the influences of coexisting mental disorders, and the degree to which adolescents feel integrated into their school and family environments. In general, adolescents with social skills deficits, major depressive disorder, psychotic disorders, and other mental disorders report feeling alienated from their peer group and family members, and experience some turmoil. Children who have had difficulty mastering expected developmental tasks all along are likely to have difficulty with the pressure to establish a well-defined identity during adolescence. Erikson used the term identity versus role diffusion to describe the developmental and psychosocial tasks challenging adolescents to incorporate past experiences and present goals into a coherent sense of self. CLINICAL FEATURES The essential features of identity problem seem to revolve around the question, “Who am I?” Conflicts are experienced as irreconcilable aspects of the self that the adolescent cannot integrate into a coherent identity. As Erikson described identity problem, youth manifests severe doubting and an inability to make decisions, a sense of isolation, inner emptiness, a growing inability to relate to others, disturbed sexual functioning, a distorted time perspective, a sense of urgency, and the assumption of a negative identity. The associated features frequently include marked discrepancy between the adolescent’s self-perception and the views that others have of the adolescent; moderate anxiety and depression that are usually related to inner preoccupation, rather than external realities; and self-doubt and uncertainty about the future, with either difficulty making choices or impulsive experiments in an attempt to establish an independent identity. Adolescents with identity problem may join “outcast” cult-like groups. A study examining relationships of social context and identity of high-risk Hispanic adolescents found that school problems and identity confusion among these adolescents were related to behavioral problems and risk-taking behaviors including alcohol use, illicit drug use, and sexual risk-taking behaviors. DIFFERENTIAL DIAGNOSIS Identity problems must be differentiated from sequelae of a mental disorder (e.g., borderline personality disorder, schizophreniform disorder, schizophrenia, or a mood disorder). At times, what initially seems to be an identity problem may be the prodromal manifestations of one of these disorders. Intense, but normal, conflicts associated with maturing, such as adolescent turmoil and midlife crisis, may be confusing, but they usually are not associated with marked deterioration in school, in vocational or social functioning, or with severe subjective distress. Considerable evidence indicates that adolescent turmoil often is not a phase that is outgrown but an indication of true psychopathology. COURSE AND PROGNOSIS The onset of identity problem most frequently occurs in late adolescence, as teenagers separate from the nuclear family and attempt to establish an independent identity and value system. The onset usually is characterized by a gradual increase in anxiety, depression, regressive phenomena (e.g., loss of interest in friends, school, and activities), irritability, sleep difficulties, and changes in eating habits. The course usually is relatively brief, as developmental lags respond to support, acceptance, and the provision of a psychosocial moratorium. Extensive prolongation of adolescence with continued identity problem can lead to the chronic state of role diffusion, which may indicate a disturbance of early developmental stages and the presence of borderline personality disorder, a mood disorder, or schizophrenia. An identity problem usually resolves by the mid-20s. If it persists, the person with the identity problem may have difficulty with career commitments and lasting attachments. Jenna, an 8-year-old girl, was adopted in Taiwan at 10 months of age by a white midwestern couple. As she grew, her vulnerability to separations became increasingly more pronounced. Jenna developed school refusal, and would exhibit outbursts of rage and misbehavior when she was forced to go to school. She pleaded with her mother to care for the many aches and pains that plagued her. By the time she reached adolescence, Jenna had an entrenched habit of cutting and self-mutilating. She responded to frustration, separations, or perceived threats of abandonment by cutting herself or burning herself with cigarette lighters. Eventually, she was able to verbalize the multiple functions that self-injury served for her. She noted that she was able to stay home from school, be in the company of her mother, and avoided the stresses of peer interactions. Jenna and her mother began a course of psychotherapy in which Jenna learned that she would still need to attend school, regardless of her cutting behavior, and her mother learned to provide incentives for Jenna to diminish her maladaptive behaviors. Over time, Jenna became more flexible and realized that she was harming herself, and not others around her. Jenna was able to return to school, and with the help of her therapist, she was able to discontinue her self-injurious behaviors and focus on succeeding in school and with her peers. (Adapted from Efrain Bleiberg, M.D.) TREATMENT Considerable consensus exists among clinicians that adolescents experiencing identity 44 - 31.18 Psychiatric Treatment of Children and A 31.18 Psychiatric Treatment of Children and Adolescents 45 - 31.18a Individual Psychotherapy 31.18a Individual Psychotherapy problems may respond to brief psychosocial intervention. Individual psychotherapy directed toward encouraging growth and development usually is considered the therapy of choice. Adolescents with identity problems often feel developmentally unprepared to deal with the increasing demands for social, emotional, and sexual independence. Issues of separation and individuation from their families can be challenging and overwhelming. Enlisting the concepts outlined by Erikson with regard to adolescent development, psychotherapy may include discussion of adolescent exploration (active search among alternatives for activities and friendships that fit) and commitment (demonstrated investment) in activities that promote independence and autonomy. Treatment is aimed at helping these adolescents develop a sense of competence and mastery about necessary social and vocational choices. A therapist’s empathic acknowledgment of an adolescent’s struggle can be helpful in the process. REFERENCES Bleiberg E. Identity problem and borderline disorders in children and adolescents In: Sadock BJ, Sadock VA, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 8th ed. Vol. 2. Philadelphia: Lippincott Williams & Wilkins; 2005:3457. Boelen PA, Vrinssen I, van Tulder F. Intolerance of uncertainty in adolescents. J Nerv Ment Dis. 2010;198:194–200. Chabrol H, Leichsenring F. Borderline personality organization and psychopathic traits in nonclinical adolescents: Relationship of identity diffusion, primitive defense mechanism and reality testing with callousness and impulsivity traits. Bull Menninger Clin. 2006;70:160. Erikson EH. Identity and the life cycle: Selected papers. Psychol Issues. 1959;1:1. Ivanovic DM, Leiva BP, Perez HT, Olivares MG, Diaz NS, Urrutia MS, Almagia AF, Toro TD, Miller PT, Bosch EO, Larrain CG. Head size and intelligence, learning, nutritional status and brain development. Head, IQ, learning, nutrition and brain. Neuropsychologica. 2004;42:1118. Mackinnon SP, Nosko A, Pratt MW, Norris JE. Intimacy in young adults’ narratives of romance and friendship predicts Eriksonian Generativity: A mixed method analysis. J Personality. 2011;79:3. Marcia J, Jossleson R. Eriksonian personality research and its implications for psychotherapy. J Personality. 2012;81:617– 629. Rossi NE, Mebert CJ. Does a quarterlife crisis exist? J Genet Psychol. 2011;172:141–161. Schwartz SJ, Mason CA, Pantin H, Wang W, Brown CH, et al. Relationships of social context and identity to problem behavior among high-risk Hispanic adolescents. Youth Sci. 2009;40:541–570. Thomas JJ. Adolescents’ conceptions of the influence of romantic relationships on friendships. J Genet Psychol. 2012;173:198–207. 31.18 Psychiatric Treatment of Children and Adolescents 31.18a Individual Psychotherapy Individual psychotherapy with children and adolescents generally begins by establishing rapport through developmentally appropriate psychoeducation regarding the target symptoms and disorders to be addressed. As a rule, the younger the child, the more extensively family members participate in the treatment. Even among adolescents, family members are often directly involved in some components of the treatment in order to achieve the maximum benefit. In recent years, randomized clinical trials have provided a body of literature to support the efficacy of cognitive-behavioral psychotherapy for a wide range of childhood psychiatric disorders including obsessivecompulsive disorder (OCD), anxiety disorders, and depressive disorders. Additional therapeutic approaches including supportive, psychodynamic, and more recently, mindfulness-based stress reduction (MBSR), mindful meditation, and yoga are sometimes incorporated into psychosocial treatments, creating an “eclectic” mixture. The initial goal of any psychotherapeutic strategy is to establish a working relationship with the child or adolescent. In general, successful individual psychotherapeutic interventions with youth also necessitate establishing a therapeutic rapport with parents. To establish a therapeutic relationship with a child of any age requires a knowledge of normal development as well as an understanding of the context in which the symptoms emerged. Individual psychotherapy with children focuses on improving adaptive skills as well as diminishing specific symptomatology. Most children do not seek psychiatric treatment; typically, they are brought to a psychotherapist due to symptoms noted by a family member, schoolteacher, or, pediatrician. Children often believe that they are being taken for treatment because of their misbehavior or as a punishment for wrongdoing. Children and adolescents are the most accurate informants of their own thoughts, feelings, moods, and perceptual experiences; however, external behavior problems are often more accurately identified by parents or teachers. Psychotherapists for children frequently function as their advocates in interactions with schools, after-school programs, and community organizations. Individual psychotherapy with a child often takes place in conjunction with family therapy, group therapy, educational remediation, and psychopharmacological interventions. PSYCHOTHERAPEUTIC TECHNIQUES AND UNDERLYING THEORIES Cognitive-Behavioral Therapy Cognitive-behavioral therapy (CBT) is an amalgam of behavioral therapy and cognitive psychology. It emphasizes how children may use thinking processes and cognitive modalities to reframe, restructure, and solve problems. A child’s distortions are addressed by generating alternative ways of dealing with problematic situations. Cognitive-behavioral strategies have been shown in multiple studies to be effective in the treatment of child and adolescent mood disorders, OCD, and anxiety disorders. A recent study compared a family-focused CBT, the “Building Confidence Program,” with traditional child-focused CBT, with minimal family involvement for children with anxiety disorders. Both interventions included coping skills training and in vivo exposure, but the family CBT intervention also included parent communication training. Compared with the child-focused CBT, family CBT was associated with greater improvement on independent evaluators’ ratings and parent reports of child anxiety, but not on children’s self-reports of improvement. Family-focused CBT has also been used in the treatment of pediatric bipolar disorder with promising results. One of the limiting factors in providing CBT to children with OCD, anxiety disorders, and depressive disorders is the lack of sufficient numbers of trained child and adolescent cognitive-behavioral therapists. A recent study addressed the feasibility of combining a CBT via clinic-plus-Internet treatment. Children who received the clinic-plus-Internet treatment showed significantly greater reductions in anxiety from pretreatment to posttreatment, and maintained gains for a period of 12 months compared with children who received no active treatment, but were on a wait-list. The Internet treatment was acceptable to families and dropout rate was minimal. Psychoanalysis and Psychoanalytic Therapy Child Psychoanalysis. Child psychoanalysis, an intensive, uncommon form of psychoanalytic psychotherapy, involves three to four sessions a week and places an emphasis on unconscious resistance and defenses. In this approach, therapists anticipate unconscious resistance and allow transference manifestations to mature to a full transference neurosis, through which neurotic conflicts are ultimately resolved. Interpretations of dynamically relevant conflicts are emphasized in psychoanalytic descriptions, and elements that are predominant in other types of psychotherapies are included. In all psychotherapy, children should derive support from a consistently understanding and accepting relationship with their therapists. Remedial educational guidance is provided when necessary. In classic psychoanalytic theory, exploratory psychotherapy is applicable to patients of all ages and involves reversing the evolution of psychopathological processes. A principal difference noted with advancing age is a sharpening distinction between psychogenetic and psychodynamic factors. The younger the child, the more the genetic and dynamic forces are intertwined. The development of pathological processes generally is believed to begin with experiences that have proved to be particularly significant to children and to have affected them adversely. Although in one sense the experiences were real, in another sense, they may have been misinterpreted or imagined. In any event, to children, these were traumatic experiences that caused unconscious complexes. Being inaccessible to conscious awareness, the unconscious elements readily escape rational adaptive maneuvers and are subject to pathological misuse of adaptive and defensive mechanisms. The result is the development of conflicts leading to distressing symptoms, character attitudes, or patterns of behavior that constitute the emotional disturbance. Psychoanalytic Psychotherapy. Psychoanalytic psychotherapy, a modified form of psychoanalysis, is expressive and exploratory and endeavors to reverse the evolution of emotional disturbance through reenacting and desensitizing traumatic events. This is achieved by having children freely express thoughts and feelings in an interview-play situation. Ultimately, therapists help patients understand feelings that they may have avoided, as well as fears and wishes that have been self-defeating. Behavioral Therapy All behavior, whether adaptive or maladaptive, is a consequence of the same basic principles of behavior acquisition and maintenance. Behavior is either learned or unlearned. What renders behavior abnormal or disturbed is its social significance. Although theories and their derivative therapeutic intervention techniques have become increasingly complex over the years, all learning can be subsumed in two global basic mechanisms. One is classic respondent conditioning, akin to Ivan Pavlov’s famous experiments, and the second is operant instrumental learning, which is associated with B. F. Skinner; the latter is also basic to both Edward Thorndike’s law of effect, which is about the influence of reinforcing consequences of behavior, and to Sigmund Freud’s pain-pleasure principle. Behavior therapy assigns the highest priority to the immediate precipitants of behavior and deemphasizes remote underlying causal determinants that are important in the psychoanalytic tradition. Respondent conditioning theory asserts that only two types of abnormal behavior exist: behavioral deficits that result from a failure to learn, and deviant maladaptive behavior that is a consequence of learning inappropriate things. Such concepts have always been an implicit part of the rationale underlying all child psychotherapy. Intervention strategies derive much of their success, particularly with children, from rewarding previously unnoticed good behavior, thereby highlighting it, and making it occur more frequently than in the past. Family Therapy Family therapies have been influenced by conceptual contributions from systems theory, communications theory, object relations theory, social role theory, ethology, and ecology. The core premise entails the idea of a family as a self-regulating, open system that possesses its own unique history and structure. This structure is constantly evolving as a consequence of dynamic interaction between the family’s mutually interdependent systems and persons who share a complementarity of needs. From this conceptual foundation, a wealth of ideas has emerged under rubrics such as family development, life cycle, homeostasis, functions, identity, values, goals, congruence, symmetry, myths, and rules; roles, such as spokesperson, symptoms-bearer, scapegoat, affect barometer, pet, persecutor, victim, arbitrator, distractor, saboteur, rescuer, breadwinner, disciplinarian, and nurturer; structure, such as boundaries, splits, pairings, alliances, coalitions, enmeshed, and disengaged; and double bind, scapegoating, and mystification. Increasingly, appreciation of the family system sometimes explains why a minute therapeutic input at a critical junction may result in far-reaching changes. Justin was a 14-year-old boy from a middle-class family enrolled in the 9th grade at a public school. He was brought in by his parents for treatment of a long-standing history of shyness and anxiety in social situations, which was more evident now that most of his peers were getting together after school and he was spending his weekends alone. Evaluation revealed social anxiety disorder as the primary disorder. Justin was initially resistant to treatment despite his wish to feel more comfortable with other people and in social situations with peers. After much discussion and some pressure from his parents, Justin began to attend a cognitive-behavioral group treatment for adolescents with social anxiety. Justin became mildly agitated each time he was scheduled for a session; however, once he arrived, he was able to participate. He began, a 16-session course of treatment combining education, cognitive restructuring, behavioral exposure, relapse prevention, and four sessions of parent involvement. As treatment progressed, Justin increased his visibility at school, and even attended a school football game with a few peers. Justin told his therapist that he wanted to go to the next school dance but was afraid that he would be embarrassed and would have to go home before the dance was over. The therapists designed several exposures whereby the various things that could happen at a dance were presented to Justin, including being offered alcohol or drugs, having a good time dancing, being left alone or ignored by his friends, or being turned down if he asked a girl to dance with him. As it turned out, Justin’s few school acquaintances ignored him and left him at the dance. Justin, prepared for this less-than-desired outcome in his group experience, asked two girls to dance, and forced himself to interact with other peers. To his surprise, despite his shyness, one girl agreed to dance with him. He considered the evening a success. Justin subsequently went to another social event with a new group of peers who seemed more accepting of him. In Justin’s case, the importance of practicing responses to potential rejections in the safety of his treatment group was crucial to his success at the dance, and it increased his motivation to continue treatment. Through his treatment, Justin became more and more appropriately prepared, through behavioral exposure and practice, to handle what might previously have been awkward and discouraging situations. (Adapted from a case contributed by Anne Marie Albano, Ph.D.) Tim was a 3-year-old child, developing normally and quite verbal, until he started preschool, at which time he suddenly refused to speak at all outside his home. Tim had begun preschool shortly after his parents had separated and his father had left the home. Prior to his parents’ separation, Tim was highly verbal and developmentally ahead of many children his age in language skill. Although he was observed constantly in preschool, he was never “caught” speaking to peers. He was described as a compliant child who didn’t smile as easily as the other children, who played with others and followed requests without problem but would not speak. During his psychiatric evaluation, it was revealed that Tim enjoyed eating Froot Loops in a favorite cup as a treat. Treatment was designed to provide incentive for speaking through the delivery of a reinforcement of high value, the Froot Loops. Hence, Froot Loops became available only in the preschool and the therapist’s office and, temporarily, were not available in his home. The therapist enacted a process of graduated shaping of communication behaviors—first nonverbal and then vocal noises—and trained the preschool teacher to do the same. Froot Loop boxes were kept in full view of Tim at all times during the initial phase of treatment and, when he was “caught” gazing at the box, the therapist or teacher would prompt Tim for acknowledgment that he wanted the treat. Pointing, looking, and nodding in their direction resulted in receiving four Froot Loops. Next, Tim was asked to make a sound or ask for the Froot Loop to receive the reward. This step was accomplished as he grunted and eventually said, “Loop.” Finally, prompts to ask for the Froot Loops in a sentence were enacted, and Tim complied with this demand. This phase of treatment took 2 days at the preschool and 2 hours of therapy to accomplish. Eventually, the boxes of Froot Loops were removed from the environments, but the teacher kept the cereal with her to deliver four Loops whenever Tim made sounds or spoke in school. This shaping procedure took an additional 3 days to result in Tim speaking to the teacher and peers, albeit in short sentences. The treat was faded—that is, delivered on a variable ratio schedule of every three to eight times that he spoke, to promote further speaking and decrease the association with the treat. By the end of the second week of training, Tim was speaking at the level he had achieved prior to his parents’ separation. Tim’s parents were cautioned to allow Tim to speak for himself in social situations (e.g., order his own food at a restaurant, say hello to others, make his own requests before providing a treat) as a way of relapse prevention. (Adapted from a case contributed by Anne Marie Albano, Ph.D.) Jenna was a 13-year-old teen with a family history of anxiety and depression. Her parents brought her to treatment because of recurrent obsessions involving contamination and germs, with corresponding compulsions during which she had convinced her parents to check her food, while she washed her hands repeatedly until they became raw and bleeding. Evaluation revealed a fear that, unless her parents checked her food for bugs or germs, the meal was likely contaminated. Jenna’s parents, attempting to ease her fear, would physically pull apart her food and examine it to her satisfaction, often spending upward of 1 hour before each meal. However, this process caused much distress and discord between Jenna and her family. Jenna’s hand washing had generalized to almost every daily activity—after opening a door, reading a book, using a pencil, or touching any object that she deemed dirty. Jenna’s evaluation led to a recommendation of behavioral therapy utilizing exposure and response prevention. This consisted of formulating a hierarchy of her obsessions and compulsions, from the least upsetting (checking food prepared by her mother) to the most upsetting (touching something that was wet or slimy and then touching her mouth). Systematically, the therapist engaged Jenna first in a series of imaginal exposures to a scene (e.g., you take a bite of hamburger and something tastes gritty to you and you realize that your mom did not check the burger) until her anxiety dropped to an acceptable level. The drop in anxiety typically took approximately 25 minutes. Next, the scene was enacted in vivo, whereby foods were introduced with “contaminants” in them (e.g., putting pieces of uncooked rice into the burger to mimic “grit”), and Jenna ate the food without having her parents check. As treatment progressed, Jenna learned that her chronic fear of becoming sick was not likely to occur. Similarly, washing rituals were addressed by having her touch items with various substances coating them and then touching her face and mouth. Jenna’s treatment entailed a 14-session program during which her parents were taught to assist her with these exposures in the home. Her parents were also instructed to refrain from engaging in her rituals. Relapse prevention plans were added to expand her range of food choices and situational contexts (cafeterias, food stands, restaurants) for exposure. By the end of treatment, Jenna was eating without the need for checking and with minimal anxiety. Moreover, she was engaging in a wide range of activities without the need to wash after touching each object. (Adapted from a case contributed by Anne Marie Albano, Ph.D.) Supportive Psychotherapy Supportive psychotherapy is particularly helpful in enabling a well-adjusted youngster to cope with emotional turmoil engendered by a crisis. It also is used to treat disturbances related to traumatic experiences, losses, mild mood disorders, and mild forms of anxiety. A 6-year-old boy was brought for treatment because of long-standing severe aggression and destruction of property. In addition to an evaluation for medication, the child was seen in twice-weekly psychoanalytically oriented psychotherapy. The beginning sessions were marked by the repeated need to set limits and contain the child’s aggressive behaviors. Two months into treatment, he began to pump himself up, roar, and announce that he was “the Incredible Hulk.” He would then proceed to stomp around the play therapy room, attempting to destroy the toys. The therapist then suggested, “You know you can’t really be the Hulk. You can pretend that you are the Hulk, and then maybe we can play this together.” After a number of similar exchanges, the child gradually allowed the therapist to join in the game with him. Over the next 6 months, the boy was able to modulate his behavior in that he was able to “play the part” of the Hulk, but without destroying property, and limiting himself to actions that were less aggressive. He was able to understand that he could pretend to be the Hulk without literally trying to be the Hulk. (Adapted from a case contributed by David L. Kaye, M.D.) Combined Psychodynamic and Behavioral Therapy Probably the most vivid examples of the integration of psychodynamic and behavioral approaches are demonstrated in the milieu of child and adolescent inpatient, residential, and partial hospital or intensive outpatient treatment programs. Behavioral change is initiated in these settings, and its repercussions are explored concurrently in individual psychotherapeutic sessions, so that the action in one arena and the information stemming from it augment and illuminate what transpires in the other arena. Alternative and Complementary Psychosocial interventions: Mindfulness-Based Stress Reduction (MBSR), Mindfulness Meditation, and Yoga Mindfulness-Based Stress Reduction (MBSR), a psychoeducational training program leading to applying the practice of mindfulness into everyday life was studied in adolescent psychiatric outpatients. Mindfulness practices focus on paying sustained attention to moment-to-moment stimuli without engaging in cognitive judgments or selfcriticism, and promoting an attitude of acceptance. In adults, this practice has been shown to facilitate improved coping and decrease symptoms of anxiety, stress, and in some cases, self-harming behaviors. The current study was a trial of approximately 100 adolescents aged 14 to 18, with heterogeneous diagnoses, who were randomized to a waitlist control group receiving treatment as usual (TAU), which consisted of individual or group therapy, or to manualized sessions of MBSR for 2 hours per week for 8 weeks. The MBSR group was led by trained instructors who facilitated the use of mindfulness practices by the participants during formal sessions and encouraged practice at home as well. The participants were tested diagnostically at the end of the 8-week study period and again at 3 months following the end of the study. The results found that both the MBSR and the TAU groups reported significantly reduced anxiety, depressive, and somatization symptoms, and improved self-esteem; but only the MBSR group reported significant declines in perceived stress, obsessive symptoms, and interpersonal problems. Furthermore, although more than 45 percent of the MBSR group showed changes in diagnoses at the end of the study (such as no longer meeting criteria for a mood disorder) none of the TAU group was found to have remitted from a diagnosis. Mindfulness meditation practices have been applied in various forms to a multitude of psychiatric conditions including mood disorders, chronic pain syndromes, anxiety disorder, and ADHD. Mindfulness, according to Kabat-Zinn, is characterized by paying complete attention to the present moment without judgment, with an ability to be aware of inner and outer experiences in the present. There are many forms of meditation which incorporate mindfulness, and both MBSR, and Mindfulness-Based Cognitive Therapy (MBCT) developed by Teasdale, can be considered forms of mindfulness meditation. There is evidence based on neuroimaging studies that mindfulness meditation can induce specific brain states. One study indicated that Vipassana meditation is associated with activation of the rostral anterior cingulate cortex as well as the dorsal medial prefrontal cortex. There is evidence to suggest that mindfulness meditations can improve attention, and that these changes may lead to clinically important improvements. Yoga originated in ancient India, and while there are many varieties, key components include physical postures, controlled breathing, deep relaxation, and meditation. Randomized controlled trials using yoga have provided evidence of its benefit as an adjunctive intervention in mild depression, sleep disturbance, and attention problems. Clinical trials comparing yoga to cooperative game playing or physical exercises in children with ADHD found moderate improvements in ADHD symptoms when yoga was added as an adjunct to medication. There is some evidence suggesting that yoga may be beneficial as an adjunctive intervention for mild depression, even in the absence of medication and potentially for schizophrenia, as an adjunct to medication. THE ROLE OF PLAY Observing play and engaging in play with children can be extremely informative in assessing developmental abilities, and in understanding sensitive situations. This is particularly relevant for young children, and for children who have experienced trauma, which is difficult to describe in words. Although the choices of play material vary among therapists, the following equipment can constitute a well-balanced playroom or play area: multi-generational families of dolls of various races; dolls representing special roles and feelings, such as police officer, doctor, and soldier; dollhouse furnishings with or without a dollhouse; toy animals; puppets; paper, crayons, paint, and blunt-ended scissors; a sponge-like ball; clay or something comparable; tools such as rubber hammers, rubber knives, and guns; building blocks, cars, trucks, and airplanes; and eating utensils. The toys should enable children to communicate through play. Therapists should avoid fragile objects that can break easily, that can result in physical injury to a child, or that can increase a child’s guilt. Psychotherapy with children and adolescents generally is more directed and active than it is with adults. Children usually cannot synthesize histories of their own lives, but they are excellent reporters of their current internal states. Even with adolescents, a therapist often takes an active role, is somewhat less open-ended than with adults, and offers more direction and advocacy than with adults. Nurturing and maintaining a therapeutic alliance may require educating children about the process of therapy. Another educational intervention may entail assigning labels to affects that have not been part of a youngster’s experience. The temptation for therapists to offer themselves as a quasi-parent role model for children may stem from helpful educational attitudes toward children. Although this may sometimes be an appropriate therapeutic strategy, therapists should not lose sight of the potential pitfalls of engaging in a highly parental role with their child and adolescent patients. PARENTS AND FAMILY MEMBERS Parents and family members are involved in child psychotherapy to varying degrees. For preschool-age children, the entire therapeutic effort may be directed toward the parents, without any direct treatment of the child. At the other extreme, children can be treated in psychotherapy without any parental involvement beyond the payment of fees and transporting the child to the therapy sessions. Most practitioners, however, prefer to maintain an alliance with parents to obtain additional information about the child. Probably the most frequent parental arrangements are those developed in child guidance clinics—that is, parent guidance focused on the child or the parent–child interaction and therapy for the parents’ own individual needs concurrent with the child’s therapy. Parents may be seen by their child’s therapist or by someone else. Recently, increasing efforts have been made to shift the focus from the child as the primary patient to the child as the family’s emissary to the clinic. In such family therapy, all or selected members of the family are treated simultaneously as a family group. Although the preferences of specific clinics and practitioners for either an individual or a family therapeutic approach may be unavoidable, the final decision regarding which therapeutic strategy or combination to use should be derived from the clinical assessment. CONFIDENTIALITY The issue of confidentiality takes on greater meaning as children grow older. Very young children are unlikely to be as concerned about this issue as are adolescents. Confidentiality usually is preserved unless a child is believed to be in danger or to be a danger to someone else. In other situations, a child’s permission usually is sought before a specific issue is raised with parents. Advantages exist to creating an atmosphere in which children can feel that all words and actions are viewed by therapists as simultaneously both serious and tentative. In other words, children’s communications do not bind therapists to a commitment; nevertheless, they are too important to be communicated to a third party without a patient’s permission. Although such an attitude may be implied, sometimes therapists should explicitly discuss confidentiality with children. Most of what children do and say in psychotherapy is common knowledge to the parents. The therapist should try to enlist parents’ cooperation in respecting the privacy of children’s therapeutic sessions. The respect is not always readily honored, because parents are naturally curious about what transpires, and they may be threatened by a therapist’s apparently privileged position. Routinely reporting to a child the essence of communications with third parties about the child underscores the therapist’s reliability and respect for the child’s autonomy. In certain treatments, the report can be combined with soliciting the child’s guesses about these transactions. A therapist also may find it fruitful to invite children, particularly older children, to participate in discussions about them with third parties. INDICATIONS Psychotherapy usually is indicated for children with psychiatric symptoms or disorders that interfere with their ability to function at home and in school, and causes significant distress. A developmental perspective always informs psychosocial interventions with a given child, so that it matches that child’s cognitive function and emotional maturity. If a psychotherapy situation is not effective, it is important to determine whether the therapist and patient are poorly matched, whether the type of psychotherapy is inappropriate to the nature of the problems, and whether the child is cognitively 46 - 31.18b Group Psychotherapy 31.18b Group Psychotherapy inappropriate for the treatment. REFERENCES Albano AM. Cognitive-behavioral psychotherapy for children and adolescents. In: Sadock BJ, Sadock VA, Ruiz P, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Philadelphia: Lippincott Williams & Wilkins; 2009:3721. Balasubramaniam M, Telles S, Doraiswamy PM. Yoga on our minds: A systematic review of yoga for neuropsychiatric disorders. Front Psychiatry. 2012;3:117. Biegel GM, Brown KW, Shapiro SL, Schubert CM. Mindfulness-based stress reduction for the treatment of adolescent outpatients: A randomized clinical trial. J Consult Clin Psychol. 2009;77:855–866. Chiesa A, Serretti A. A systematic review of neurobiological and clinical features of mindfulness meditations. Psychol Med.2010;40:1239–1252. Kaye DL. Individual psychodynamic psychotherapy. In: Sadock BJ, Sadock VA, Ruiz P, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Vol. II Philadelphia: Lippincott Williams & Wilkins; 2009:3707. Kober D, Martin A. Inpatient psychiatric, partial hospital, and residential treatment for children and adolescents. In: Sadock BJ, Sadock VA, Ruiz P, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Vol. II, Philadelphia: Lippincott Williams & Wilkins; 2009:3766. Kratochvil CJ, Wilens TE. Pediatric psychopharmacology. In: Sadock BJ, Sadock VA, Ruiz P, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Vol. II Philadelphia: Lippincott Williams & Wilkins; 2009:3756. Pumariega A. Community-based treatment. In: Sadock BJ, Sadock VA, Ruiz P, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Vol. II, Philadelphia: Lippincott Williams & Wilkins; 2009:3772. Rostain AL, Franklin ME. Brief psychotherapies for childhood and adolescence In: Sadock BJ, Sadock VA, Ruiz P, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Vol. II. Philadelphia: Lippincott Williams & Wilkins; 2009:3715. Rubia K. The neurobiology of meditation and its clinical effectiveness in psychiatric disorders. Biol Psychiatry. 2009;82:1– 11. Sargent J. Family therapy. In: Sadock BJ, Sadock VA, Ruiz P, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Vol. II. Philadelphia: Lippincott Williams & Wilkins; 2009:3741. Schlozman SC, Beresin EV. The treatment of adolescents. In: Sadock BJ, Sadock VA, Ruiz P, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Vol. II. Philadelphia: Lippincott Williams & Wilkins; 2009:3777. Siqueland L, Rynn M, Diamond GS. Cognitive behavioral and attachment based family therapy for anxious adolescents: Phase I and II studies. J Anxiety Disord. 2005;19:361. Spence SH, Holmes JM, March S, Lipp OV. The feasibility and outcome of clinic plus internet delivery of cognitivebehavior therapy for childhood anxiety. J Consult Clin Psychol. 2006;74:614. Zylowska L, Ackerman DL, Yang MH, Futrell JL, Horton NL, Hale TS, Pataki C, Smalley SL. Mindfulness meditation training in adults and adolescents with ADHD: A feasibility study. J Attention Dis. 2008;11:737–746. 31.18b Group Psychotherapy Therapeutic groups for children and adolescents are varied in terms of problems addressed, age of patients, group structure, and therapeutic approach implemented. Group formats have been used to treat a broad range of clinical symptoms, including anger-management for aggressive children and adolescents, social skills improvement, support groups for survivors of childhood sexual abuse, and other traumatic events such as the September 11th World Trade Center tragedy. In addition, groups have also been settings for the treatment of adolescents with social anxiety and OCD, and youth with depressive disorders. Groups have successfully used cognitive-behavioral techniques to treat childhood anxiety disorders, adolescents with substance abuse, and youth with specific learning disorders. Support groups for youth exposed to loss have provided evidence of efficacy, including data from a study investigating the benefits of a psychotherapy group for adolescent survivors of homicide victims. Group therapies can be utilized with children of all ages using developmentally appropriate formats. The groups can focus on behavioral, educational, and social skills and psychodynamic issues. The mode in which the group functions depends on children’s developmental levels, intelligence, and problems to be addressed. In behaviorally oriented and cognitivebehavioral groups, the group leader is a directive, active participant who facilitates prosocial interactions and desired behaviors. In groups using psychodynamic approaches, the leader may monitor interpersonal interactions less actively than in behavior therapy groups. Gathering children and adolescents into groups may lead to greater psychological impact than treating them individually. A number of factors, described by Irving Yalom, may contribute to the effectiveness of groups. These factors include the following theoretical components: Hope: Hope may be generated by gathering with others who are experiencing similar difficulties and by observing others actively mastering the problems. Universality: Children and adolescents with psychiatric disorders often feel isolated and alienated from peers. Working together in groups may diffuse the isolation and help children and adolescents view their disorder as only a small part of their overall identity. Imparting Information: Children and adolescents are familiar with a format of gaining new information in a group setting, such as in school. The group therapy format provides an opportunity to reinforce learning when the child or adolescent “helps” or demonstrates what he or she has learned to peers. Altruism: Helping other peers in a group setting by supporting them and identifying with their struggles can improve a child or adolescent’s self-esteem and help them gain a sense of mastery over their own issues. Improved Social Skills: Group therapy is a safe format in which children and adolescents with poor social skills can improve their interpersonal and communication abilities under the supervision of a leader and with peers who also benefit from the practice scenarios. Groups can be highly effective modalities to provide peer feedback and support to children who are either socially isolated or unaware of their effects on their peers. Groups with very young children generally are highly structured by the leader and use imagination and play to foster socially acceptable peer relationships and positive behavior. Therapists must be keenly aware of the level of children’s attention span and the need for consistency and limit setting. Leaders of preschool-age groups can model supportive adult behavior in meaningful ways for children who have been deprived or neglected. School-age children’s groups can be single sex or include both boys and girls. School-age children are more sophisticated in verbalizing their feelings than preschoolers, but they also benefit from structured therapeutic games. Children of school age need frequent reminders about rules, and they are quick to point out infractions of the rules to each other. Interpersonal skills can be addressed nicely in group settings with school-age children. Same-sex groups are often used among adolescents. Physiological changes in early adolescence and the new demands of high school lead to stress that may be ameliorated when groups of same-age peers compare and share. In older adolescence, groups more often include both boys and girls. Even with older adolescents, the leader often uses structure and direct intervention to maximize the therapeutic value of the group. Adolescents who are feeling dejected or alienated may find a special sense of belonging in a therapy group. Keith was a high-functioning, 14-year-old boy diagnosed with autism spectrum disorder. Keith was an awkward-looking adolescent who seemed younger than his chronological age. His academic level was above average, but his social development was odd. His pedantic speaking style contributed considerably to his social isolation, particularly after starting 7th grade. He was referred to a group of adolescents with social skills problems in order to improve his ability to make friends and have more successful social interactions. Initially, Keith limited his participation to monosyllabic answers to direct questions, and then he would go back to reading a book on the history of Napoleon, his favorite subject and object of fascination. Group members chose to ignore him after a while. Over a period of several weeks, his interest in the book seemed to abate. Keith brought it, but it remained unopened on his lap. He would make an occasional remark, which was often not related to the topic of conversation. The other adolescents in the group seemed to respect his “differentness”; however, it was still difficult to have successful social interactions. Two months later a very shy 13-year-old boy joined the group. After a few sessions Keith developed an unexpected interest in the newer member and sat near him and encouraged him to interact with the group. Soon Keith was not bringing a book any longer and was more involved with group members. In response to the group leader’s guidance and practice exercises in the group, Keith learned to respond to social cues in a more appropriate manner, and although he continued having morbid preoccupations with power and a fascination with Napoleon, he was able to converse with group members about more pertinent social topics. Keith’s increasing social skills and greater interest in people was clinically evident. Social skills practice within the group became a most significant tool to help Keith with his interpersonal interactions in school and with his family. (Adapted from a case contributed by Alberto C. Serrano, M.D.) PRESCHOOL-AGE AND EARLY SCHOOL-AGE GROUPS Work with a preschool-age group usually is structured by a therapist through the use of a particular technique, such as puppets or artwork. In therapy with puppets, children project their fantasies onto the puppets in the same way as in ordinary play. Here, the group aids the child less by interaction with other members than by action with the puppets. In play group therapy, the emphasis rests on children’s interactional qualities with each other and with the therapist in the permissive playroom setting. A therapist should be a person who can allow children to produce fantasies verbally and in play but who can also use active restraint when children undergo excessive tension. The toys are the traditional ones used in individual play therapy. The children use the toys to act out aggressive impulses and to relive their home difficulties with group members and with the therapist. The children selected for group treatment have a common social hunger and need to be like their peers and be accepted by them. Selected children usually include those with phobias, effeminate boys, shy and withdrawn children, and children with disruptive behavior disorders. Modifications of these criteria have been used in group psychotherapy for autistic children, parent group therapy, and art therapy. A modification of group psychotherapy has been used for toddlers with physical disabilities who show speech and language delays. The experience of twice-weekly group activities involves mothers and children in a mutual teaching–learning setting. This experience has proved effective for mothers who received supportive psychotherapy in the group experience; their formerly hidden fantasies about their children emerged and were dealt with therapeutically. SCHOOL-AGE GROUPS Activity group psychotherapy is based on the idea that corrective experiences in a therapeutically conditioned environment may increase appropriate social interactions between children and with adults. The format uses interview techniques, verbal explanations of fantasies, group play, work, and other communications. In this type of group psychotherapy, children verbalize in a problem-oriented manner, with the awareness that problems brought them together and that the group aims to change them. They report dreams, fantasies, daydreams, and unpleasant experiences. Therapists vary in their use of time, co-therapists, food, and materials. Most groups meet after school for at least 1 hour, although other group leaders prefer a 90-minute session. Some therapists serve food during the last 10 minutes; others prefer serving times when the children are together for talking. Food, however, does not become a major feature and is never central to the group’s activities. PUBERTAL AND ADOLESCENT GROUPS Group therapy methods similar to those used in younger-age groups can be modified to apply to pubertal children, who are often grouped monosexually. Their problems resemble those of late latency-age children, but they (especially the girls) are also beginning to feel the effects and pressures of early adolescence. Groups offer help during a transitional period; they seem to satisfy the social appetite of preadolescents, who compensate for feelings of inferiority and self-doubt by forming groups. This therapy takes advantage of the influence of the socialization process during these years. Because pubertal children experience difficulties in conceptualizing, pubertal therapy groups tend to use play, drawing, psychodrama, and other nonverbal modes of expression. The therapist’s role is active and directive. Activity group psychotherapy has been the recommended group therapy for pubertal children who do not have significantly disturbed personality patterns. The children, usually of the same sex and in groups of not more than eight, freely engage in activities in a setting especially designed and planned for its physical and environmental characteristics. Samuel Slavson, a pioneer in group psychotherapy, pictured the group as a substitute family in which the passive, neutral therapist becomes the surrogate for parents. The therapist assumes various roles, mostly in a nonverbal manner, as each child interacts with the therapist and other group members. Currently, however, therapists tend to see the group as a form of peer group, with its attendant socializing processes, rather than a reenactment of the family. Late adolescents, 16 years of age and older, often may be included in groups of adults. Group therapy has been useful in the treatment of substance-related disorders. Combined therapy (the use of group and individual therapy) also has been used successfully with adolescents. OTHER GROUP SITUATIONS Groups are also helpful in more focused treatments, such as specific social skills training for children with ADHD, cognitive-behavioral group interventions for depressed children and for children with bereavement problems or eating disorders. In these more specialized groups, the issues are more specific, and actual tasks (as in social skills groups) can be practiced within the group. Some residential and day treatment units use group psychotherapy techniques. Group psychotherapy in schools for underachievers and children from low socioeconomic levels has relied on reinforcement and on modeling theory, in addition to traditional techniques, and has been supplemented by parent groups. In controlled conditions, residential treatment units have been used for specific studies in group psychotherapy, such as behavioral contracting. Behavioral contracting with reward–punishment reinforcement provides positive reinforcements among preadolescent boys with severe concerns in basic trust, low self-esteem, and dependence conflicts. Somewhat akin to formal residential treatment units are social group work homes. For children who undergo many psychological assaults before placement, supportive group psychotherapy offers ventilation and catharsis, but more often it succeeds in letting children become aware of the enjoyment of sharing activities and developing skills. Public schools—also a structured environment, although not usually considered the best site for group psychotherapy—have been used by several workers. Group psychotherapy as group counseling readily lends itself to school settings. One such group used gender- and problem-homogeneous selection for groups of six to eight students, who met once a week during school hours over 2 to 3 years. INDICATIONS Many indications exist for the use of group psychotherapy as a treatment modality. Some indications are situational; a therapist may work in a reformatory setting, in which group psychotherapy seems to reach adolescents better than individual treatment does. Another indication is time economics; more patients can be reached in a given time by the use of groups than by individual therapy. Group therapy best helps a child at a given age and developmental stage and with a given type of problem. In young age groups, children’s social hunger and their potential need for peer acceptance help determine their suitability for group therapy. Criteria for unsuitability are controversial and have been loosened progressively. PARENT GROUPS In group psychotherapy, as in most treatment procedures for children, parental difficulties can present obstacles. Sometimes, uncooperative parents refuse to bring a child or to participate in their own therapy. The extreme of this situation reveals itself when severely disturbed parents use a child as their channel of communication to work out their own needs. In such circumstances, a child is in the unfortunate position of receiving positive group experiences that seem to create havoc at home. Parent groups, therefore, can be a valuable aid to group psychotherapy for their children. A recent study of a cognitive-behavioral group intervention for parents to learn how to utilize therapeutic interventions with their anxiety disordered children suggested that parent groups to teach these skills can be successfully utilized with their children. Parents of children in therapy often have difficulty understanding their children’s ailments, discerning the line of demarcation between normal and pathological behavior, relating to the medical establishment, and coping with feelings of guilt. Parent groups assist in these areas and help members formulate guidelines for action. REFERENCES Baer S, Garland EJ. Pilot study of community-based cognitive behavioral group therapy for adolescents with social phobia. J Am Acad Child Adolesc Psychiatry. 2005;44:258. Eggers CH. Treatment of acute and chronic psychoses in childhood and adolescence. MMW Fortschr Med. 2005;147:43. Haen C. Rebuilding security: Group therapy with children affected by September 11. Int J Group Psychother. 2005;55:391. Kreidler M. Group therapy for survivors of childhood sexual abuse who have chronic mental illness. Arch Psychiatr Nurs. 2005:19:176. 47 - 31.18c Residential, Day, and Hospital Treatme 31.18c Residential, Day, and Hospital Treatment Laugeson EA, Frankel F, Gangman A, Dillon AR, Mogil C. Evidence-based social skills training for adolescents with autism spectrum disorders: The UCLA PEERS Program. J Autism Dev Discord. 2012;42:1025–1036. Laugeson EA, Frankel F. Social Skills for Teenagers with Developmental and Autism Spectrum Disorders: The PEERS Treatment Manual. New York: Routledge; 2010. Liddle HA, Rowe CL, Dakof GA, Ungaro RA, Henderson CE. Early intervention for adolescent substance abuse: Pretreatment to posttreatment outcomes of a randomized clinical trial comparing multidimensional family therapy and peer group treatment. J Psychoactive Drugs. 2004;36:49. Manassis K, Mendlowitz SL, Scapillato D, Avery D, Fiksenbaum L, Freire M, Monga S, Owens M. Group and individual cognitive-behavioral therapy for childhood anxiety disorders: A randomized trial. J Am Acad Child Adolesc Psychiatry. 2002:41:14243. Mishna F, Muskat B. “I’m not the only one!” Group therapy with older children and adolescents who have learning disabilities. Int J Group Psychother. 2004;54:455. Muris P, Meesters C, van Melick M. Treatment of childhood anxiety disorders: A preliminary comparison between cognitive-behavioral group therapy and a psychological placebo intervention. J Behav Ther Exp Psychiatry. 2002;33:143. O’connor MJ, Laugeson EA, Mogil C, Lowe E, Welch-Torres K, Keil V, Paley B. Translation of an evidence-based social skills intervention for children with prenatal alcohol exposure in a community mental health setting. Alcohol Clin Exp Res. 2012;36:141–152. Stallard P, Sayal K, Phillips R, Tahylor JA, Spears M, Anderson R, Araya R, Lewis G, Millings A, Montgomery AA. Classroom based cognitive behavioural therapy in reducing symptoms of depression in high risk adolescents: Pragmatic cluster randomised controlled trial. BMJ. 2012;345:e6058. Thienemann ML. Child psychiatry: Group psychotherapy. In: Sadock BJ, Sadock VA, Ruiz P, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Vol. 2. Philadelphia: Lippincott Williams & Wilkins; 2009:3731. Wilens TE, Rosenbaum JF. Transitional aged youth: A new frontier in child and adolescent psychiatry. J Am Acad Child Adolesc Psychiatry. Sep 2013;52(9):887–890. 31.18c Residential, Day, and Hospital Treatment Inpatient, partial hospital, and residential treatment are designed for the management of acute stabilization, stepdown care, and longer-term management of children and adolescents with psychiatric disorders. Given the limited number of psychiatric inpatient units for children and adolescents, however, intensive outpatient programs and partial hospital treatment programs are often used for children with severe psychiatric disorders. Partial hospital programs are increasingly being offered by managed care companies as alternatives to hospitalization to contain treatment cost. These programs are designed to serve the needs of children and adolescents with severe disorders who require immediate psychosocial and/or pharmacological; interventions but who may not meet the acuity criteria of “medical necessity” for hospitalization. Residential treatment centers are appropriate settings for children and adolescents with psychiatric disorders who require a highly structured and supervised setting for several months or longer. Such settings provide a stable, consistent environment with a high level of psychiatric monitoring that is less intensive than in a hospital. Children and adolescents with serious psychiatric disturbances are sometimes admitted to residential facilities due to family situations in which appropriate supervision and parenting are impossible. Dan was a 16-year-old adolescent boy with a long history of depression and multiple suicide attempts. He was admitted to a local adolescent psychiatric inpatient unit after for a life-threatening suicide attempt. At the end of the first week of hospitalization, Dan’s family’s managed care company refused continued coverage, since they determined that he was no longer an acute suicide risk. Dan was remorseful about his recent suicide attempt and was determined not to repeat his self-destructive behavior. However, due to continued serious depressive symptoms and chronic family dysfunction, the inpatient treatment team did not feel that Dan was ready to be discharged to weekly outpatient treatment. Dan was transferred to a partial hospital program affiliated with the inpatient unit. Over the course of Dan’s 8-week treatment, he developed a strong therapeutic alliance with his individual therapist, and the psychoeducation provided to the family resulted in the beginning of meaningful changes. The partial hospital program child psychiatrist met with Dan regularly, managed his medication, and collaborated with his therapist to manage his suicidal ideation. At the end of 8 weeks, Dan’s depressive symptoms were decreased, and he was safely transitioned to outpatient therapy and returned to school successfully. The partial hospital program allowed for a safe transition from full hospitalization with continued consolidation of progress in a highly structured system. (Adapted from case material courtesy of Laurel J. Kiser, Ph.D., M.B.A., Jerry Heston M.D., and David Pruitt, M.D.) Mark was an 8-year-old boy referred to a rural community mental health center for evaluation and treatment. Mark presented with extreme irritability, labile mood, tantrums, and physical violence toward his peers and adults. Even when he was not having a tantrum, he seemed discontent and irritated and had a short fuse. He had received multiple school suspensions and was at risk for expulsion. His family psychiatric history was positive for schizophrenia in his maternal grandmother. Upon finishing his outpatient psychiatric evaluation, the clinician recommended participation in a newly established partial hospital/day treatment program that used a behavioral management program close to Mark’s elementary school. The clinician also recommended a trial of fluoxetine to determine whether Mark’s irritability would be ameliorated, and individual therapy, social skills group, and family therapy. During Mark’s 6-month participation in the day program, his behavioral management program extended into the classroom setting as well as in therapeutic activities. His daily goals included increasing compliance, decreasing anger outbursts, and decreasing physical aggression. He was able to improve peer relations while receiving immediate feedback and direct instruction on social skills in a group setting and also in his individual therapy. Each staff member was able to consistently apply behavior management principles in their domain areas. Mark’s parents actively participated in family therapy sessions and parent conferences. Mark seemed to be benefitting from the fluoxetine and was less irritable. Although he still had occasional outbursts, they were milder and shorter. Mark was gradually transitioned to half a day in a regular classroom setting, and he remained the other half day in the day program. After 8 more weeks of this transition, he was able to return to his public school. (Adapted from case material courtesy of Laurel J. Kiser, Ph.D., M.B.A., Jerry Heston, M.D., and David Pruitt, M.D.) HOSPITALIZATION Psychiatric hospitalization is necessary when a child or adolescent is contemplating or exhibiting dangerous behaviors directed at him or herself or toward others. The most frequent reasons for psychiatric hospitalization among youth include suicidal thoughts or behavior, and aggressive and assaultive behaviors. Safety, stabilization, and initiation of effective treatment are the main goals of hospitalization. In some cases, psychiatric hospitalization may be a given child’s first experience of a stable, safe environment. Hospitals are often the most appropriate places to initiate a new psychopharmacological agent, especially when side effects are prevalent, in order to provide around-the-clock observations of a child’s behavior. Children who have been maltreated often show remission of certain symptoms by virtue of being removed from a stressful and abusive environment. Given the frequency of uncontrollable aggression as the trigger for many psychiatric admissions among youth, inpatient units must provide safe and effective ways to defuse and stabilize aggressive and violent acts. Placing a child or adolescent on the verge of a violent act in a contained room away from the rest of the milieu is one method of de-escalating a potentially violent situation. Both restraint and seclusion have been considered therapeutic interventions for youth who cannot control aggressive impulses, but given the rare but reported deaths of patients by asphyxiation during restraint procedures, there have been efforts to reduce this intervention. However, seclusion and restraint cannot be abandoned until another form of intervention is found to be highly effective. In some cases, psychopharmacological interventions, that is, “chemical restraint,” has been utilized to defuse acutely dangerous situations on an inpatient unit. Optimally, identifying and recognizing antecedents of aggressive behaviors and intervening before the aggression is enacted is the goal. Inpatient care is a setting for stabilization and the initiation of treatment, with the expectation that when a child or adolescent is discharged to a less restrictive environment, the patient will no longer pose a danger to him or herself or others, and that treatment and support services will be in place for continued care. Partial Hospital In most cases, children and adolescents who attend partial hospital, or day treatment programs, have serious mental disorders and might warrant psychiatric hospitalization without the program’s support. Family therapy, group and individual psychotherapy, psychopharmacology, behavioral management programs, and special education are integral parts of these programs. Partial hospital programs are excellent alternatives for children and adolescents who require more intensive support, monitoring, and supervision than is available in the community, but who can live successfully at home if they receive the proper level of intervention. The concept of daily comprehensive therapeutic experiences that do not require removing children from their homes or families is derived partly from experiences with a therapeutic nursery school. The main advantages of partial hospital programs are that children remain with their families and the families can be more involved in day treatment than they are in residential or hospital treatment. Partial hospital also is much less expensive than residential treatment. At the same time, the risks of day treatment include a child’s relative social isolation and confinement to a narrow band of social contacts in the program’s disturbed peer population. Indications. The primary indication for a partial hospital plan is the need for a more structured, intensive, and specialized treatment program than can be provided on an outpatient basis. At the same time, the home in which the child is living should be able to provide an environment that is at least not destructive to the child’s development. Children who are likely to benefit from day treatment may have a wide range of diagnoses, including autistic disorder, conduct disorder, ADHD, and mental retardation. Exclusion symptoms include behavior that is likely to be destructive to the children themselves or to others under the treatment conditions. Therefore, some children who threaten to run away, set fires, attempt suicide, hurt others, or significantly disrupt the lives of their families while they are at home are not suitable for day treatment. Programs. Ingredients that lead to a successful partial hospital program include clear administrative leadership, team collaboration, open communication, and an understanding of children’s behavior. A major function of child-care staff in partial hospital programs is to provide positive experiences and a structure that enables the children and their families to internalize controls and to function better than in the past. Because the ages, needs, and range of diagnoses of children who may benefit from some form of day treatment vary, many day treatment programs have been developed. Some programs specialize in the special educational and structured environmental needs of mentally retarded children. Others offer therapeutic efforts designed to treat children with autism and schizophrenia. Still other programs provide the total spectrum of treatment usually found in full residential treatment, of which they may be an extension. Children may move from one part of the program to another and may be in residential treatment or partial hospital according to their needs. A school program is always a major component of partial hospital treatment. Attempts have been made to analyze the treatment outcome of partial hospitalization. Many different dimensions exist to analyze the overall benefits of such programs; assessment of level of improvement in clinical status, academic progress, peer relationships, community interactions (legal difficulties), and family relationships are some pertinent areas to measure. In a follow-up 1 year after discharge from a partial hospital program, comparison of patients at admission and 1-year post-discharge showed statistically significant improvement in clinical symptoms on each subscale of the Child Behavior Checklist, except for sex problems. Improvements were found in mood, somatic complaints, attention problems, thought problems, delinquent behavior, and aggressive behavior. The assessment of long-term effectiveness of day treatment is fraught with difficulties, and may differ when measuring a child’s maintenance of gains, a therapist’s view of psychological gains, or cost-to-benefit ratios. The lessons learned from day treatment programs have encouraged mental health disciplines to have services follow children, rather than have separate programs, which result in discontinuity of care. The experiences of partial hospital programs for psychiatric conditions of children and adolescents have also encouraged pediatric hospitals and departments to adopt models that promote continuity of care for children with chronic physical illness. RESIDENTIAL TREATMENT Children in residential treatment often have combinations of severe psychiatric disorders and severely troubled families who cannot adequately care for their children. In some cases, a child or adolescent requires a more structured environment than is possible at home. In other cases, a family is unable to oversee a child’s psychiatric treatment due to their own psychiatric illness, substance abuse, or medical debilitation. In cases of child abuse or neglect, a family does not provide a safe and nurturing environment for a child. When families are available and motivated, their participation is strongly encouraged while their children are in residential treatment. The aim is to enable them to reunite with their children and care for them at home in the future. Staff and Setting Staffing patterns include various combinations of child-care workers, teachers, social workers, psychiatrists, pediatricians, nurses, and psychologists; therefore, residential treatment can be very expensive. The Joint Commission on the Mental Health of Children made the following structural and setting recommendations: In addition to space for therapy programs, there should be facilities for a first-rate school and a rich evening activity program, and there should be ample space for play, both indoors and out. Facilities should be small, seldom exceeding 60 patients in capacity with a limit of 100 patients, and they should make provisions for children to live in small groups. The centers should be located near the families they serve and should be readily accessible by public transportation. They should be located for ready access to special medical and educational services and to various community resources, including consultants. The centers should be open institutions whenever possible; locked buildings, wards, or rooms should be required only rarely. In designing residential programs, the guiding principle should be that children should be removed from their normal life settings the least possible distance in space, in time, and in the psychological texture of the experience. Indications Most children who are referred for residential treatment have had multiple evaluations by professionals, such as school psychologists, outpatient psychotherapists, juvenile court officials, or state welfare agency staff. Attempts at outpatient treatment and foster home placement usually precede residential treatment. Sometimes, the severity of a child’s problems or the inability of a family to provide for the child’s needs prohibits sending a child home. Many children sent to residential treatment centers have disruptive behavior problems in addition to other problems, including mood disorders and psychotic disorders. In some cases, serious psychosocial problems, such as physical or sexual abuse, neglect, indigence, or homelessness, necessitate out-of-home placement. The age range of the children varies among institutions, but most children are between 5 and 15 years of age. Boys are referred more frequently than girls. An initial review of data enables the intake staff to determine whether a particular child is likely to benefit from the treatment program; often, for every child accepted for admission, three are rejected. The next step usually is interviews with the child and the parents by various staff members, such as a therapist, a group-living worker, and a teacher. Psychological testing and neurological examinations are given, when indicated, if they have not already been performed. The child and parents should be prepared for these interviews. Milieu Most of a child’s time in a residential treatment setting is spent in the milieu. The staff consists of clinicians and care workers who offer a structured environment that forms a therapeutic milieu; the environment places boundaries and limitations on the children. Tasks are defined within the limits of children’s abilities; incentives, such as additional privileges, encourage them to progress rather than regress. In milieu therapy, the environment is structured, limits are set, and a therapeutic atmosphere is maintained. The children often select one or more staff members with whom to form a relationship; through this relationship, they express, consciously and unconsciously, many of their feelings about their parents. The child-care staff should be trained to recognize such transference reactions and to respond to them in a way that differs from the children’s expectations, which are based on their previous or even current relationships with their parents. This requires an awareness of countertransference in staff members. To maintain consistency and balance, the group-living staff members must communicate freely and regularly with each other and with the other professional and administrative staff members of the residential setting, particularly the children’s teachers and therapists. Behavior modification principles are typically embedded into the daily program for children in residential settings. A recent study examined the association between use of antipsychotic medication and seclusion/restraint (S/R) frequency in the management of acute aggressive behavior in adolescents in residential facilities. Adolescents who were in the moderate and high groups for having S/R were significantly more likely to have changes in antipsychotic medication and receive higher doses of medication. However, even with high doses, their rates of S/R continued to be frequent. These findings bring into question the efficacy of antipsychotic agents for managing acute aggression in residential settings. Education Children in residential treatment frequently have severe learning disorders, disruptive behavior, and ADHD. Usually, the children cannot function in a regular community school and consequently need a special on-grounds school. A major goal of the ongrounds school is to motivate children to learn. The educational process in residential treatment is complex; Table 31.18c-1 shows its components. Table 31.18c-1 Education Process in Residential Treatment Therapy Most residential facilities use a basic behavior modification program to set guidelines and to give the residents a concrete sense of how to earn privileges. These behavioral programs range in detail and intensity. Some programs operate with level systems that are associated with privileges and responsibilities. Some programs use a token economy system in which residents earn points for appropriate behavior and for meeting specific goals. Most programs include basic tasks of living as well as specific therapeutic goals for the residents. Psychotherapy offered in these programs generally is supportive and oriented toward reunion with the family when possible. Insight-oriented psychotherapy is included when it can be used by a resident. Parents Concomitant work with parents is essential. Children usually have a strong tie to at least one parent, no matter how disturbed the parent may be. Sometimes, a child idealizes the parent, who repeatedly fails the child. Other times, the parent has an ambivalent or unrealistic expectation that the child will return home. In some instances, the parent must be helped to enable the child to live in another setting when it is in the child’s best interest. Most residential treatment centers offer individual or group therapy for parents, couples, or marital therapy, and in some cases, conjoint family therapy. DAY TREATMENT The concept of daily comprehensive therapeutic experiences that do not require removing children from their homes or families is derived partly from experiences with a therapeutic nursery school. Day hospital programs for children were then developed, and the number of programs continues to grow. The main advantages of day treatment are that children remain with their families and the families can be more involved in day treatment than they are in residential or hospital treatment. Day treatment also is much less expensive than residential treatment. At the same time, the risks of day treatment are a child’s social isolation and confinement to a narrow band of social contacts in the program’s disturbed peer population. Indications The primary indication for day treatment is the need for a more structured, intensive, and specialized treatment program than can be provided on an outpatient basis. At the same time, the home in which the child is living should be able to provide an environment that is at least not destructive to the child’s development. Children who are likely to benefit from day treatment may have a wide range of diagnoses, including autistic disorder, conduct disorder, ADHD, and mental retardation. Exclusion symptoms include behavior that is likely to be destructive to the children themselves or to others under the treatment conditions. Therefore, some children who threaten to run away, set fires, attempt suicide, hurt others, or significantly disrupt the lives of their families while they are at home may not be suitable for day treatment. Programs The same ingredients that lead to a successful residential treatment program apply to day treatment. These ingredients include clear administrative leadership, team collaboration, open communication, and an understanding of children’s behavior. Indeed, having a single agency offer both residential and day treatment has advantages. A major function of child-care staff in day treatment for psychiatrically disturbed children is to provide positive experiences and a structure that enables the children and their families to internalize controls and to function better than in the past regarding themselves and the outside world. Again, the methods used are essentially similar to those in full residential treatment programs. Because the ages, needs, and range of diagnoses of children who may benefit from some form of day treatment vary, many day treatment programs have been developed. Some programs specialize in special educational and structured environmental needs of mentally retarded children. Others offer special therapeutic efforts required to treat children with autism and schizophrenia. Still other programs provide the total spectrum of treatment usually found in full residential treatment, of which they may be a part. Children may move from one part of the program to another and may be in residential treatment or day treatment according to their needs. The school program always is a major component of day treatment, and psychiatric treatment varies according to a child’s needs and diagnosis. Results Recently, attempts have been made to analyze the treatment outcome of day treatment and partial hospitalization. Many different dimensions exist to analyzing overall benefits of such programs. Assessment of level of improvement in clinical status, academic progress, peer relationships, community interactions (legal difficulties), and family relationships are some pertinent areas to measure. In a recent follow-up 1 year after discharge from a partial hospital program, comparison of patients at admission and 1-year post-discharge showed statistically significant improvement in clinical symptoms on each subscale of the Child Behavior Checklist, except for sex problems. These improvements were in mood symptoms, somatic complaints, attention problems, thought problems, delinquent behavior, and aggressive behavior. The assessment of long-term effectiveness of day treatment is fraught with difficulties, from the point of view of a child’s maintenance of gains, a therapist’s view of psychological gains, or costto-benefit ratios. At the same time, the advantage of day treatment has encouraged further development of programs. Moreover, the lessons learned from day treatment programs have moved mental health disciplines toward having services follow children, rather than perpetuating discontinuities of care. The experiences of day treatment for psychiatric conditions of children and adolescents have also encouraged pediatric hospitals and departments to adopt a model that promotes continuity of care for the medical treatment of children with chronic physical illnesses. REFERENCES Aarons GA, James S, Monn AR, Raghavan R, Wells RS, Leslie LK. Behavioral problems and placement change in a national child welfare sample: A prospective study. J Am Acad Child Adolesc Psychiatry. 2010;49:70–80. Baeza I, Correll CU, Saito E, Aranbekova D, Kapoor S, Chekuri R, De Hert M, Carbon M. Frequency, characteristics and management of adolescent inpatient aggression. J Child Adolesc Psychopharmacol. 2013;23:271–281. Damnjanovic M, Lakic A, Stevanovic ED, Jovanovic A. Effects of mental health on quality of life in children and adolescents living in residential and foster care: a cross-sectional study. Epidemiol Psychiatr Sci. 2011;20:257–262. Epstein RA Jr. Inpatient and residential treatment effects for children and adolescents: A review and critique. Child Adolesc Psychiatr Clin N Am. 2004;13:411. Geller JL, Biebel K. The premature demise of public child and adolescent inpatient beds: Part I: Overview and current conditions. Psychiatric Q. 2006;77:251. Geller JL, Biebel K. The premature demise of public child and adolescent inpatient beds: Part II: Challenges and implications. Psychiatr Q. 2006;77(4):273–291. Kober D, Martin A. Inpatient psychiatric, partial hospital and residential treatment for children and adolescents. In: Sadock BJ, Sadock VA, Ruiz P, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Philadelphia: Lippincott Williams & Wilkins; 2009:3766. Miller L, Riddle MA, Pruitt D, Zachik A, DosReis S. Antipsychotic treatment patterns and aggressive behavior among 48 - 31.18d Pharmacotherapy 31.18d Pharmacotherapy adolescents in residential facilities. J Behav Health Serv Res. 2013;40:97–110. Noftle JW, Cook S, Leschied A, St Pierre J, Stewart SL, Johnson AM. The trajectory of change for children and youth in residential treatment. Child Psychiatry Hum Dev. 2011;42:65–77. 31.18d Pharmacotherapy Over the last decade, increasing evidence has emerged regarding the efficacy and safety of psychopharmacological agents to treat child and adolescent psychiatric disorders. Randomized placebo-controlled trials have confirmed the short-term efficacy of selective serotonin reuptake inhibitors (SSRIs), for depressive disorders, anxiety, and OCD; second-generation antipsychotics (SGAs) for psychosis and aggression; and multiple central nervous system stimulants for ADHD. Published data support the short-term efficacy and safety of fluoxetine, sertraline, fluvoxamine, and escitalopram in the treatment of youth depression, anxiety disorders and OCD. First-line evidence-based treatment for ADHD, has preferentially shifted toward longacting stimulant medications, including methylphenidate preparations (Concerta) and amphetamine and amphetamine salt preparations (Adderall XR). Significant progress in the field has been made through multi-site, National Institute of Mental Health (NIMH)–funded research comparing types of treatment with treatment combinations of pharmacological interventions and psychosocial treatments, for disorders including OCD and major depressive disorders, and anxiety disorders. Studies repeatedly found that cognitive-behavioral psychotherapy in combination with an SSRI has advantages over either alone. Another area of progress has been evidence-based treatment of ADHD in younger age groups. The NIMH Preschooler with ADHD Treatment Study (PATS) was the first multisite study of ADHD preschool children, treated first with a parent training component and followed, if necessary, by administration of methylphenidate. This regimen was found to be effective and safe. Double-blind, placebo-controlled studies have provided evidence for the efficacy of fluoxetine, sertraline, and escitalopram treatment for depressive disorders in youth, and the Food and Drug Administration (FDA) has approved both fluoxetine and escitalopram in the treatment of adolescent depression. Fluoxetine, sertraline, and fluvoxamine have been shown to have positive results based on randomized controlled trials (RCTs) in the treatment of OCD in youth. Although the FDA has not yet approved SSRIs in the treatment of child and adolescent anxiety, positive random-controlled trials (RCTs) exist for fluoxetine, sertraline, paroxetine, and fluvoxamine in the treatment of youth anxiety. In 2004, the FDA released a statement on the recommendation of the Psychopharmacologic Drugs and Pediatric Advisory Committees of a “black-box” warning relating to an increased risk for suicidality in pediatric patients for all antidepressant medications. The advisory committees came to the conclusion that an increased risk of suicidal behaviors existed, although there were no suicides completed among the data reviewed. All of the antidepressant medications must include the black box warning for pediatric patients regardless of whether they have been studied in pediatric populations. Currently, the SGAs, also known as serotonin-dopamine antagonists (SDAs), have generally replaced the conventional antipsychotics (dopamine receptor antagonists) in the treatment of psychotic disorders and for aggressive behavior management. THERAPEUTIC CONSIDERATIONS As psychopharmacologic interventions for childhood psychiatric disorders have gained an evidence base, establishing a therapeutic alliance, identifying and monitoring target symptoms, and promoting medication compliance are important components of successful clinical outcomes. Teamwork between the child, parents, and psychiatrist is critical in successful treatment of childhood disorders with psychopharmacologic agents. An evaluation for psychopharmacotherapy must first include an assessment of a child’s psychopathology and physical condition to rule out any predisposition for side effects (Table 31.18d-1). An assessment of the child’s caregivers focuses on their ability to provide a safe, consistent environment in which a clinician can conduct a drug trial. The physician must consider the risk-to-benefit ratio and must explain it to the patient, if he or she is old enough, and to the child’s caregivers and others (e.g., child welfare workers) who may be involved in the decision to medicate. Table 31.18d-1 Diagnostic Processes of Biological Therapy The clinician must obtain baseline ratings before medicating. Behavioral rating scales help objectify the child’s response to medication. The physician generally starts at a low dose and titrates upward on the basis of the child’s response and the appearance of adverse effects. Optimal drug trials cannot be rushed (e.g., by insurance-imposed, inadequately short hospital stays or by infrequent outpatient visits), nor can drug trials be prolonged by the physician’s insufficient contact with the patient and the caregivers. The success of drug trials often hinges on the physician’s daily accessibility. CHILDHOOD PHARMACOKINETICS Compared with adults, children have greater hepatic capacity, more glomerular filtration, and less fatty tissue. Thus, stimulants, antipsychotics, and tricyclic drugs are eliminated more rapidly by children than by adults; lithium (Eskalith) may also be eliminated more rapidly, and children may be less able to store drugs in their fat. Because of children’s quick elimination, the half-lives of many medications may be shorter in children than in adults. Little evidence indicates that clinicians can predict a child’s blood level from the dosage or a treatment response from the plasma level. Relatively low serum levels of haloperidol seem to be adequate to treat Tourette’s disorder in children. No correlation is seen between the methylphenidate (Ritalin) serum level and a child’s response. The data are incomplete and conflicting about major depressive disorder and serum levels of tricyclic drugs. Serum level is related to response for tricyclic drugs in the treatment of enuresis. With lithium therapy, a ratio of lithium concentration in saliva to that in serum can be established for a child by averaging three to four individual ratios. The average ratio can then be used to convert subsequent saliva levels to serum levels and, thus, avoid some venipuncture in children who are stressed by blood tests. As with serum levels, regular clinical monitoring for adverse effects is necessary. Table 31.18d-2 lists representative agents, their indications, dosages, adverse reactions, and monitoring requirements. Table 31.18d-2 Pharmacologic Agents for Psychiatric Disorders in Children Adolescents STIMULANT AGENTS, ATOMOXETINE, AND α-AGONIST AGENTS Stimulant pharmacologic agents remain the primary treatment for ADHD in children, adolescents, and adults. Multiple studies support the efficacy of stimulant medications for ADHD. Current practice is leaning toward more use of once-a-day, long-acting preparations of stimulants such as methylphenidate, amphetamine and amphetamine salts, and dex-methylphenidate (Focalin LA). The most frequently researched and used stimulant is methylphenidate. Dextroamphetamine (Dexedrine) has comparable efficacy and, unlike methylphenidate, is approved by the FDA for children 3 years of age and older; the starting age for methylphenidate is 6 years. The amphetamine, Adderall, combines dextroamphetamine and amphetamine salts. The extended-release preparations, such as Concerta and Adderall XR, have the advantages of coverage of symptoms throughout the school day without the necessity of taking another dose, as well as a more continuous delivery of medication. Stimulants reduce hyperactivity, inattentiveness, and impulsivity in about 75 percent of children with ADHD. The effects are not paradoxical, because normal children respond similarly. The dose-related adverse effects of stimulants are listed in Table 31.18d-3. Table 31.18d-3 Common Dose-Related Side Effects of Stimulants The methylphenidate transdermal patch (Daytrana) is approved by the FDA for the treatment of ADHD in children age 6 to 12 years. Daytrana comes in patches that can deliver 15 mg, 20 mg, and 30 mg when worn for 9 hours per day. The medication begins to have its effects on the target symptoms of ADHD approximately 2 hours after the patch is placed, and continues to deliver medication throughout the wear time. Given that its active ingredient is methylphenidate, the side effects are generally the same as those for methylphenidate, except for the potential skin irritation that may emerge from wearing the patch. The patch should not be worn in the presence of a heating pad or electric blanket because heat increases the rate of methylphenidate delivery into the skin. Patients with glaucoma or known hypersensitivity to methylphenidate products should not begin treatment with Daytrana. Daytrana has the advantages of being able to deliver medication until the patch is removed and, for children who are unable to swallow pills, Daytrana offers a unique administration option. Lisdexamfetamine dimesylate (LDX), sold as Vyvanse, is a pro-drug of dextroamphetamine upon cleavage of the lysine portion of the molecule. LDX was created to be longer lasting than dextroamphetamine and less likely to become a drug of abuse because it requires enzymes to convert it to dextroamphetamine. LDX has been approved by the FDA for the treatment of ADHD in children 6 to 12 years of age as well as in adults with ADHD. In contrast to Adderall, which contains approximately 75 percent dextroamphetamine, LDX comprises the dextro enantiomer only. In trials, LDX has also been shown to be effective and safe in the treatment of adolescents with ADHD. Similar to other stimulant agents, the most common side effects of LDX were decreased appetite, headache, insomnia, decreased weight, and irritability. Recent studies support the use of atomoxetine (Strattera), a norepinephrine reuptake inhibitor, as an efficacious nonstimulant treatment for ADHD in children and adolescents. Atomoxetine is well absorbed after ingestion and reaches its maximal plasma concentration after about 1 to 2 hours. Common side effects of atomoxetine include abdominal discomfort, decreased appetite, dizziness, and irritability. Rarely, minor increases in blood pressure and heart rate have been noted. Atomoxetine is metabolized by the cytochrome P450 (CYP) 2D6 hepatic enzyme system, and a fraction of the population (about 7 percent of Caucasians and 2 percent of African Americans) are poor metabolizers, which may increase the plasma half-life by about fivefold. When combined with other medications that inhibit CYP 2D6, such as fluoxetine and paroxetine, diminished metabolism of atomoxetine can occur, and the dose may need to be decreased. Atomoxetine is generally initiated at 0.5 mg/kg given once per day and increased to a therapeutic dose ranging between 1.4 mg/kg and 1.8 mg/kg, either in one dose or in two divided doses. SECOND GENERATION ANTIPSYCHOTICS (SGAS) AND CONVENTIONAL ANTIPSYCHOTIC AGENTS The SGAs represent a major advance in the pharmacological treatment of schizophrenia in children and adolescents, as well as in adults. The atypical antipsychotic agents have largely replaced traditional antipsychotics because of their more favorable side-effect profiles, greater effectiveness for negative symptomatology, and mood-stabilizing effects. Although the SGAs are generally recommended currently as first-line agents in the treatment of psychotic disorders in children and adolescents, only one controlled NIMH trial has been conducted using an atypical agent in the treatment of schizophrenia for youth. This study examined clozapine and found it to be superior to haloperidol for treating positive and negative symptoms of schizophrenia in 21 youth. The serious drawbacks of clozapine, however, limit it as a first-line agent for this disorder. In the NIMH trial, five participants developed significant neutropenia, and two experienced seizures. Clozapine is generally used only for treatment-resistant schizophrenia. Open label trials in youth with schizophrenia have suggested efficacy of other atypical antipsychotic agents such as olanzapine, risperidone, and quetiapine. Case reports have suggested that ziprasidone is effective. One of the main side effects of the atypical antipsychotic agents is significant weight gain. A newer atypical agent, aripiprazole awaits clinical trials to confirm its potential to be an efficacious and more weight neutral agent for the treatment of childhood psychoses. Although conventional antipsychotics, such as haloperidol, loxapine (Loxitane), and thioridazine (Mellaril) have been shown to be significantly superior to placebo in the treatment of psychosis in youth, given their side effect profiles they are typically chosen as first-line treatments. Schizophrenia with onset in late adolescence is treated, as is adult-onset schizophrenia. Aggressive, explosive, and assaultive behaviors associated with disruptive behavior disorders, psychotic disorders, and posttraumatic stress disorders have been treated with antipsychotic agents with varying reports of success. Randomized controlled trials with several atypical antipsychotics, such as risperidone, olanzapine, quetiapine (Seroquel), and aripiprazole (Abilify), have provided evidence of effectiveness for behavioral improvement, with fewer long-term adverse effects than typical antipsychotics. When conduct disorder is associated with ADHD, a trial of a stimulant is indicated; stimulants are faster acting than atypical antipsychotics or mood-stabilizing agents used in clinical practice to control dangerously aggressive behaviors. The management of severe aggression, disruptive behavior, and ADHD remains a challenge. Combinations of antipsychotics with mood-stabilizing agents or stimulants are sometimes used in treatment-resistant cases, although few studies attest to the efficacy or safety of drug combinations. Newer “atypical” antipsychotic medications— SDAs—such as risperidone, olanzapine, clozapine (Clozaril), ziprasidone (Geodon), and aripiprazole have enabled a wider range of treatment-resistant patients to benefit from neuroleptic treatment. The SDAs are believed to relieve both the positive and negative symptoms of schizophrenia and to produce less risk of extrapyramidal adverse effects and less potential for the development of tardive dyskinesia. Nevertheless, all antipsychotics pose some risk of extrapyramidal adverse effects and tardive dyskinesia. One challenge in obtaining optimal pharmacological treatment for children is to decrease maladaptive behaviors while promoting productive academic functioning. To this end, clinicians must consider adverse effects of medication that result in cognitive “dulling.” Certain pharmacological agents used in pediatric populations are associated with a specific disorder or with target symptoms that appear in several disorders. For example, haloperidol was shown in past studies to be effective in the treatment of Tourette’s disorder, but it has also been used to control severe aggression. The highpotency antipsychotics haloperidol and pimozide (Orap) still have the greatest body of evidence as effective medications for Tourette’s disorder, although they also have considerable drawbacks. Pimozide prolongs the QT interval and, thus, requires electrocardiography (ECG) monitoring. Clonidine, a presynaptic α-adrenergic blocking agent, is less effective than either of the above-mentioned antipsychotics, but has the advantage of avoiding the risk for tardive dyskinesia; sedation is a frequent side effect of clonidine. Tic disorders often coexist with ADHD in children and adolescents. Stimulant use is controversial; it can precipitate tics and should be avoided in these patients, although recent studies indicate that the prohibition may not be totally warranted. Clonidine sometimes reduces tics in both ADHD and the comorbid cases. SELECTIVE SEROTONIN REUPTAKE INHIBITOR (SSRI) ANTIDEPRESSANTS AND OTHER ANTIDEPRESSANTS SSRI antidepressants have been found in randomized clinical trials to have efficacy in the treatment of childhood anxiety disorders, depressive disorders, and OCD. A substantial evidence base exists for the efficacy of SSRIs in the treatment of separation anxiety, generalized anxiety disorder, and social phobia in children and adolescents. Thus, SSRIs are currently recommended as first-line medications in the treatment of childhood anxiety. Separation anxiety disorder, generalized anxiety disorder, and social phobia are often studied together because they so commonly coexist. A given child with one of the preceding anxiety disorders has a 60 percent chance of having a second one, and in 30 percent of cases, all three are comorbid. Alprazolam (Xanax) may be helpful in separation anxiety disorder, but randomized clinical trials are still needed. The SSRIs currently are the drugs of choice in the pharmacological treatment of depressive disorders in children and adolescents. Given the FDA placement of the “blackbox” warning on all antidepressants used in children and adolescents because of the slightly increased risk of suicidal behaviors, close monitoring of suicidal ideation and behavior is imperative. Although most side effects of SSRIs are tolerable, anecdotal recent reports indicate occasional SSRI-induced apathy in children and adolescents. Previously, clomipramine proved effective in diminishing obsessions and compulsions in children and adolescents and was generally well tolerated. However, the SSRIs have a more favorable adverse-effect profile and appear to be as effective as clomipramine. MOOD-STABILIZING AGENTS Classic mania in children and adolescents is treated as it is treated in adults. Use of lithium in treating adolescent mania has been supported in many open trials. Divalproex is used frequently to treat bipolar disorder in children and adolescents. A recent double-blind, randomized pilot study comparing quetiapine (400 to 600 mg a day) or divalproex (serum level 80 to 120 mg/mL) in a trial lasting approximately 1 month, found that quetiapine is at least as effective as divalproex in treating acute manic symptoms. Reduction of symptoms occurred more quickly with quetiapine compared with divalproex. Lithium has been shown in multiple investigations to reduce aggression in conduct disorder, and propranolol (Inderal) has been chosen as an agent to control aggression in open trials, although no evidence supports its use in children and adolescents. Carbamazepine (Tegretol) has not been shown to be effective in controlling aggression in child and adolescent conduct disorders. Table 31.18d-4 summarizes the effects of drugs on cognitive tests of learning functions. In children with learning disorders who have attention problems, even in the absence of meeting full criteria for ADHD, methylphenidate facilitates performance on several standard cognitive, psycholinguistic, memory, and vigilance tests, but does not improve children’s academic achievement ratings or teacher ratings. Cognitive impairment from psychotropic drugs, especially antipsychotics, may be an even greater problem for persons who are mentally retarded than for those with learning disorders. Table 31.18d-4 Effects of Psychotropic Drugs on Cognitive Tests of Learning Functionsa BENZODIAZEPINES Sleep terror disorder and sleepwalking disorder occur in the transition from deep deltawave sleep (stages 3 and 4) to light sleep. Benzodiazepines may be effective in these disorders. They work by reducing both delta-wave sleep and arousals between sleep stages. The medications should be used temporarily and only in severe cases, because tolerance to the medications develops. Cessation of these medications can lead to severe rebound worsening of the disorders, and reducing delta sleep in children may have deleterious effects; thus, behavioral approaches are preferred for these disorders. Patients with early-onset panic disorder and panic attacks have benefited from clonazepam (Klonopin) in several open trials. DESMOPRESSIN Desmopressin (DDAVP) is effective in about 50 percent of patients with intractable enuresis. Improvements with DDAVP range from diminished wetting with less urine volume, to complete cessation of bedwetting. Desmopressin has been used intranasally in dosages of 10 to 40 mg a day. When used over months, nasal discomfort can occur, and water retention is potentially a problem. Patients who respond with full dryness should continue to take the medication for several months to prevent relapses. Desmopressin is now available in oral tablets, and a controlled multicenter study found equal efficacy between intranasal and oral administration of desmopressin in the treatment of enuresis. A dose of 400 mg of oral desmopressin was the study condition associated with greater effectiveness than the lower 200 mg used. ADVERSE EFFECTS AND COMPLICATIONS Antidepressants Adverse effects related to antidepressants have diminished significantly since SSRI antidepressants have been widely accepted as first-line treatments for depressive disorders in children and adolescents. Tricyclics are rarely recommended because of the significant risks of dangerous adverse effects. The adverse effects of tricyclics for children usually are similar to those for adults and result from the drugs’ anticholinergic properties. These effects include dry mouth, constipation, palpitations, tachycardia, loss of accommodation, and sweating. The most serious adverse effects in children are cardiovascular; diastolic hypertension is more common and postural hypotension occurs more rarely than in adults. ECG changes are most likely seen in children receiving high doses. Slowed cardiac conduction (PR interval greater than 0.20 seconds or QRS interval greater than 0.12) may necessitate lowering the dosage. FDA guidelines limit dosages to a maximum of 5 mg/kg a day. The drugs can be toxic in an overdose, and in small children, ingestion of 200 to 400 mg can be fatal. When the dosage is lowered too rapidly, withdrawal effects occur, mainly gastrointestinal symptoms—cramping, nausea, and vomiting—and sometimes apathy and weakness. Antipsychotics The SGAs have generally replaced the conventional antipsychotics as first-line agents in the treatment of all psychotic disorders in children and adolescents. Historically, the best-studied antipsychotics given to pediatric age groups are chlorpromazine (Thorazine) and haloperidol. High-potency and low-potency antipsychotics are thought to differ in their adverse-effect profiles. The phenothiazine derivatives (chlorpromazine and thioridazine) have the most pronounced sedative and atropinic actions, whereas the high-potency antipsychotics are commonly believed to be associated with extrapyramidal reactions, such as parkinsonian symptoms, akathisia, and acute dystonias. The risk of tardive dyskinesia in relation to antipsychotics leads to caution in the use of drugs. Tardive dyskinesia, which is characterized by persistent abnormal involuntary movements of the tongue, face, mouth, or jaw and sometimes the extremities, is a known hazard when giving antipsychotics to patients of all age groups. No known treatment is effective. Because transient choreiform movements of the extremities and trunk are common after abrupt discontinuation of antipsychotics, clinicians must distinguish these symptoms from persistent dyskinesia. REFERENCES Correll CU, Kratocvil CJ, March J. Developments in pediatric psychopharmacology: Focus on stimulants, antidepressants and antipsychotics. J Clin Psychiatry. 2011;72:655–670. Findling RL, Adeyi B, Dirks B, Babcock T, Shecner B, Lasser R, DeLeon A, Ginsberg LD. Parent-reported executive function behaviors and clinician ratings of attention-deficit/hyperactivity disorder symptoms in children treated with lisdexamfetamine. J Child Adolesc Psychopharmacol. 2013;23:28–35. Findling RL, Childress AC, Cutler AJ, Gasior M, Hamdani M, Ferreira-Cornwell MC, Squires L. Efficacy and safety of lisdexamfetamine dimesylate in adolescents with attention-deficit/hyperactivity disorder. J Am Acad Child Adolesc Psychiatry. 2011;50;395–405. Franklin ME, Sapyta J, Freeman JB, Khanna M, Compton S, Almirall D. Moore P, Choate-Summers M, Garcia A, Edson AL, Foa EB, March JS. Cognitive behavior therapy augmentation of pharmacotherapy in pediatric obsessive-compulsive disorder: The Pediatric OCD Treatment Study II (POTS II) randomized controlled trial. JAMA 2011;306:1224–1232. Ginsburg GS, Kendall PC, Sakolsky D, Compton SN, Piacentini J, Albano AM, Walkup JT, Sherrill J, Coffey KA, Rynn MA, Keeton CP, McCracken JT, Bergman L, Iyengar S, Birmaher B, March J. Remission after acute treatment in children and adolescents with anxiety disorders: findings from The CAMS. J Consult Clin Psychol. 2011;79:806–813. Greenhill, LL, Hechtman, L. Attention-deficit disorders. In: Sadock BJ, Sadock VA, & Ruiz P, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Vol. 2. Philadelphia. Lippincott Williams & Wilkins; 2009;3560. Hammerness PG, Perrin JM, Shelley-Abrahamson R, Wilens TE. Cardiovascular risk of stimulant treatment in pediatric attention-deficit/hyperactivity disorder: Update and clinical recommendations. J Am Acad Child Adolesc Psych. 2011;50:978–990. Hughes CW, Emslie GJ, Crimson ML, Posner K, Birmaher B, Ryan N, Jensen P, Curry J, Vitiello B, Lopez M, Shon SP, Piszka SR, Trivedi MH, and The Texas Consensus Conference Panel on Medication Treatment of Childhood Major Depressive Disorder. Texas Children’s Medication Algorithm Project: Update from Texas Consensus Conference Panel on medication treatment of childhood major depressive disorder. J Am Acad Child Adolesc Psychiatry. 2007;46:667–686. Joshi SV. Teamwork: The therapeutic alliance in pediatric pharmacotherapy. Child Adolesc Psychiatr Clin N Am. 2006;12:239. Nilsen TS, Eisemann M, Kvernmo S. Predictors and moderators of outcome in child and adolescent anxiety and depression: A systematic review of psychological treatment studies. Eur Child Adolesc Psychiatry. Feb 2013;22(2):69–87. 49 - 31.18e Psychiatric Treatment of Adolescents 31.18e Psychiatric Treatment of Adolescents Pearson GS. Use of polypharmacy with children and adolescents. J Child Adolesc Psychiatr Nurs. May 2013;26(2):158–159. Rynn M, Puliafico A, Heleniak C, Rikhi P, Ghalib K, Vidair H. Advances in pharmacotherapy for pediatric anxiety disorders. Depress Anxiety. 2011;28:76–87. Vitiello B. Research in child and adolescent psychopharmacology: Recent accomplishments and new challenges. Psychopharmacology. 2007;19:5. Vitiello B. An update on publicly funded multisite trials in pediatric psychopharmacology. Child Adolesc Psychiatr Clin N Am. 2006;15:1. Wagner DK. Pharmacotherapy for major depression in children and adolescents. Prog Neuropsychopharmacol Biol Psychiatry. 2005;29:819. Walkup JT, Albano AM, Piacentini J. Cognitive behavioral therapy, sertraline or a combination in childhood anxiety. N Engl J Med. 2008;359:2753–2766. Zuddas A, Zanni R, Usala T. Second generation antipsychotics (SGAs) for nonpsychotic disorders in children and adolescents: A review of the randomized controlled studies. Eur Neuropsychopharmacol. 2011;21:600–620. 31.18e Psychiatric Treatment of Adolescents Adolescence, biologically beginning with puberty, is a period in which social, intellectual, and sexual development take place alongside specific brain processes that enhance teens’ abilities for increased abstract reasoning and greater sensitivity to social nuances. However, the developmental brain processes are spread over many years, and maturation is subject to individual variation. Inherent in development is continuing change; however, most adolescents adapt to changes gradually, and their path toward greater autonomy and independence is not characterized by perpetual crises and struggle. Milestones achieved by adolescents during their developmental journey to adulthood are typically reached without overwhelming strife or intervention. However, psychiatric treatment is indicated for an adolescent who develops a disturbance of thought, affect, or behavior that disrupts normal functioning. In adolescents, disruption of functioning influences eating, sleeping, and school function, as well as relationships with family and peers. A variety of serious psychiatric disorders, including schizophrenia, bipolar disorder, eating disorders, and substance abuse typically have their onset during adolescence. In addition, the risk for completed suicide drastically increases in adolescence. Although some degree of stress is virtually universal in adolescence, most teenagers who do not develop serious mental disorders cope well with environmental demands. Teenagers with preexisting mental disorders often experience exacerbations during adolescence and may become frustrated, alienated, and demoralized. Clinicians and parents seeking a window into an adolescent’s viewpoint should be sensitive to their self-perceptions. A range of emotional maturity exists in teens of the same chronological age. Issues characteristic of adolescence are related to new evolving identities, the development of sexual activity, and developing plans to meet future life goals. DIAGNOSIS Adolescents can be assessed with a focus on general progress in accomplishing the tasks of individuating and developing a sense of autonomy. For many adolescents in today’s culture, school performance and peer relationship successes are the primary barometers of healthy functioning. Adolescents with normative intellectual function who are deteriorating academically, or teens who become isolated from peers, are typically experiencing significant psychological disturbance, which merits investigation. Questions to be asked regarding adolescents’ stage-specific tasks are the following: What degree of separation from their parents have they achieved? What sort of identities are evolving? How do they perceive their past? Do they perceive themselves as responsible for their own development or only the passive recipients of their parents’ influences? How do they perceive themselves with regard to the future, and how do they anticipate their future responsibilities for themselves and others? Can they think about the varying consequences of different ways of living? How do they express their sexual and affectionate interests? These tasks occupy the lives of all adolescents and normally are performed at different times. Adolescents’ family and peer relationships must be evaluated. Do they perceive and accept both “good and bad” qualities in their parents? Do they feel comfortable with their peers and romantic partners as “separate persons” with needs that may not completely match their own? Respect and acceptance of an adolescent’s subcultural and ethnic background are essential. INTERVIEWS Adolescent patients and their parents should be interviewed separately in a comprehensive psychiatric evaluation. Other family members also may be included, depending on their involvement in the teenager’s life and difficulties. Clinicians often prefer to see the adolescent first, however; in order to develop a rapport with the adolescent and promote being an advocate for the adolescent and avoid the appearance of being the parents’ agent. In psychotherapy with an older adolescent, the therapist and the parents usually have little contact after the initial part of the therapy, because ongoing contact inhibits the adolescent’s desire to open up. Interview Techniques Adolescents may feel pressured by their parents to receive psychiatric treatment and may at first be defensive, or appear guarded. Clinicians must establish themselves as trustworthy and helpful adults to promote a therapeutic alliance. They should encourage adolescents to tell their own stories, without interrupting to check discrepancies; such a tactic may make the therapist seem correcting and disbelieving. Clinicians should ask patients for explanations and theories about what happened. Why did these behaviors or feelings occur? When did things change? What caused the identified problems to begin when they did? Sessions with adolescents generally follow the adult model; the therapist sits across from the patient. In early adolescence, however, board games may help to stimulate conversation in an otherwise quiet, anxious patient. Language is crucial. Even when a teenager and a clinician come from the same socioeconomic group, their language use is seldom the same. Psychiatrists should use their own language, explain any specialized terms or concepts, and ask for an explanation of unfamiliar in-group jargon or slang. Many adolescents do not talk spontaneously about illicit substances and suicidal tendencies but do respond honestly to a therapist’s questions. A therapist may need to ask specifically about each substance and the amount and frequency of its use. The sexual histories and current sexual activities of adolescents are increasingly important pieces of information for adequate evaluation. The nature of adolescents’ sexual behavior often is a vignette of their whole personality structures and ego development, but a long time may elapse in therapy before adolescents begin to talk about their sexual behavior. A 15-year-old adolescent male was referred for a psychiatric evaluation by his high school counselor when he disclosed that he was late to school each day because it took him 3 hours to get ready in the mornings. Even after he finally got to school, he often missed classes and was found in the bathroom. In speaking to his counselor, he further disclosed that he had developed a number of bedtime and morning rituals that took longer and longer to complete because if he did them incorrectly, he had to repeat them. They included checking the locks on the windows and doors, placing objects in the “right” places on his dresser, and repeating a prayer 16 times. He also revealed that when in the bathroom, he had to wash his hands a certain way and dry them “just so,” or he feared something terrible would happen. He had not wanted his parents to know about his difficulties, and he often told them that he had headaches or stomachaches, which made him late. However, he did explain some of his difficulties to his parents during the course of his psychiatric evaluation. His evaluation revealed significant OCD and social phobia. Treatment was initiated, including use of fluoxetine, an SSRI; CBT; and problem-solving family therapy. Over the course of 6 months, his OCD responded well to the combination of medications and CBT, and he was relieved that his family learned ways of helping him both at home and in school. (Adapted from case material courtesy of Eugene V. Beresin, M.D. and Steven C. Schlozman, M.D.) A 14-year-old girl, one of the stars of her high school gymnastic team, began increasing her daily exercise and restricting her diet after her coach indicated that she should lose a few pounds. She became fixated on the size of her thighs and belly, and once she started losing weight, she found that she was not satisfied and wanted to lose a few more pounds. Over the next four months she lost so much weight that her coach and pediatrician no longer allowed her to participate in athletics. Although she was heartbroken about being restricted from gymnastics and planned to eat enough to be able to participate again with her team, she was unable to gain weight, and continued to lose more. She became increasingly terrified of getting fat and secretly exercised any chance she could. She was a perfectionist in academics as well as in gymnastics. She had started her menses 6 months previously, but after she lost a significant amount of weight, her menses stopped. She was seen by a therapist and she and her parents agreed to a meal plan that would result in weight gain, but her family was baffled because she continued to lose more weight. Finally, when it became clear that she was not able to be gain weight under the supervision of her family and her outpatient therapist, she was hospitalized, and the diagnosis of anorexia nervosa, was established. After a 30-day hospitalization with a modest weight gain, she was stepped down to a partial hospital program in which she was supervised for all of her meals, and went home at night. She remained in this program for 8 weeks, and was able to gain 1 to 2 pounds per week. As part of this program, her weight was monitored twice weekly, her vital signs were monitored, and she participated in family therapy, individual psychodynamic psychotherapy, and weekly meetings with a nutritionist. In her psychotherapy, over the course of the next year, she was able to understand that her anorexia had served to prevent her from separating from her parents and kept her close to home and isolated from her peers. She learned that she was slower to mature than many of her peers and felt unable to cope with the social pressures of being a high school student. Over time, she was able to maintain her weight and begin to socialize with friends whom she hadn’t seen for many months. When she was able to maintain an optimal weight she was thrilled to be able to resume her athletics, and she began to develop closer friendships. (Adapted from case material courtesy of Eugene V. Beresin, M.D., and Steven C. Schlozman, M.D.) TREATMENT Psychiatric treatment of an adolescent can occur in numerous venues and modalities. Treatment can take place in individual or group settings, and can include interventions that are pharmacological (when indicated), psychosocial, and from an environmental perspective. The best choices for treatment must take into account the characteristics of the individual adolescent and the family or social milieu. Adolescents’ striving for autonomy may complicate problems of compliance with therapy and may result in the need for stabilization in inpatient settings, whereas this level of care might not be necessary at a different stage of life. The following discussion is less a set of guidelines than a brief summary of what each treatment modality can or should offer. Individual Psychotherapy Individual psychosocial modalities with an evidence base for efficacy with adolescents include cognitive-behavioral treatments for diagnoses of anxiety disorders, mood disorders, and OCD. Interpersonal therapy is a technique that has been used to treat mood disorders in adolescents. Few adolescent patients are trusting or open without considerable time and testing of therapists, and it is helpful to anticipate the testing period by letting patients know that it is expected and is natural and healthy. Pointing out the likelihood of therapeutic problems—for instance, impatience and disappointment with the psychiatrist, with the therapy, with the time required, and with the often intangible results—may help keep problems under control. Therapeutic goals should be stated in terms that adolescents understand and value. Although they may not see the point in exercising self-control, enduring dysphoric emotions, or forgoing impulsive gratification, they may value feeling more confident than in the past and gaining more control over their lives and the events that affect them. Typical adolescent patients need a relationship with a therapist they can perceive as a real person, whom they feel respected by and they can trust. The therapist may seem like another parent in some respects, since adolescents still need appropriate guidance, especially in situations of high-risk behaviors. Thus, a professional who is impersonal and anonymous is a less useful model than one who can accept and respond rationally to an angry challenge or confrontation without fear or false conciliation—one that can impose limits and controls when adolescents cannot, can admit mistakes and ignorance, and can openly express the gamut of human emotions. Combined Pharmacotherapy and Psychotherapy Current evidence suggests that for many psychiatric disorders, optimal treatment includes a combination of psychosocial and psychopharmacological interventions. Randomized clinical trials have provided evidence of the superiority of CBT in combination with SSRIs in the treatment of mood disorders, OCD, and anxiety disorders, to name a few. ADHD is often comorbid with additional disorders, thus, although the Multimodal Treatment Study of Children with ADHD (MTA) found that psychosocial interventions did not add to the efficacy of stimulant treatments for the core symptoms of ADHD, it is important to consider that other concurrent disorders that affect overall functioning often require psychosocial treatments. Advances in drug development have widened the choice of medications to treat mood disorders (e.g., SSRIs) and schizophrenia (e.g., SGAs, including risperidone [Risperdal], olanzapine [Zyprexa], and clozapine [Clozaril]). Although these medications have been used to treat psychiatric disorders in adolescents, more research is required to determine their efficacy and safety profiles for treatment of adolescent psychopathology. A 17-year-old girl complained of recurrent episodes of rapid heartbeat, sweating, trembling, and a fear that she was “going crazy.” Her first episode had occurred in her high school cafeteria during a “college night” event, when multiple college representatives were displaying their college’s information packets. After running out of the cafeteria, she stood outside of her school and the episode gradually dissipated over a period of about 15 minutes. Although she was a little nervous about going back to school the next day, she did not have another episode. She had almost forgotten about the episode, when it happened again, and even more intensely, when she was shopping at the mall and talking about college applications with her friends. After this episode, she became fearful of going out alone to the shopping mall. She was at the beginning of her senior year in high school, considering her options for college and was planning to take her SAT for the last time. Her parents wanted her to maintain the family tradition and pressured her to try for the same college from which her mother graduated. She was not opposed to applying to her mother’s alma mater, but was very angry and upset about her parents’ pressure on her to make a commitment to this school as her first choice. She became irritable and tearful, and she was experiencing several panic attacks per week, all of which indicated that she needed to get some help. She was evaluated by a psychiatrist and started on Lexapro (escitalopram) to alleviate the panic disorder symptoms, as well as weekly psychotherapy. The psychotherapy focused on the patient’s conflicts with her parents, highlighting her chronic concern that she could not meet parental expectations and fears of her independence. Medication appeared to reduce symptoms of tachycardia, tremulousness, decreased her irritability, and diminished her preoccupation with lack of competence. Psychotherapy and medication were both maintained for the next 8 months during her last year in high school. (Adapted from case material courtesy of Cynthia R. Pfeffer, M.D.) Group Psychotherapy In many ways, group psychotherapy is a natural setting for adolescents. Most teenagers are more comfortable with peers than with adults. A group diminishes the sense of unequal power between the adult therapist and the adolescent patient. Participation varies, depending on an adolescent’s readiness. Not all interpretations and confrontations should come from the parent-figure therapist; group members often are adept at noticing symptomatic behavior in each other, and adolescents may find it easier to hear and consider critical or challenging comments from their peers. Group psychotherapy usually addresses interpersonal and current life issues. Some adolescents, however, are too fragile for group psychotherapy or have symptoms or social traits that are too likely to elicit peer group ridicule; they need individual therapy to attain sufficient ego strength to struggle with peer relationships. Conversely, other adolescents must resolve interpersonal issues in a group before they can tackle intrapsychic issues in the intensity of one-on-one therapy. Family Therapy Family therapy is the primary modality when adolescents’ difficulties mainly reflect a dysfunctional family (e.g., teenagers with school refusal, runaways). The same may be true when developmental issues, such as adolescent sexuality and striving for autonomy, trigger family conflicts or when family pathology is severe, as in cases of incest and child abuse. In these instances, adolescents usually need individual therapy as well, but family therapy is mandatory if an adolescent is to remain in the home or return to it. Serious character pathology, such as that underlying antisocial and borderline personality disorders, often develops from highly pathogenic early parenting. Family therapy is strongly indicated whenever possible for such disorders, but most authorities consider it adjunctive to intensive individual psychotherapy when individual psychopathology has become so internalized that it persists regardless of the current family status. Inpatient Treatment Residential treatment schools often are preferable for long-term therapy, but hospitals are more suitable for emergencies, although some adolescent inpatient hospital units also provide educational, recreational, and occupational facilities for long-term patients. Adolescents whose families are too disturbed or incompetent, who are dangerous to themselves or others, who are out of control in ways that preclude further healthy development, or who are seriously disorganized require, at least temporarily, the external controls of a structured environment. Long-term inpatient therapy is the treatment of choice for severe disorders that are considered wholly or largely psychogenic in origin, such as major ego deficits that are caused by early massive deprivation and that respond poorly or not at all to medication. Severe borderline personality disorder, for example, regardless of the behavioral symptoms, requires a full-time corrective environment in which regression is possible and safe and in which ego development can take place. Psychotic disorders in adolescence often require hospitalization; however, psychotic adolescents often respond to appropriate medication well enough that therapy is feasible in an outpatient setting, except during exacerbations. Adolescent patients with schizophrenia who exhibit a longterm deteriorating course may require hospitalization periodically. Day Hospitals In day hospitals, which have become increasingly popular, adolescents spend the day in class, individual and group psychotherapy, and other programs, but they go home in the evenings. Day hospitals are less expensive than full hospitalization and usually are preferred by patients. CLINICAL PROBLEMS Atypical Puberty Pubertal changes that occur 2.5 years earlier or later than the average age are within the normal range. Body image is so important to adolescents, however, that extremes of the norm may be distressing to some, either because markedly early maturation subjects them to social and sexual pressures for which they are unready or because late maturation makes them feel inferior and excludes them from some peer activities. Medical reassurance, even if based on examination and testing to rule out pathophysiology, may not suffice. An adolescent’s distress may show as sexual or delinquent acting out, withdrawal, or problems at school that are sufficiently serious to warrant therapeutic intervention. Therapy also may be prompted by similar disturbances in some adolescents who fail to achieve peer-valued stereotypes of physical development despite normal pubertal physiology. Substance-Related Disorders Some experimentation with psychoactive substances is almost ubiquitous among adolescents, especially if this category of behavior includes alcohol use. Most adolescents, however, do not become abusers, particularly of prescription drugs and illegal substances. Any regular substance abuse represents disturbance. Substance abuse sometimes is self-medication against depression or schizophrenic deterioration and sometimes it signals a character disorder in teenagers whose ego deficits render them unequal to the stresses of puberty and the tasks of adolescence. Some substances, including cocaine, have a physiologically reinforcing action that acts independent of preexisting psychopathology. When substance abuse covers an underlying illness or is a maladaptive response to current stresses or disturbed family dynamics, treatment of the underlying cause may diminish the substance use; in most cases of significant abuse, however, the drug-taking behavior typically requires intervention. Substance abuse treatments typically include a 12-step program with behavioral monitoring to accomplish sobriety as well as the ability to verbalize regarding the motivations for substance use. These philosophies are adapted to inpatient, intensive outpatient, and once-a-week outpatient treatment. Suicide Suicide is the third leading cause of death among adolescents. Many hospital admissions of adolescents result from suicidal ideation or behavior. Among adolescents who are not psychotic, the highest suicidal risks occur in those who have a history of parental suicide, who are unable to form stable attachments, who display impulsive behavior, and who abuse alcohol or other substances. Many adolescents who complete suicide have backgrounds that include long-standing family conflict and social problems since early childhood and the escalation of subjective distress under the pressure of a sudden perceived conflict or loss. Early childhood loss of parents also can increase the risk of depression in adolescence. Adolescents who are susceptible to rapid and extreme mood swings and a history of impulsive behavior are at greater risk of responding to despair with impulsive suicide attempts. Abuse of alcohol and other substances are known added risks for suicidal behavior in adolescents with suicidal ideations. The developmentally predictable “omnipotent” attitudes of adolescents may cloud the immediate sense of permanence of death and result in impulsive self-destructive behavior in adolescents. During a psychiatric evaluation of an adolescent with suicidal thoughts, plans and past attempts must be discussed directly when the concern arises and information is not volunteered. Recurring suicidal thoughts should be taken seriously, and a clinician must evaluate the imminent clinical danger requiring inpatient hospitalization versus an adolescent’s ability to engage in an agreement or contract mandating that the adolescent will seek help before engaging in self-destructive behavior. Adolescents typically are honest in their refusal of such agreements, and, in such cases, hospitalization is indicated. Hospitalization of a suicidal adolescent by a clinician is an act of serious, protective concern. REFERENCES Beresin EV, Schlozman SC. The treatment of adolescents. In: Sadock BJ, Sadock VA, Ruiz, P, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Vol. 2. Philadelphia: Lippincott Williams & Wilkins; 2009:3777. Biegel GM, Brown KW, Shapiro SL, Schubert CM. Mindfulness-based stress reduction for the treatment of adolescent psychiatric outpatients: A randomized clinical trial. J Consult Clin Psychol. 2009;5:855–866. Connor DF, McLaughlin TJ, Jeffers-Terry M, O’Brien WH, Stille CJ, Young LM, Antonelli RC. Targeted child psychiatric services: A new model of pediatric primary clinician–child psychiatry collaborative care. Clin Pediatr (Phila). 2006;45:423. Laugeson EA, Frankel F, Gantman A, Dillon AR, Mogil C. Evidence-based social skills training for adolescents with autism spectrum disorders: The UCLA PEERS Program. J Autism Dev Disord. 2012;42:1025–1036. Leckman JF. The risks and benefits of antidepressants to treat pediatric-onset depression and anxiety disorders: A developmental perspective. Psychother Psychosom. 2013;82(3):129–131. Lundh A, Forsman M, Serlachius E, Lichtenstein P, Landen M. Outcomes of child psychiatric treatment. Acta Psychiatr Scand. Jul 2013;128(1):34–44. Mathyssek CM, Olino TM, Hartman CA, Ormel J, Verhulst FC, Van Oort FV. Does the Revised Child Anxiety and Depression Scale (RCADS) measure anxiety symptoms consistently across adolescence? The TRAILS study. Int J Methods Psychiatric Res. Mar 2013;22(1):27–35. Mufson L, Dorta KP, Wickramaratne P, Nomura Y, Olfson M, Weissman MM. A randomized effectiveness trial of interpersonal psychotherapy for depressed adolescents. Arch Gen Psychiatry. 2004;61(6):577. Nevels RM, Dehon EE, Gontkovsky ST, Alexander K. Psychopharmacology of aggression in children and adolescents with primary neuropsychiatric disorders: A review of current and potentially promising treatment options. Exp Clin Psychopharmacol. 2010;8:184–201. Olfson M, Marcus SC, Shaffer D. Antidepressant drug therapy and suicide in severely depressed children and adolescents: A case-control study. Arch Gen Psychiatry. 2006;63:865. Richardson T, Stallard P, Velleman S. Computerised cognitive behavioural therapy for the prevention and treatment of depression and anxiety in children and adolescents: A systematic review. Clin Child Fam Psychol Rev. 2010;13:275–290. Romano E, Zoccolillo M, Paquette D. Histories of child maltreatment and psychiatric disorder in pregnant adolescents. J Am Acad Child Adolesc Psychiatry. 2006;45:329. Seidman LJ. Neuropsychological functioning in people with ADHD across the lifespan. Clin Psychol Rev. 2006;26:466. Stallard P, Sayal K, Phillips R, Taylor JA, Spears M, Araya R. Classroom based cognitive behavioral therapy in reducing symptoms of depression in high risk adolescents: pragmatic cluster randomised controlled trial. BMJ. 2012;345:e6058. 50 - 31.19 Child Psychiatry Special Areas of Inter 31.19 Child Psychiatry: Special Areas of Interest 51 - 31.19a Forensic Issues in Child Psychiatry 31.19a Forensic Issues in Child Psychiatry 31.19 Child Psychiatry: Special Areas of Interest 31.19a Forensic Issues in Child Psychiatry Forensic evaluations of youth span a broad spectrum of situations and settings, including child custody during a parental divorce, trauma and abuse situations, and juvenile offender evaluations pertaining to juvenile and criminal court cases. Child and adolescent psychiatrists are increasingly being sought out by patients and attorneys for evaluations and expert opinions related to child sexual and physical abuse, to criminal behaviors perpetrated by minors, and to evaluate the relations between traumatic life events and the emergence of psychiatric symptoms in children and adolescents. As more youth enter the juvenile justice system, an increasing need exists for forensic psychiatrists with expertise in evaluation and treatment for detainees and committed youths. The specific tasks and role of a child and adolescent psychiatric forensic evaluator are distinctly different from a child and adolescent psychiatrist doing a clinical evaluation and clinical treatment intervention. In clinical settings, child mental health professionals provide psychotherapy, medication evaluations, and advocacy for youth with psychiatric diagnoses. As a forensic child psychiatric evaluator, however, the main task is to be an expert, to report objective psychiatric findings related to the questions asked. Two essential characteristics of a forensic evaluator, in contrast to a clinician are (1) the relationship between the evaluator and the patient is not therapeutic, rather, it is information seeking, and (2) there are clear limits of confidentiality in this situation, that is, the information disclosed during a forensic evaluation may be brought to court, or to an attorney, or to whomever initiated the evaluation. Society’s view of children and their rights has evolved dramatically. In 1980, the American Academy of Child and Adolescent Psychiatry (AACAP) published a code of ethics that was developed to publicly endorse the ethical standards of this discipline. The code is based on the assumption that children are vulnerable and unable to take adequate care of themselves; as they mature, however, their capacity to make judgments of, and choices about, their well-being develops as well. The code has several caveats: From the standpoint of child and adolescent psychiatrists, issues of consent, confidentiality, and professional responsibility must be seen in the context of overlapping and potentially conflicting rights of children, parents, and society. Confidentiality, or intensive trust, refers to the relationship between two persons with respect to the “entrustment of secrets.” Until the 1970s, little attention was paid to issues of confidentiality pertaining to minors. In 1980, among the items in the AACAP Code of Ethics, six principles were related to confidentiality. Breaches and limits of confidentiality can be obtained in cases of child abuse or maltreatment or for purposes of appropriate education. Although unnecessary with a child or adolescent, consent for disclosure should be obtained when possible. In 1979, the American Psychiatric Association (APA) stated that a child 12 years of age could give consent for disclosure of confidential information and, with the exception of safety issues, a minor’s consent is required for disclosure of information to others, including the child’s parents. According to the AACAP Code of Ethics, the consent of a minor is not required for disclosure of confidential information. Specific ages for consent are not addressed in the code. Child and adolescent psychiatrists often face the dilemma of weighing the potential benefits and possible harm in sharing information obtained confidentially from a child with the child’s parents. Although the smoothest transition occurs when the child and the physician agree that certain information can be shared, in many situations that border on “dangerousness to the child or others,” the child or adolescent does not agree to share the information with a parent or another responsible adult. Among adolescents, these secrets that are sometimes shared with a psychiatrist may involve drug or alcohol use, unsafe sex practices, or a thrillseeking act that places the adolescent in danger. A psychiatrist may choose to work with the child or adolescent toward agreeing to share confidential information when it is determined by the treating psychiatrist that the probable outcome would be beneficial. The initial treatment contract, however, limits confidentiality to situations of “danger” to the child or others. CHILD CUSTODY Child custody evaluations by child and adolescent psychiatrists may be initiated by divorcing parents who cannot come to an agreement regarding custody of their children, or can be requested by an attorney. Attorneys are most likely to seek child custody evaluations when allegations are made of parental incompetence, or issues of alleged physical or sexual abuse arise. Comprehensive custody evaluations by mental health professionals may play a significant role in successful negotiations of custody by parents without the necessity of proceeding to a trial. The evolution of child custody decision-making has been influenced by increasing awareness and recognition of the rights of children and women, as well as by a broadening perspective on the developmental and psychological needs of the children involved. Historically, children were considered to be their fathers’ property. At the beginning of the 20th century, the “tender years” doctrine became the standard for determining child custody. According to this doctrine, the relationship between mother and infant, later generalized to mother and child, is responsible for the optimal emotional development of the child; the doctrine thus supported custody decisions in the mother’s favor in most cases. With this doctrine as its guide, psychological issues in developing children became an acceptable dimension to consider in the determination of custody. In controversial and unclear cases, psychological expert testimony began to be accepted as a valuable part of child custody decision-making. The “best interest of the child” standard replaced the “tender years” doctrine and expanded considerations of the optimal parent to include assessing issues of emotional climate, safety, and educational and social opportunities for the children. The “best interest of the child” grew from the movement to support legislation about the rights of children in the areas of compulsory education, child labor laws, and child abuse and neglect protection laws. Therefore, although “best interest” standards have broadened the dimensions considered in evaluating which parent is best able to serve the best interest of the child, how to measure these qualities in a parent remains vague. In view of the lack of clarity regarding what specific parameters in a parent best correspond to the interest of the child, child and adolescent psychiatrists have increasingly been asked to help make decisions by defining relevant psychological conditions in parents and in the relationships between parents and children. Psychiatric evaluators may be asked to give an opinion about child custody at various points during the separation and divorce process. Sometimes, a psychiatric evaluation is requested by the parents before any legal action occurs. When the parents and an evaluator can agree on custody decisions before the legal process, a court is likely to go along with these decisions rather than launch an additional investigation. A psychiatric evaluation may be ordered by the court or by the attorneys representing the feuding parents. In such cases, an evaluator is faced with two disgruntled parents, who often are consumed by their mutual conflict to the point that neither is willing to compromise, even in the child’s interest. The advantage in such cases, however, is that evaluators represent the court and can act as advocate for the child without the same pressures that an evaluator hired by only one parent faces. A psychiatric evaluation also may be initiated by a guardian ad litem, an attorney who is appointed by the court to represent the child. Psychiatric evaluators also may be requested to give an opinion about custody during a mediation process. Mediation is a legal process that usually involves one attorney and one evaluator. Because mediation can occur outside the judicial system, some families may prefer it to going through a trial. In addition to custody, psychiatric evaluators often are asked to give opinions about visitation. In undertaking a custody evaluation, an evaluator is expected to determine the best interests of the child while keeping in mind the standard elements that the court considers. These considerations include the wishes of the parents and the child; relationships with significant others in the child’s life; the child’s adjustment to the current home, school, and community; the psychiatric and physical health of all parties; and the level of conflict and potential danger to the child under the care of either parent. A psychiatric evaluator must maintain his or her role as an advocate for the best interest of the child and does not consider the fairest outcome for parents. The psychiatric evaluator conducts a series of interviews, often including at least one separate interview with each parent and the child alone and one interview with the child and both parents. The evaluator may obtain a written waiver of confidentiality from all parties because he or she may have made disclosures to opposing attorneys and in court before the judge. The evaluator uses direct questioning as well as observations of the relationships between the child and each parent. The age and developmental needs of the child are considered in making a judgment regarding which parent may better serve the child’s interests. As part of the psychiatric assessment of the child custody evaluation, the evaluator determines the need for psychiatric treatment of any of the parties involved. The child custody evaluation generally is provided in a written report. This document is not confidential and can be used in court. The report contains a description of the relationship between the child and parents, the capabilities of the parents, and finally, the custody recommendations. In view of data supporting the importance of continuing a relationship with both parents in most cases, it is recommended that joint custody be considered before other options. When sufficient cooperation exists to negotiate for joint custody, the best interests of the child often are served. Joint custody may not be the best option for a child when the relationship of the child with either parent is jeopardized and undermined by the other. The next most frequent choice when joint custody is not advisable is full custody by one parent with visitation rights for the other parent. The parent awarded full custody should be able to support the visitations and relationship with the noncustodial parent. In custody disputes involving a biological parent and a nonbiological parent, the biological parent generally has the right to custody unless he or she is shown to be unable to provide for the child. After the custody evaluation has been submitted in writing, the results must be communicated to the parents, the child, and possibly their respective attorneys. The evaluator may be called on to testify in court, and the parties can use the custody evaluation to mediate other areas of their dispute. Many complications can occur in an ongoing bitter dispute between divorcing or divorced parents. Both true and false allegations of psychiatric illness, drug or alcohol abuse, or sexual or physical abuse are not uncommon during custody battles. The evaluator must be prepared to verify any allegations and to carefully discuss their effects on custody and visitation. Evidence suggests that markedly elevated numbers of unfounded allegations of child sexual abuse occur during the course of custody disputes. Tremain, age 9 years, has been in a therapeutic foster home for 2 years, having been removed from his home along with his younger sister, due to profound neglect, as well as physical abuse. Although he is receiving cognitive-behavioral therapy, medication, and a social skills group, he remains volatile, and typically becomes more aggressive and regressed after weekly supervised visits with his mother. Tremain’s sister has been reunited with their mother, and his guardian ad litem requests that a child psychiatrist perform a forensic evaluation to determine whether visits should continue. She reviews extensive records, evaluates each parent, obtains history from them and the foster parent, and then observes a visit. Tremain’s little sister totally dominates the visit, and her mother is at a loss to control her aggressive and hyperactive behavior. Tremain is passive and clingy with his mother. According to the social work supervisor, this is a fairly typical visit. When the child and adolescent psychiatrist meets individually with Tremain, he expresses concern that his sister is probably being abused at home, and he likes to check on her during these visits. Tremain wants to go home, but he says that his mother has too many problems to take care of him. Tremain has developed a positive relationship with his foster father and, in contrast, has little to say about his biological mother. The child and adolescent psychiatrist recommends a psychiatric evaluation of the sister, but Tremain’s mother does not follow through. The child and adolescent psychiatrist recommends cutting visits back to monthly, but Tremain’s anxiety and aggressive behavior persist around these limited visits. It also becomes apparent that Tremain’s mother is not up to the demands of caring for two special-needs children, as she is having difficulty containing her little daughter. The child and adolescent psychiatrist recommends delaying efforts at parental reunification however, maintaining contact between Tremain, his mother, and his sister. (Adapted from case material from Diane H. Schetky, M.D.) JUVENILE OFFENDERS According to the AACAP Practice Parameter for Child and Adolescent Forensic Evaluations, at least 2.7 million youth younger than 18 years are arrested each year in the U.S., and more than 1 million youth will have a formal interaction with the juvenile justice system. Historically, a separate juvenile court system in the United States occurred by statute in the state of Illinois in the late 1800s. Its mandate was to rehabilitate rather than to punish. Despite the protective intentions of the legal system, children and adolescents involved in the juvenile justice system are at high risk for multiple psychiatric disorders and suicidal thoughts and behavior. The omission of various constitutional safeguards, such as the rights to counsel, confrontation, and crossexamination of an accuser, eventually led to criticism and disillusionment with this system. Juvenile offenders of small and significant crimes often were sent to state-run residential programs that were criticized for being overcrowded, neglectful, and frankly abusive. Despite the strong sentiment to increase due process protection for juveniles rather than pretrial, trial, and sentencing, the juvenile court system includes intake, adjudication, and disposition. The intake is a determination of whether probable cause exists that the youth committed a crime. A youth who confesses may be diverted from the court system altogether at this time, and appropriate plans for rehabilitation can be made in a community setting. For more serious crimes or when juveniles deny perpetrating a crime, the process continues. Juveniles must be represented by counsel, and an attorney is provided if the family cannot afford to provide its own. Unlike adult court, in juvenile court, guilt or innocence is determined by a judge, not a jury. The case is argued by a prosecuting attorney and a defense attorney, and the judge is bound by the same standards as in adult court; that is, a judgment of delinquency requires proof beyond a reasonable doubt. When the charge is substantiated and the judgment is for delinquency, the juvenile is an “adjudicated delinquent.” Disposition must next be determined. Dispositions include a wide range of options, from placement in youth correctional facilities, to residential treatment settings, to psychiatric hospitalizations for further evaluation. Delinquent acts refer to ordinary crimes committed by juveniles; status offenses refer to behaviors that would not be criminal if perpetrated by adults, such as truancy, running away, or drinking alcohol. Sometimes, youths who are believed to have committed a serious crime are turned over (receive a waiver) to adult criminal court. DEVELOPMENTAL IMMATURITY VERSUS JUVENILE COMPETENCE TO STAND TRIAL A growing body of research is elucidating the significance of “developmental immaturity” on the capacity of children and young adolescents’ competency to stand trial. Starting in the 1960s, the Supreme Court mandated a series of due process rights in juvenile court proceedings including the rights to notice of charges, an adversarial hearing with representation by counsel, the ability to cross-examine witnesses, and a trial transcript. Furthermore, juveniles have the right to a hearing prior to being transferred to adult court, and use of the standard of proof beyond a reasonable doubt to sustain a delinquency petition; however, there is no mention of the right to be competent to stand trial in delinquency proceedings. In the 1960 landmark case Dusky v. United States, the Supreme Court established a minimum national standard for competency in criminal proceedings for adults. This standard mandates that in order for a defendant to be competent to stand trial, he or she must possess “sufficient present ability to consult with his lawyer with a reasonable degree of rational understanding and a rational as well as factual understanding of the proceedings against him.” Thus, there is no legal requirement for juveniles to be competent to participate in delinquency proceedings; however, many states have adopted their own competency standards for their juvenile courts. This is critical insofar as research suggests that developmental level has a definite impact on a child or early adolescent’s understanding of legal concepts, and long-term implications of legal decisions, and thereby influences their competency to stand trial. The following two U.S. Supreme Court rulings have provided legal stipulations pertaining to the limitations of developmental immaturity and legal culpability. (1) In the case, Roper v. Simmons (2005), a successful argument was made that, among other reasons, but including a juvenile’s normative immaturity, including impulsive decision making, susceptibility to peer pressure, and transitory behavior patterns, youth younger than 18 years of age should be excluded from the death penalty. (2) In 2010, in the case Graham v. Florida, using the developmental information that fueled the exclusion of minors from the death penalty, the Court ruled that a life sentence without parole for a juvenile offender, (with the exclusion of homicide cases), constituted cruel and unusual punishment. In one study using vignettes to elicit responses from children and adolescents pertaining to competent participation in legal proceedings, findings included that children 11 years to 13 years were less able to recognize risks and long-term consequences associated with their decisions and were more likely than adults to accept plea agreements; whereas youths up to 15 years also demonstrated greater compliance with authority figures in their decision making, compared to adults. Researchers in this field have concluded that due to developmental status alone, young juveniles are at greater risk to make poor decisions on their own behalf in the context of working with their own attorneys. There are many factors that may influence a juvenile’s competency, and several are essential in a competency evaluation of a juvenile, including: (1) age, with special consideration in any child of 12 years or younger; (2) developmental stage with respect to judgment, reasoning, responsibility, risk perception, suggestibility, temperance (seeking advice rather than acting without the facts), and future orientation; (3) assessment of mental disorder and intellectual level. MENTAL HEALTH NEEDS OF YOUTH IN THE JUVENILE JUSTICE SYSTEM Youth in the juvenile justice system are at extremely high risk for psychiatric disturbance, and unmet mental health needs have reached such high proportions that they are of public health concern. Adolescents in juvenile justice residential facilities not only have higher rates of psychiatric disorders, including depression, substance use, and suicidal behavior, but they are also significantly more likely to have been victims of physical and sexual abuse, educational failure, and family conflict. A survey of 991 youth at an initial juvenile justice intake revealed high levels of suicidal ideation with recent attempts more common in females, and youth with major depression or substance use disorders, and those who were violent offenders. Few studies, however, have documented the needs of juveniles in residential facilities and the medical and psychiatric care available. A recent study collected data from the U.S. Department of Justice censuses of all public and private juvenile justice facilities in the United States: The Juvenile Residential Facilities Census (JRFC) and the Census of Juveniles in Residential Placement (CJRP) investigated data on death rates of youth under the age of 21 years who had been charged with, or adjudicated for, an offense and are housed in that facility because of the offense. In the 2-year period, a total of 62 deaths of youth occurred. The leading cause of death was from suicide (20 cases), followed by accidents (17 cases), illness (14 cases), and homicides by nonresidents (6 cases). No deaths resulted from acquired immunodeficiency syndrome (AIDS), homicide by another resident, or an injury that occurred before placement. The risk for death of youth in juvenile justice facilities was found to be 8 percent higher than the death rate for the general population of adolescents aged 15 to 19 years. Above all, the risk for suicide is clearly increased in the juvenile justice facility compared with the general population, indicating a significant need for increased mental health evaluation and treatment in this population. FORENSIC ASPECTS OF SCHOOL BULLYING The forensic aspects of school bullying have increased over the last two decades, particularly in the wake of the serious incidents of school violence that took place in the mid-1990s such as the Columbine school shootings. The responsibilities of schools to protect students and safeguard against injury have extended from a duty to care, to a duty to protect. Bullying is typically observed in four different realms: physical, relational, verbal, and cyberbullying. Forensic evaluations often begin with a referral from an attorney, the court, or a family. After obtaining a comprehensive history of the youths involved in a reported bullying incident, the evaluator is tasked with determining if the alleged bullying has had a negative impact on the mental health and well-being of the victim. In some cases, investigators have reported findings of increased suicidality in the bully as well as the victim. One study reported that victims of cyberbullying attempt suicide twice as often as other youth. THE RELATIONSHIPS BETWEEN TRAUMA, ABUSE, AND VIOLENT DELINQUENCY Child and adolescent psychiatrists are frequently sought out to evaluate children or adolescents who have been exposed to a traumatic or adverse life event and are exhibiting a variety of violent and delinquent behaviors. The child and adolescent psychiatrist may be asked to determine whether a child or adolescent is experiencing posttraumatic stress disorder or whether a given set of symptoms is likely to have been caused by exposure to the adverse life event. It is clear from surveys of delinquent adolescents that there is a relationship between posttraumatic stress disorder, previous histories of trauma and abuse, and aggressive behavior. Some researchers argue that evidence supports a trauma-related psychopathology in youth that evolves into aggressive behavior, and often into delinquency. It appears that brain circuits that monitor “threat response,” that is, circuits that run from the medial nucleus of the amygdala to the medial hypothalamus and to the periaqueductal gray matter, are overly reactive in reactive/affective/defensive/impulsive aggression (RADI, also referred to as “hot” aggression), as well as in planned or predatory aggression (PIP, also referred to as “cold” aggression). Particularly in RADI, structures may have become dysregulated by traumatic emotional activation, resulting in a lack of subtle differentiation between emotions such as sadness, anger, and fear. The result is that any stress is perceived as a threat, and activates the “defense” system, leading to the flight or fight decisions. The final response seems to be “fight,” a response triggered during abusive or lifethreatening situations in which escape seems impossible. In another study, a cognitive mechanism for the link between abusive parenting and violent delinquency is offered. In this retrospective study of 112 adolescents (male 90; female 22), ages 12 to 19 years who were incarcerated in a juvenile detention facility pending criminal charges, participants completed questionnaires pertaining to exposure to abusive and nonabusive discipline, expressed and converted shame, and violent delinquency. The authors defined shame as a state in which negative attributions of the self and self-blame are made as a result of perceived failure in meeting their own expected standards. Higher levels of shame have been found among youth exposed to trauma. Converted shame is an expression of externalizing blame to others so that hostility is directed away from oneself, and decreases one’s own sense of responsibility for something negative, such as abuse. Converted shame can serve as a self-protective attribution. The findings of this study led to subjects’ responses, which fell into four groups: (1) Low shame, and low blaming of others; (2) Converters: low shame and high blaming of others, (3) Expressers: high shame, and low blaming of others, (4) High shame and high blaming of others. Subjects who were in group 2 (low shame and high blaming of others) had significantly more exposure to abusive parenting, and exhibited significantly more violent delinquent behaviors than those in group 3 (high shame, low blaming of others). Thus, although converting shame is “meant” to be self-protective, and a potentially adaptive response to consistent abusive parenting, those adolescents who strongly blamed others appeared to develop more violent delinquency. The authors considered the violent delinquency a pathological response to trauma. Dr. Sullivan is called by a defense attorney to review discovery material in a case that alleges permanent harm and suffering in 6-year-old Travis, who is alleged to have been sexually abused at age 3 in his day care center. Dr. Lane, the forensic expert for the plaintiff, has evaluated the child and performed psychological testing of him and concluded that the boy’s conduct problems are all related to the alleged abuse, which the child has difficulty recalling. His early history on the boy is cursory, however, and he has little information about the mother, who is a single parent, and he did not review medical records. In her thorough review of discovery material, Dr. Sullivan learns that Travis has witnessed extensive domestic violence and his mother’s rape, shown signs of hyperactivity since age 2, and has exhibited much anxiety related to his mother’s safety and several separations from her at times when she was unable to care for him owing to depression. Travis also has had delayed language development. Dr. Lane, at the time of his deposition, was asked why he had not asked about these matters. He said he considered the mother’s personal life a private matter and did not see its relevance to the litigation. Dr. Sullivan, when deposed, points out that many other factors beside the alleged abuse might account for Tony’s behavioral problems. (Adapted from case material from Diane H. Schetky, M.D.) REFERENCES American Academy of Child and Adolescent Psychiatry: Practice Parameter for Child and Adolescent Forensic Evaluations. J Am Acad Child Adolesc Psychiatry. 2011;50:1299–1312. Bernet W, Corwin D. An evidence-based approach for estimating present and future damages from child sexual abuse. J Am Acad Psychiatry Law. 2006;34:224. Deitch M, Barstow A, Lukens L, Reyna R. From time out to hard time: Young children in the adult criminal justice system. Austin, TX: The University of Texas at Austin, LBJ School of Public Affairs, 2009. Dusky v. United States, 362 U.S. 402 (1960). Freeman BW, Thompson C, Jaques C. Forensic aspects and assessment of school bullying. Psychiatr Clin N Am. 2012;35:877–900. Gold J, Sullivan MW, Lewis M. The relation between abuse and violent delinquency: The conversion of shame to blame in juvenile offenders. Child Abuse Neglect. 2011;459–467. Graham v. Florida, 2010 U.S. LEXIS 3881. Hinduja S, Patchin JW. Bullying, cyberbullying, and suicide. Arch Suicide Res. 2010;14:206–221. Klomek A, Sourander A, Gould M. Bullying and suicide: Detection and intervention. Psychiatric Times. 2011;28:2. O’Donnell PC, Gross B. Developmental incompetence to stand trial in Juvenile Courts. J Forensic Sci. 2012;57:989–996. Roper V. Simmons, 543 U.S. 551 (2005). Schetky DH. Forensic child and adolescent psychiatry. In: Sadock BJ, Sadock VA, Ruiz P, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Vol. 2. Philadelphia: Lippincott Williams & Wilkins; 2009:3834. Soulier M. Juvenile offenders. Psychiatr Clin N Am. 2012;35:837–854. Steiner H, Silverman M, Karnik NS, Huemer J, Plattner B, Clark CE. Psychopathology, trauma and delinquency: Subtypes of aggression and their relevance for understanding young offenders. Child Adolesc Psychiatry Ment Health. 2011;5:21–32. Ttofi MM, Farrington DP, Losel F. The predictive efficiency of school bullying versus later offending: A systematic/metaanalytic review of longitudinal studies. Crim Behav Ment Health. 2011;21:80–89. Waller EM, Daniel AE. Purpose and utility of child custody evaluations: The attorney’s perspective. J Am Acad Psychiatry Law. 2005;33:199. Wingrove TA. Is immaturity a legitimate source of incompetence to avoid standing trial in a juvenile court? Neb Law Rev. 2007;86:488–514. Zablotsky B, Bradshaw CP, Anderson C, Law PA. The association between bullying and the psychological functioning of 52 - 31.19b Adoption and Foster Care 31.19b Adoption and Foster Care children with autism spectrum disorders. J Dev Behav Pediatr. 2013;34(1):1–8. 31.19b Adoption and Foster Care According to the U.S. Department of Health and Human Services, 408,425 children and adolescents were in foster care in the United States in 2010. Most children entering foster care have experienced multiple traumatic events including neglect, or abuse, which are typically the precipitant for their removal from their biological parents. One study estimated that 26 percent of children in the United States will experience a traumatic event by the age of 4 years. Over the last decade, specifically between the years of 2000 to 2010, the number of evaluations for suspected child maltreatment has increased by 17 percent, according to another study. Foster care is intended to be temporary out-of-home care, provided by the welfare system, for children and adolescents whose immediate families are unable to care for them. Given the severity of the pathology of vulnerable parents; however, care is often needed for many months and years. In 1997, President Clinton signed the Adoption and Safe Families Act, a law designed to improve provisions for child safety, to decrease the length of time that a child remains in foster care without long-term planning, and to limit the amount of time in which a biological parent has to undergo rehabilitation to 12 months. An additional law was added to allocate federal funds for independent living assistance for adolescents and young adults aged 16 to 21 to assist them in transitioning to independent living. EPIDEMIOLOGY AND DEMOGRAPHICS OF FOSTER CARE The number of children entering foster care due to maltreatment has risen in the last decade by 19 percent. Of those children who entered foster care, there was an increase of 60 percent in the number who were identified as emotionally disturbed. In the United States, one of the most common scenarios of children being placed in foster care involves parental substance abuse, which leads to inability of the parent to care for their children. The National Center on Addiction and Substance Abuse of Columbia University reported that seven of ten abused or neglected children had parents with substance abuse. Furthermore, children in foster care were more often being raised by a single mother prior to placement compared to children in the community. Minority children are overrepresented in the foster care population. In a study utilizing birth records and child protective service (CPS), black children were more than twice as likely to be referred due to maltreatment, be substantiated as victims of maltreatment, and enter the foster care system before age 5 years, compared to white children. However, low socioeconomic black children had a lower rate of referral, substantiation, and placement in foster care than socioeconomically similar white children. Among Latinos, children of U.S.-born mothers were significantly more likely to have involvement with CPS, compared to Latino children of foreign-born mothers. However, after adjusting for socioeconomic factors, the relative risk of referral, substantiation, and entry into the foster care system was significantly higher for all Latino children than for white children. Approximately 38 percent of children in the foster care system are African American, more than three times their representation in the general population. Whites make up approximately 48 percent, and Hispanics make up almost 15 percent of foster children; 55 to 69 percent are girls, and 83.4 percent enter foster care at a mean age of 3 years. Children placed in care as infants are more likely to stay in care. Those younger than 5 years of age currently comprise the fastest growing segment of the foster care population. Studies reveal that up to 62 percent of foster children had prenatal drug exposure. NEEDS OF FOSTER CARE CHILDREN Children entering foster care have enormous mental health needs; more than 80 percent of them have developmental, emotional, or behavioral problems. It is estimated that up to 70% of these children have diagnosable psychiatric disorders. In addition, according to one study, quality of life (QOL) is significantly poorer among children in the foster care system than children in the general community. Children and adolescents living in residential care rated their QOL as poorer than those living with foster families. Up to 50 percent of foster care children exhibit depressive symptoms, and self-reports of anxiety problems occur in about 36 percent. QOL is adversely affected by the presence of mental health problems, and those youth with greater mental health difficulties rated their QOL as poorer whether in residential facilities or foster placement. In a review of the literature, psychiatric disorders found with increased frequency in foster youth were attention-deficit/hyperactivity disorder (ADHD), posttraumatic stress disorder, conduct disorders, attachment disorders, substance abuse, depression, and eating disorders. In addition to high rates of psychiatric disorders, foster care youth are referred to pediatric clinics more frequently due to multiple health problems compared to community youth. Growth abnormalities (including failure to thrive), neurological abnormalities, neuromuscular disorders, language disorders, cognitive delays, and asthma are prevalent. Health care costs in foster care youth are six to ten times that of matched non–foster-care peers. Among children 0 to 5 years of age, approximately 25 percent are seriously emotionally damaged; attachment disorders are increasingly diagnosed. Foster care children use the full range of mental health services: outpatient, acute inpatient, day treatment, partial hospitalization, and residential treatment. Adolescents in foster care are at increased risk for substance abuse, teenage pregnancies, and sexually transmitted diseases, including human immunodeficiency virus (HIV). With public health care increasingly adopting a managed health care system, which is designed to limit care, grave concern exists that the provision and delivery of services to this medically and psychiatrically vulnerable population may be seriously compromised. KINSHIP CARE FOR FOSTER CHILDREN More states are recognizing kinship care as an alternative placement option and are authorizing licensing and reimbursement to kinship caregivers who are generally female (mostly maternal grandmothers), of low income, of low education, and of minority status. Currently, nationwide, approximately 23 percent of African American children are in foster kinship care. It is unknown just how many children are in informal kinship care within the African American population, which has had a long cultural tradition of taking in children of family members who are unable to care for their offspring. The few studies available indicate that outcomes, although mixed, are somewhat more positive than for those children in nonkinship care. Children reportedly receive more positive regard from caregivers in kinship care, and a consistent outcome, when it works, is that it provides more stability than nonrelative foster care. Most foster children have consistently said that they would rather be with a family member than stay in the system. When foster children feel embraced by their families of origin, and the latter can provide appropriate nurturance and access to good therapeutic services, the foster children’s sense of identity and belonging is less disrupted. However, no demonstrable difference is seen in the need for mental health, medical, and special educational services for these children. THERAPEUTIC FOSTER CARE Therapeutic foster care (TFC) has emerged as a cost-effective alternative to the more restrictive residential treatment center (RTC). Therapeutic effectiveness is mixed. TFC is designed to provide nurturing family-based care with specialized treatment interventions from an interdisciplinary treatment team. Therapeutic foster parents are meant to be the agents of therapeutic change, functioning as extenders of the clinical treatment team. Because of the children’s special needs, therapeutic foster parents must have more extensive training than other foster parents, receive a higher reimbursement, and receive more intensive monitoring, supervision, and support from the foster care agency. Although the concept of TFC is promising, good outcome data do not show consistent success. Several models exist, but implementation that shows fidelity to empirically tested models is often spotty. Some models have proved too expensive and complicated to implement in the real-world setting. The concept of professional therapeutic parents, who are paid competitive full-time wages to care for special needs foster children, holds promise as an alternative to current prevailing practice. Clinical practice demonstrates that, when adequate and appropriate intensive in-home services with good case management is provided in a well-managed foster care setting, children can show significant gains. CULTURAL COMPETENCE Anna McPhatter defines cultural competence as the ability to use knowledge and cultural awareness to design psychosocial interventions that support and sustain healthy client– system functioning within a cultural context that is meaningful to the client. Because American society is still significantly encumbered by racial conflicts, some children have been denied placement with families of a different race, and have ended up in long-term foster care rather than in a permanent adoption placement. The Association of Black Social Workers went on record as opposing transracial placement of African American children. In 1978, the Indian Child Welfare Act transferred to Tribal Courts the power to make placement decisions about Native American children to reverse the practice of placement in non–Native American homes. Adoption studies have shown that it is not inherently harmful for children to be cross-racially adopted. Congress has passed legislation, the Multiethnic Placement Act of 1994, facilitating transracial adoptions, while maintaining the language of cultural awareness in placement decisions. The need for cultural sensitivity, respect, and a capacity to facilitate a foster child’s cultural development and identity are well acknowledged. These issues must be addressed in training providers of foster care services. PSYCHOLOGICAL ISSUES IN FOSTER CARE CHILDREN Family risk factors including alcohol and drug abuse in parents, parental neglect and abuse, and cognitive or mental or physical health problems in parents, as well as low socioeconomic status and low social support, are strongly associated with a child being placed out of the home. Psychiatric and behavior problems in the child may also contribute to being placed out of the home. Among children who return home, 40 percent reenter the foster care system. These children struggle with issues of abandonment, neglect, rejection, and physical, emotional, and sexual maltreatment. The child’s age, home environment, and the specific reasons for going into placement affect the emotional issues that the child must handle. Early abandonment and neglect can lead to anaclitic depression. Attachment issues are prevalent in this young population, because there has been no opportunity to form secure attachments with consistent nurturing figures in early life. Foster children are often unprepared for separations, which can be abrupt and repeated in the current foster care climate. Early separation from the primary caretaker is considered a major trauma for a child and sets the stage for vulnerability to subsequent trauma. Those children who bounce from foster home to foster home have their capacity to form enduring emotional attachments compromised; trust becomes a lifelong challenge. Children who have experienced traumatic physical and sexual abuse often become mistrustful, hypervigilant, aggressive, impulsive, oppositional, and avoidant as they attempt to negotiate a world that they experience as threatening, hostile, and uncaring. When a child’s early developmental period is spent in a psychosocial environment of trauma, aggression, and lack of empathy from adults, the psychological seeds are sown for later violence against the self and others. A wide range of behavior problems is likely to emerge in foster care children given their early family experiences. A pervasive problem is one of dysregulation: dysregulation of behavior, emotions and affect, attention, and sleep. The empirical data on the neurobiology of maltreatment on the developing brain reveals that stress hormones play an important role in adaptation and coping, and that these capacities are compromised in varying degrees of severity in abused and neglected children. The data also show that, because of the developmental plasticity of the brain, appropriate early intervention can induce remediation and repair at the neurobiological level. Nick, a 5-year-old, was placed in foster care because of maternal substance abuse and inability to take care of her child. When seen for a psychiatric evaluation, it was noted that all of his primary teeth were full of dental cavities. The foster mother was asked about dental care, and she responded that the dentist had said that he would wait until the teeth had fallen out, because they were his first set of teeth and did not require intervention. This response aroused suspicion that neglect in the foster family was exacerbating Nick’s hyperactive and aggressive behaviors. A neglect report was made and the investigation revealed that Nick was not only neglected, but was also being physically abused in that foster care placement. Subsequent to removal and placement with a nurturing and responsible foster family, Nick has shown considerable emotional stabilization, does well academically and socially, and is now being adopted by that family. (Adapted from case material Marilyn B. Benoit, M.D., Steven L. Nickman, M.D., and Alvin Rosenfeld, M.D.) FAMILY PRESERVATION Family preservation has come under increasing scrutiny in the last decade. Estimates on the percentage of children who are reportedly reunited vary from 66 to 90 percent. Philosophically, family reunification appears to be the right thing to do, yet approximately 40 percent of reunified children reenter out-of-home care. The field needs discriminating criteria that would identify psychosocial profiles of families that could best benefit from family preservation services. In 1996, the Child Welfare League of America (CWLA) acknowledged the failure of family preservation efforts and requested that child welfare policy makers rethink the current use of intensive family preservation. Recent research has validated poor outcomes with family preservation. Hopes are that the Adoption and Safe Families Act of 1997 will give child welfare agencies the opportunity to step back from the myopic view of family preservation and to consider the needs of the child as the major priority. The AACAP and the CWLA jointly launched a national effort to address the mental health needs of children in foster care. This effort is supported by a broad-based coalition of agencies that are all stakeholders in foster care. The coalition proposes that the foster care system be child focused, but inclusive of the biological and foster families in intervention planning on the child’s behalf if families are to be preserved. One case of a 7-year-old boy who was in foster care for 2 years is illustrative of why some family preservation efforts fail. When James was returned to his biological mother, she was in a new marriage with a new baby. Her husband was new to parenting. The family was financially strapped and lived under harsh conditions. James’ mother completed the required parenting course for resuming custody of her child, and seemed pleased to have him back with her; however, no supports were put in place to assist this young couple financially or with any family therapy, psychoeducation, or case management interventions. Frequent and increasingly urgent calls to the child welfare family reunification services were made to seek respite and financial help, but this was not possible. The outcome for James was that he was reabused and had to reenter the foster care system. This outcome represents a failure of the system, but also translates into a debilitated family, with a profound sense of failure. (Adapted from case material from Marilyn B. Benoit, M.D., Steven L. Nickman, M.D., and Alvin Rosenfeld, M.D.) FOSTER CARE OUTCOMES AND RESEARCH INITIATIVES The overall quality of available outcome studies is poor. Some patterns, however, recur across studies. Several studies reveal that 15 to 39 percent of the homeless are foster care graduates, who are also overrepresented among adult substance abusers and clients in the criminal justice system. It is likely that the reasons that initially precipitated the child’s foster care placement contributed to the negative adult outcomes. Studies indicate that children entering care who have been victimized, who have substance-abusing parents or parents with major mental illness or high criminality, or both, and who come from homes with a high degree of domestic violence are at greater risk of having poor outcomes. Research on early maltreatment indicates that the influence of maltreatment on brain development can be profound over the life span. Developmental disabilities occur in more than 50 percent of the foster care population. Children returned to their families of origin typically have fared worse than those who have remained in long-term placement. Several studies report findings indicating that multiple placements and poor parental involvement consistently lead to negative outcomes. Federal mandate requires states to maintain a tracking system for children in foster care. New reporting systems, the Adoption and Foster Care Analysis and Reporting System (AFCARS) and the Statewide Automated Child Welfare Information System (SACWIS), are available nationwide. States are being monitored for compliance with their use, and continued federal funds are contingent on the implementation of these information systems. Because foster care placement is the result of psychosocial environmental failure, fixing the existing system requires more than good information systems. Integration of sound, theory-driven, child-focused, family-centered services, collaboratively funded by multiple governmental agencies, is essential. Through the use of longitudinal, research-based performance measures, reliable data are emerging. The National Institutes of Mental Health (NIMH) has funded some research focusing on foster care children and youth. The complexity of the impact of ever-changing psychosocial variables makes this type of research challenging. Despite that, it must be done if welfare dollars are to be spent doing the right thing for needy children and their families. In 2004, in a groundbreaking study, the Pew Commission on Children in Foster Care made sweeping recommendations to overhaul the system, stating that “children deserve more from our child welfare system.” HISTORY OF ADOPTION Adoption has existed in different forms throughout history. In ancient Babylonia, it provided for the transmission of property or artisan’s skills, whereas, in the Roman Empire, it was often used to elevate the status of an adult protégé. In some Pacific islands, adoption of young children formed part of an exchange system between related clans. Concerns expressed by adopted persons about not knowing their roots are as ancient as they are contemporary. Euripides’ Ion contains a touching dialogue between a woman in search of the child she had given up years before and a young priest of Apollo, who does not know that he is the woman’s son and says that the only mother he knows is Apollo’s priestess. Historically, closed adoptions were common practice. That was done to ensure the sealed identities of birth and adoptive parents and was believed to be in the best interests of adopted children. That practice is now considered flawed; contemporary, although still controversial, thinking is that most adoptees should grow up knowing of their adoption status, as well as the identities of their birth parents. Currently, adoptees, as well as many birth parents and adoptive parents, increasingly have shared interests in legislation that affects the open or closed status of birth records and the placement of children in families. The phrase adoption triad has come to stand for these shared interests. Several other organizations represent each of these three groups, and those organizations often have divergent agendas. Since the 1980s, adoption practice has been profoundly affected by federal legislation. EPIDEMIOLOGY OF ADOPTION Estimates suggest that between 2.5 and 3.5 percent of children in the United States are adopted, with more than 2 percent adopted by nonrelatives, and about 1.5 percent in relative adoptions, which include stepparents. Foster care children who are adopted account for about 15 percent of all adopted children. Approximately 125,000 children are adopted each year, in a variety of scenarios. Infants may be relinquished by their biological parents at birth and adopted through private agencies. These adoptions are increasingly “open,” with some continued contact with biological parents. About 50,000 babies are adopted in this manner each year. Another 50,000 children are adopted through the child welfare system, and these children have often been exposed to multiple foster home placements before they are adopted. These adoptees range in age, with more than half of them being older than 6 years of age, and the majority of them having experienced significant early abuse or neglect. INTERNATIONAL ADOPTION International adoptions have been growing over the last two decades. Each year more than 20,000 children are adopted from overseas, and many of these are transracial adoptions. More than 17,000 children were adopted from Guatemala, for example, in the last two decades. In the Guatemalan adoptees, the mean age was 1.5 years and the children had previously resided in orphanages, foster homes, or mixed-care settings. Investigation of the health records of international adoptees who were evaluated in an international adoption specialty clinic in the U.S. revealed that younger children at the time of adoption have better growth, language development, cognitive skills, and competence in activities of daily living compared to children who were older at time of adoption. Among children matched for age, gender, and time from adoption to evaluation, those who were previously living in foster care were observed to have higher cognitive scores and improved growth compared to children who had resided in orphanages. These findings support the priority of adoptive placement at younger ages and that foster care has benefits over orphanage care. EARLY CHILDHOOD VERSUS LATE ADOPTION Data suggest that earlier age adoption predicts better outcome than adoption in middle or late childhood. A recent prospective study examined factors related to successful outcome in public adoption of children ranging in age from 5 to 11 years of age. Prospective data were collected from domestic adoptions in the United Kingdom at the 1st year, and 6 years later on 108 adoptees who were placed primarily because of situations involving childhood abuse and neglect. Outcome was assessed by the disruption rate and measures of psychological adaptation. At the adolescent follow-up, 23 percent of the adoption placements had been disrupted, 49 percent were continuing with positive adaptations, and 28 percent were ongoing but with significant conflicts. Four factors contributed independently to the risk of disruptions: older age at placement, report of being singled out and rejected by siblings, time in care, and greater degree of behavioral problems. Given that almost half of the placements were ongoing, it is apparent that later childhood age of adoption can also be successful; assessment of the constellation of the adoptive families, and of the children’s behavioral problems, may determine the likelihood of positive outcome for school-aged child adoptees. BIRTH PARENTS: SEARCH AND REUNION The increasing trend toward open adoption allows the opportunity for adoptees to more easily search and successfully find their birth parents. Many adoptive parents choose open adoptions in the belief that they can experience a greater connection with the child if they have some relationship with the birth mother. Some adoptees want to develop an ongoing relationship with birth parents, but many who search are satisfied to meet birth parents without further correspondence. Outcomes of reunions with birth parents vary widely. In some cases, especially when the birth parents are well functioning and welcoming toward their child, the adoptee may experience a sense of relief and joy in knowing that their birth mother is no longer vulnerable. REFERENCES Brenner E, Freundlich M. Enhancing the safety of children in foster care and family support programs: Automated critical incident reporting. Child Welfare. 2006;85:611. Briggs-Gowan MJ, Ford JD, Fraleigh L, McCarthy K, Carter AS. Prevalence of exposure to potentially traumatic events in a healthy birth cohort of very young children in the northeastern United States. J Traum Stress. 2010;23:725–733. Conn AM, Szilagyi MA, Franke TM, Albertin CS, Blumkin AK, Szilagyi PG. Trends in child protection and out-of-home care. Pediatrics. 2013;132:712–719. Carnochan S, Moore M, Austin MJ. Achieving timely adoption. Journal of Evidence-Based Social Work. 2012;10:210–219. 53 - 31.19c Child Maltreatment and Neglect 31.19c Child Maltreatment and Neglect Damnjanovic M, Lakic A, Sevanovic D, Jovanovic A. Effects of mental health on quality of life in children and adolescents living in residential and foster care: A cross-sectional study. Epidemiol and Psychiatr Sci. 2011;20:257–262. Garcia AR, Pecora PJ, Aisenberg E. Institutional predictors of developmental outcomes among racially diverse foster care alumni. Am J Orthopsychiatry. 2012;82:573–584. Greeson JK, Briggs EC, Kisiel C, Layne CM, Ake III GS, Ko SJ, et al. Complex trauma and mental health in children and adolescents placed in foster care: Findings from the National Child Traumatic Stress Network. Child Welfare. 2011;90:91–108. Horowitz SM, Hurlburt MS, Cohen SD, Zhang J. Predictors of placement for children who initially remained in their homes after an investigation for abuse or neglect. Child Abuse Neglect. 2011;35:188–198. Lehmann S, Havik OE, Havik T, Heiervang. Mental disorders in foster children: A study of prevalence, comorbidity and risk factors. Child Adolesc Psychiatry Ment Health. 2013;7:39. McWey LM, Henderson TL, Tice SN. Mental health issues and the foster care system: An examination of the impact of the Adoption and Safe Families Act. J Marital Fam Ther. 2006;32:195. Oswald SH, Fegert JM, Goldbeck L. Posttraumatic stress symptoms in foster children following maltreatment and neglect. Verhaltenstherapie. 2010;20:37–44. Putnam-Hornstein E, Needell B, King B, Johnson-Motoyama M. Racial and ethnic disparities: A population-based examination of risk factors for involvement with child protective services. Child Abuse Neglect. 2013;37:33–46. The Pew Commission on Children in Foster Care. Fostering the Future: Safety Permanence and Well-Being for Children in Foster Care. Washington, DC; 2004. Rushton A, Dacne C. The adoption of children from public care: A prospective study of outcome in adolescence. J Am Acad Child Adolesc Psychiatry. 2006;45:877. Sexson SB. Adoption and Foster Care. In: Sadock BJ, Sadock VA, Ruiz P. eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Vol. II. Philadelphia: Lippincott Williams & Wilkins; 2009:3784. Wilcox BL, Weisz, Miller MK. Practical guidelines for educating policy makers: The family impact seminar as an approach to advancing the interests of children and families in the policy arena. J Clin Child Adolesc Psychol. 2005;34:638. 31.19c Child Maltreatment, Abuse and Neglect Child maltreatment includes all types of abuse and neglect and is a major public health concern in the United States. The Centers for Disease Control and Prevention (CDC) estimate that one in every five children in the United States has been a victim of child maltreatment. Among the CDC’s estimates of maltreated children, 9 percent were victims of physical abuse, 1 percent were victims of sexual abuse, 4 percent were victims of neglect, and 12 percent experienced emotional abuse. Estimates of children maltreated in the United States each year are close to 1 million, and the annual number of deaths caused by abuse or neglect is reported to be about 1,500. A majority of child neglect and abuse occurs in infancy and early childhood, negatively impacting overall brain development, and disrupting time-sensitive developmental brain processes. A growing body of research suggests that child maltreatment potentially results in longterm damage in the neuroendocrine system, cell loss, and delays in myelination in the hippocampus and prefrontal cortex, as well as a chronic inflammatory state independent of clinical comorbidities. The National Longitudinal Study on Adolescent Health investigated the prevalence, risk factors, and health consequences of maltreatment in 12,118 adolescents. Maltreated adolescents retrospectively reported the most common experiences were being left home alone as a child, (reported by 41.5 percent of the sample), physical assault (reported by 28.4 percent), physical neglect (reported by 11.8 percent), and sexual abuse (reported by 4.5 percent). Each type of maltreatment was associated with at least eight of the ten adolescent health risks examined, including self-report of depression, regular alcohol use, binge drinking, marijuana use, overweight status, generally “poor” health, inhalant use, and aggressive behaviors, including fighting and hurting others. Clearly, the effects of self-reported maltreatment had far ranging and long-lasting associations with multiple detrimental consequences. The identification, management, and treatment of child maltreatment require cooperative efforts between professionals, including primary care physicians, emergency room staff, law enforcement, attorneys, social service staff, and mental health professionals. Perpetrators typically deny abuse or neglect and maltreated children often fear disclosure of their abuse or neglect. DEFINITIONS DSM-5 The Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5) lists Child Maltreatment and Neglect in the section “Other Conditions That May Be a Focus of Clinical Attention.” The presence of Child physical abuse, Child sexual abuse, Child neglect, and Child psychological abuse can be coded as confirmed or suspected and as an Initial encounter or a Subsequent encounter. Under a subcategory of “other circumstances related to” each form of child maltreatment or neglect, five “V” coded clinical situations related to maltreatment can be coded. These include the following (1) Encounter for mental health services for victim of child maltreatment by parent, (2) Encounter for mental health services for victim of nonparental child maltreatment, (3) Personal history (past history) of childhood maltreatment, (4) Encounter for mental health services for perpetrator of parental child maltreatment, (5) Encounter for mental health services for perpetrator of nonparental child maltreatment. Federal Law The Child Abuse Prevention and Treatment Act was passed in 1974 and has been amended several times, most recently in 2003. In federal law, child abuse and neglect mean, at a minimum, any recent act or failure to act on the part of a parent or caretaker that results in death, serious physical or emotional harm, or sexual abuse or exploitation. It also includes an act or failure to act that presents an imminent risk of serious harm. In federal law, sexual abuse means the employment, use, persuasion, inducement, enticement, or coercion of any child to engage in or to assist any other person to engage in any sexually explicit conduct (or simulation of such conduct for the purpose of producing a visual depiction of such conduct) or the rape (and in cases of caretaker or interfamilial relationships, statutory rape), molestation, prostitution, or other forms of sexual exploitation of children or incest with children. State Law A large mass of legal definitions and guidelines exists at the state level. The legal definitions of terms related to the maltreatment of children vary from one jurisdiction to another, so clinicians should be aware of the definitions used in their own locale. The following generic definitions are used in this section. Neglect Neglect, the most prevalent form of child maltreatment, is the failure to provide adequate care and protection for children. Children can be harmed by malicious or ignorant withholding of physical, emotional, and educational necessities. Neglect includes failure to feed children adequately and to protect them from danger. Physical neglect includes abandonment, expulsion from home, disruptive custodial care, inadequate supervision, and reckless disregard for a child’s safety and welfare. Medical neglect includes refusal, delay, or failure to provide medical care. Educational neglect includes failure to enroll a child in school and allowing chronic truancy. Physical Abuse Physical abuse can be defined as any act that results in a nonaccidental physical injury, such as beating, punching, kicking, biting, burning, and poisoning. Some physical abuse is the result of unreasonably severe corporal punishment or unjustifiable punishment. Physical abuse can be organized by damage to the site of injury: skin and surface tissue, the head, internal organs, and skeletal. Emotional Abuse Emotional or psychological abuse occurs when a person conveys to children that they are worthless, flawed, unloved, unwanted, or endangered. The perpetrator may spurn, terrorize, ignore, isolate, or berate the child. Emotional abuse includes verbal assaults (e.g., belittling, screaming, threats, blaming, or sarcasm), exposing the child to domestic violence, overpressuring through excessively advanced expectations, and encouraging or instructing the child to engage in antisocial activities. The severity of emotional abuse depends on (1) whether the perpetrator actually intends to inflict harm on the child and (2) whether the abusive behaviors are likely to cause harm to the child. Some authors believe that the terms emotional or psychological abuse should not be used and that verbal abuse more accurately describes the pathological behavior of the caregiver. Sexual Abuse Sexual abuse of children refers to sexual behavior between a child and an adult or between two children when one of them is significantly older or uses coercion. The perpetrator and the victim may be of the same sex or the opposite sex. The sexual behaviors include touching breasts, buttocks, and genitals, whether the victim is dressed or undressed; exhibitionism; fellatio; cunnilingus; and penetration of the vagina or anus with sexual organs or objects. Sexual abuse can involve behavior over an extended time or a single incident. Developmental factors must be considered in assessing whether sexual activities between two children are abusive or normative. In addition to the forms of inappropriate sexual touching, sexual abuse also refers to sexual exploitation of children, for instance, conduct or activities related to pornography depicting minors and promoting or trafficking in prostitution of minors. Ritual Abuse Cult-based ritual abuse, which includes satanic ritual abuse, is physical, sexual, or psychological abuse that involves bizarre or ceremonial activity that is religiously or spiritually motivated. Typically, multiple perpetrators abuse multiple victims over an extended period. Ritual abuse is a controversial concept; some professionals believed in the 1990s that ritual abuse was a common, horrible phenomenon in society, whereas others were skeptical about most allegations and descriptions of ritual abuse. Perpetrators of Abuse Some lack of consistency is seen in who may be defined as an abuse perpetrator. Usually, a person must be a parent or designated caregiver to be charged with neglect, physical abuse, or emotional abuse. Another adult (e.g., a stranger) who injures a child would be charged with battery, not with child abuse. On the other hand, a caretaker or any other person could be charged with child sexual abuse. State laws vary in this regard. ETIOLOGY Physical Abuse Although child abuse occurs at all socioeconomic levels, it is highly associated with poverty and psychosocial stress, parental substance abuse, and mental illness. Child maltreatment is strongly correlated with less parental education, underemployment, poor housing, welfare reliance, and single parenting. Child abuse tends to occur more often in families characterized by domestic violence, social isolation, parental mental illness, and drug and alcohol abuse. The probability of maltreatment may be increased by risk factors in the child such as prematurity, intellectual disability, and physical handicap. In addition, the risk of child abuse increases in families with many children. Sexual Abuse Social, cultural, physiological, and psychological factors all contribute to the breakdown of the incest taboo. Incestuous behavior has been associated with alcohol abuse, overcrowding, increased physical proximity, and rural isolation that prevents adequate extrafamilial contacts. Some communities may be more tolerant of incestuous behavior. Major mental disorders and intellectual deficiency have been described in some perpetrators of incest and sexual abuse. CLINICAL FEATURES Maltreated children manifest a variety of emotional, behavioral, and somatic reactions. These psychological symptoms are neither specific nor pathognomonic: The same symptoms can occur without any history of abuse. The psychological symptoms manifested by abused children and the behaviors of abusive parents can be organized into clinical patterns. Although it may be helpful to note whether a particular case falls into one of these patterns, that in itself is not diagnostic of child abuse. Physically Abused Children In many cases, the physical examination and radiological evaluation show evidence of repeated suspicious injuries. Abused children display behaviors that should arouse the suspicions of the health professional. For example, these children may be unusually fearful, docile, distrustful, and guarded. On the other hand, they may be disruptive and aggressive. They may be wary of physical contact and show no expectation of being comforted by adults, they may be on the alert for danger and continually size up the environment, and they may be afraid to go home. The literature regarding the psychological consequences of physical abuse and neglect indicates a wide range of effects: affect dysregulation, insecure and atypical attachment patterns, impaired peer relationships involving increased aggression or social withdrawal, and academic underachievement. Physically abused children exhibit a range of psychopathology, including depression, conduct disorder, ADHD, oppositional defiant disorder, dissociation, and posttraumatic stress disorder (PTSD). Physically Abusive Parents Abusive parents often feel significant guilt, and may delay seeking help for the child’s injuries, fearful that the child will be taken away. Often the history of how a child sustained injuries given by the parents is implausible or incompatible with the physical findings. Parents may blame a sibling or claim that the children injured themselves. The characteristics of abusive parents often include a history of abuse in their own early lives, a lack of empathy for the child, unrealistic expectations of the child, and an impaired parent–child attachment. Katie, 3 years of age, had been exhibiting new negative and aggressive behavior at preschool beginning 3 months after the birth of her brother. Katie’s teacher observed her increased irritability and aggression, at times pushing other children, and she had recently hit a classmate with a wooden block, causing a laceration of the child’s lip. When Katie’s teacher took her aside to talk about her behavior, she noticed several bruises on Katie’s arms and face. When her teacher asked Katie how she had gotten the bruises, Katie replied “my mommy’s boyfriend gets mad at me and hits me with his belt.” The teacher reported suspected child abuse to Child Protective Services. Katie’s teacher also called her mother to let her know what was happening, and suggested that they take Katie for a psychiatric evaluation. Katie’s baby brother was colicky and slept only for short periods of time throughout the day and night. He stopped crying only when his mother held him. Her mother, therefore, had little time for Katie, and the mother’s boyfriend was left to take care of Katie on evenings after day care and on weekends. He began to drink more than usual and became increasingly irritable. Katie’s mother and her boyfriend often argued, and Katie had seen her mother physically pushed and threatened by her boyfriend. Katie, who was a bright, curious, and talkative child, had tried to be helpful by asking to hold the baby. When refused, however, Katie became upset and would lie on the floor and have a tantrum. Katie began to have difficulty falling asleep and awoke repeatedly during the night. Katie’s mother’s boyfriend would become extremely angry when Katie would wake him up, and often told her to shut up and slapped her when she told him that she couldn’t go back to sleep. On many occasions, he responded to her tantrums or repeated demands for attention by hitting her with his belt. Child Protective Services suggested that mother’s boyfriend voluntarily move out, and no longer spend time alone with Katie caring for her, which he did begrudgingly, and Katie and her mother began a family therapy program that included parenting training for Katie’s mother and, a behavioral program to help Katie with her tantrums. Katie’s mother’s boyfriend attended Alcoholics Anonymous (AA) meetings and stopped drinking. He was able to control his anger, and was allowed to visit the home, as long as Katie’s mother was present. Within the next three months, Katie’s aggressive behavior had ceased, and she was less irritable and was no longer having tantrums. She was doing well with peers, was sleeping through the night, and was no longer afraid to be at home. (Adapted from case material from William Bernet, M.D.) Sexually Abused Children A variety of symptoms, behavioral changes, and diagnoses sometimes occur in sexually abused children: anxiety symptoms, dissociative reactions and hysterical symptoms, depression, disturbances in sexual behaviors, and somatic complaints. Anxiety Symptoms. Anxiety symptoms include fearfulness, phobias, insomnia, nightmares that directly portray the abuse, somatic complaints, and PTSD. Dissociative Reactions and Hysterical Symptoms. The child may exhibit periods of amnesia, daydreaming, trance-like states, hysterical seizures, and symptoms of dissociative identity disorder. Depression. Depression may be manifested by low self-esteem and suicidal and selfmutilative behaviors. Disturbances in Sexual Behaviors. Some sexual behaviors are particularly suggestive of abuse, such as masturbating with an object, imitating intercourse, and inserting objects into the vagina or anus. Sexually abused children may display sexually aggressive behavior toward others. Other sexual behaviors are less specific, such as showing genitals to other children and touching the genitals of others. A younger child may manifest age-inappropriate sexual knowledge. In contrast to these overly sexualized behaviors, the child may avoid sexual stimuli through phobias and inhibitions. Somatic Complaints. Somatic complaints include enuresis, encopresis, anal and vaginal itching, anorexia, bulimia, obesity, headache, and stomachache. These symptoms are not pathognomonic. Nonabused children may exhibit any of these symptoms and behaviors. For example, normal, nonabused children commonly exhibit sexual behaviors, such as masturbating, displaying their genitals, and trying to look at people who are undressing. Approximately one third of sexually abused children have no apparent symptoms. Many adults who were abused as children have no significant abuse-related symptoms. On the other hand, the following factors tend to be associated with more severe symptoms in the victims of sexual abuse: greater frequency and duration of abuse, sexual abuse that involved force or penetration, and sexual abuse perpetrated by the child’s father or stepfather. Other factors associated with poorer prognosis are the child’s perception of not being believed, family dysfunction, and lack of maternal support. Also, multiple investigatory interviews appear to increase symptoms. Intrafamilial Sexual Abuse Incest can be defined strictly as sexual relations between close blood relatives, that is, between a child and the father, uncle, or sibling. Because of increased reporting, sibling incest is an area of growing concern. In its broader sense, incest includes sexual intercourse between a child and a stepparent or stepsibling. Although father–daughter incest is the most common form, incest can also involve father and son, mother and daughter, and mother and son. Intrafamilial sexual abuse and other sexual abuse that occurs over a period of time is characterized by a particular pattern or sequence of steps. Victims of sexual abuse recount a gradual progression of boundary violations by the perpetrator, starting with tiny invasions and escalating to serious, overwhelming intrusions. Healthy, selfconfident children rebuff the intrusions directly (via temper tantrums and verbal disagreements) or indirectly (through silence and distancing maneuvers) or by adopting any strategy that causes the offender to refrain. Sexual abuse that occurs over a period of time evolves through five phases: engagement, sexual interaction, secrecy, disclosure, and suppression. Engagement Phase. The perpetrator induces the child into a special relationship. The daughter in father–daughter incest has frequently had a close relationship with her father throughout her childhood and may be pleased at first when he approaches her sexually. Sexual Interaction Phase. The sexual behaviors progress from less to more intrusive forms of abuse. As the behavior continues, the abused daughter becomes confused and frightened, because she never knows whether her father will be parental or sexual. If the victim tells her mother about the abuse, the mother may not be supportive. The mother often refuses to believe her daughter’s reports or refuses to confront her husband with her suspicions. Because the father provides special attention to a particular daughter, her brothers and sisters may distance themselves from her. Secrecy Phase. The perpetrator threatens the victim not to tell. The father, fearful that his daughter may expose their relationship and often jealously possessive of her, interferes with the girl’s development of normal peer relationships. Disclosure Phase. The abuse is discovered accidentally (when another person walks into the room and sees it), through the child’s reporting it to a responsible adult, or when the child is brought for medical attention and an alert clinician asks the right questions. Suppression Phase. The child often retracts the statements of the disclosure because of family pressure or because of the child’s own mental processes. That is, the child may perceive that violent or intrusive attention is synonymous with interest or affection. Many incest survivors rally around their perpetrators, seeking to capture any modicum of tenderness or interest. At times, affection for the perpetrator outweighs the facts of abuse, and children recant their statements about sexual assault, regardless of substantiated evidence of molestation. A family with a comfortable financial situation lived in a pleasant, clean house in a nice neighborhood, but they had no friends. Their four teenagers never had visitors. One day, the oldest girl, 17 years of age, went to the police and told them that she had a baby at home and that her own father was the father of the baby. The teen said that her father had been having sexual relations with her for more than 4 years and that he was now doing the same with her younger sisters. The mother admitted suspecting the situation for years, but she had not reported it to the authorities for fear of losing her husband and her children. (Courtesy of William Bernet, M.D.) Extrafamilial Sexual Abuse Of course, sexual abuse is not limited to incest. Children can be abducted and sexually abused by strangers. A perpetrator may observe a playground and may identify a child who is not closely supervised. A pedophile may molest this child and hundreds of other children before he or she is apprehended. For the child, this is usually a single, isolated experience. On the other hand, children can be repeatedly abused by trusted adults, such as teachers, counselors, family friends, and clergy. In this scenario, the pedophilic perpetrator grooms the child over a period of time. He or she gains the friendship of children through enjoyable activities and gifts, introduces sexual activities that may seem innocent and even pleasurable, and progresses to more intrusive activities. The pedophile encourages secrecy. A solo sex ring is a form of child sexual abuse that involves one adult perpetrator and multiple child victims, who may know about each other’s sexual activities with the perpetrator. A sex ring may also involve multiple perpetrators and multiple victims. Neurobiological and Health Consequences of Child Maltreatment Current data document long-term physical and mental health consequences of child physical abuse, sexual abuse, emotional abuse, and neglect. Severe physical abuse and repeated sexual abuse cause changes in the child’s developing brain that persist into adulthood. A review of 20 studies concluded that child maltreatment is associated with future increased levels of inflammatory markers such as increased C-reactive protein (CRP), fibrinogen, and proinflammatory cytokines. The association of child maltreatment with an increased state of inflammatory markers in adulthood is a robust finding. However, it is not clear how this occurs, and how it impacts functioning. According to the CDC, and the Child Maltreatment report, long-term consequences of child maltreatment lead to increased risk of multiple physical illnesses and high risk behaviors such as alcoholism and drug abuse, which in turn can lead to depression, unemployment, and unstable relationships. Physical abuse, emotional abuse, and neglect are strongly related to future depressive disorders, anxiety disorders, eating disorders, suicidal behaviors, drug use, and risky sexual behavior. Child maltreatment is also associated with a host of physical conditions and illnesses, including ischemic heart disease, liver disease, adolescent pregnancy, chronic obstructive pulmonary disease, fetal death, and skeletal fractures. Studies have demonstrated that adults with childhood histories of maltreatment are at higher risk for abnormalities on magnetic resonance imaging (MRI) of the brain that indicate reduced size of the adult hippocampus. These abnormalities are more pronounced on the left side of the brain. Deficient integration exists between the left and right hemispheres, manifested by reduced size of the corpus callosum. These neurobiological effects of child maltreatment probably mediate the behavioral and psychological symptoms that follow abuse, such as increased aggressiveness, heightened autonomic arousal, depression, and memory problems. EVALUATION PROCESS The evaluation of a child or adolescent who may have been physically or sexually abused depends on its circumstances and context. Practitioners must consider whether they are conducting a forensic evaluation, which has legal implications and may ultimately be used in court, or a clinical evaluation, which is done for a therapeutic purpose. A forensic evaluation emphasizes collecting accurate and complete data to determine—as objectively as possible—what happened to the child. Was the injury an accident, was it self-inflicted, or was it a result of parental abuse? Was the child actually sexually abused, or was he or she indoctrinated to believe that he or she was abused? The data collected in a forensic evaluation must be preserved in a reliable manner through audiotape, videotape, or detailed notes. The results of the forensic evaluation are organized into a report that is read by attorneys, a judge, and others. The emphasis in a therapeutic evaluation is to assess psychological strengths and weaknesses, to make a clinical diagnosis, to develop a treatment plan, and to lay the foundation for continuing psychotherapy. The clinician is also interested in determining what happened to the child, but it is not as essential to distinguish facts from fantasies. Compared with the forensic evaluation, the psychotherapist does not need to keep such detailed records and ordinarily does not prepare a report for court. In addition to distinguishing a forensic examination from a therapeutic consultation, a number of factors can affect the evaluation of a child who was abused or may have been abused: whether one is a pediatrician in an emergency department or a child psychiatrist in an office, whether a parent or another person is suspected of the abuse, the severity of the abuse and the victim’s relationship to the perpetrator, whether physical signs of abuse are obvious or absent, the age and gender of the child, and the degree of anxiety, defensiveness, anger, or mental disorganization that the child exhibits. Often, the examiner must be creative and persistent. From the psychiatric perspective, the interview is usually the primary source of information, and the physical examination is secondary. In practice, children who may have been neglected or sexually abused are interviewed first and are later given a physical examination and other tests. A child who has been physically abused is more likely to have a physical examination that may be followed by a psychiatric interview. When the child is brought to the emergency room, a detailed and spontaneous account of the injury should be obtained promptly from parents or other caregivers before secondary details and rationalizations cloud the information provided. The interviewer should allow the caregiver to explain, to expound, to derail, or to detour the story line. An abuser or codependent parent may claim to have happened on the injured child in a coma or bleeding from some unknown trauma or to have noticed significant bruising, burns, or a crooked extremity while bathing the child. Comparing the parents’ histories can provide valuable insight into how power is wielded in the family unit. A one-month-old baby girl was transferred from a rural hospital to a university medical center because of a reported near sudden infant death syndrome (SIDS). The child was unresponsive and required mechanical ventilation. A nuclear magnetic resonance imaging (MRI) study revealed bilateral subdural hematomas, subarachnoid hemorrhage, and hemorrhage in the parenchyma of the brain. An X-ray skeletal survey showed two posterior rib fractures. An ophthalmologist observed extensive retinal hemorrhages. After the child was admitted to the Pediatric Intensive Care Unit, the child abuse consultant interviewed the parents separately. The mother, 28 years of age, said that she had recently started a new job. The baby was perfectly fine when she left her in the care of her live-in boyfriend, the child’s biological father. The father, 24 years of age, said that when he checked on the baby, he found her not breathing, blue, and unresponsive. He ran to report this to a neighbor and then called 911. The child abuse consultant suggested to the father that the baby must have been injured in some way and asked whether the father had any explanation for this injury. The father said, “I shook the baby after I found her not breathing.” The consultant concluded that severe child abuse had occurred in the form of shaken baby syndrome. The consultant notified child protective services and the local police department, so that they could initiate and coordinate their investigation. (Courtesy of William Bernet, M.D.) Suspected Sexual Abuse. The examiner should consider the possibility that the parents are not telling the truth. This situation is more complex, however, than suspected physical abuse. For example, the mother may wish to avoid the discovery of father–daughter incest by blaming the child’s genital injury on another child or a stranger. In another scenario, the mother may concoct an allegation of incest when the child had never been abused at all. The first version protects a father who is guilty; the second version implicates a father who is innocent. The examiner should determine how the allegation originally arose and what subsequent statements were made. Determine the emotional tone of the first disclosure (e.g., whether the disclosure arose in the context of a high level of suspicion of abuse). Determine the sequence of previous examinations, the techniques used, and what was reported. Try to determine whether the previous interviews may have distorted the child’s recollections. If possible, review transcripts, audiotapes, and videotapes of earlier interviews. Seek a history of overstimulation, prior abuse, or other traumas. Consider other stressors that could account for the child’s symptoms. The examiner should also ask about exposure to other possible male and female perpetrators. In Either Case. Whether physical or sexual abuse is involved, a pertinent psychosocial history should be collected and organized, including the following: Symptoms and behavioral changes that sometimes occur in abused children Confounding variables, such as psychiatric disorder or cognitive impairment, that may need to be considered Family’s attitude toward discipline, sex, and modesty Developmental history from birth through periods of possible trauma to the present Family history, such as earlier abuse of or by the parents, substance abuse by the parents, spouse abuse, and psychiatric disorder in the parents Underlying motivation and possible psychopathology of adults involved Collateral Information The evaluator should consider requesting collateral information from the following people, after obtaining authorizations: protective services, school personnel, other caregivers (e.g., babysitters), other family members (e.g., siblings), the pediatrician, and police reports. Child Interview Several structured and semistructured interview protocols have been developed that were designed to maximize the amount of accurate information and to minimize mistaken or false information provided by children. These approaches include the Cognitive Interview, which encourages witnesses to search their memories in various ways, such as recalling events forward and then backward. The Step-Wise Interview is a funnel approach that starts with open-ended questions and, if necessary, moves to more specific questions. The interview protocol developed at the National Institute of Child Health and Human Development (NICHD) includes a series of phases and makes use of detailed interview scripts. Although these protocols may be particularly important in a forensic context, experienced clinicians endorse flexibility and consistent good-hearted behavior by the interviewer. As with seeing any patient, the evaluator must size up the situation and use techniques that are likely to help the youngster become comfortable and communicative. One victim might need a favorite object (e.g., a teddy bear or a toy truck); another might need to have a particular person included in the interview. Some children are comfortable talking; others prefer to draw pictures. An unrelated joke, a shared cookie, or a picture on the evaluator’s wall may lead to a disclosure of abuse. Important comments might be made while chatting during the break time, instead of during the structured interviews. GENOTYPE AND MALTREATMENT: RISK AND PROTECTIVE FACTORS Two studies of Caucasian males have provided evidence that particular genotypes with high levels of monoamine oxidase A (MAOA) seem to protect against the malignant impact of childhood maltreatment on the development of conduct disorder and antisocial behavioral patterns. Subjects in a prospective cohort design involving courtsubstantiated cases of child abuse and neglect and matched comparison groups were followed into adulthood. A composite index of violent and antisocial behavior (VASB) was created based on arrest record, self-report, and diagnostic information. Genotypes associated with high levels of MAOA activity were correlated with less risk of violent and antisocial behavior in later life for Caucasians, but this effect was not found for non-Caucasians. This result was not replicated in a group of adolescents with respect to the development of adolescent conduct disorder. Further studies are needed to understand the possible links between genotypes of high levels of MAOA and potential behavioral outcomes. TREATMENT AND PREVENTION STRATEGIES The immediate strategic intervention is to ensure the child’s safety, which may require the child’s removal from an abusive or neglectful home environment. Physicians are among a group of professionals who are mandated by law to report suspected child abuse or neglect to the local protective services agency. Several evidence-based psychotherapies now exist in the treatment of childhood abuse and neglect. These include Multisystemic Therapy for Child Abuse and Neglect (MSTCAN), Parent-Child Interaction Therapy (PCIT), adapted for children who have been physically abused, and Combined Parent-Child Cognitive Behavioral Therapy (CPCCBT). MST-CAN uses a home-based model in which therapists come to the home to involve families in a highly monitored positive interactional approach toward their physically abused children. Parents receive support and guidance to care for their children in a less harsh, nonneglectful manner. This approach has been shown to reduce behavioral problems in the children, while increasing parental understanding of meeting their children’s needs in a safe environment. PCIT consists of combined treatment for parents and children in which parenting is coached directly by the therapist and practiced in sessions with parents and children together. Typically, therapists observe parent–child interactions through a one-way mirror and coach parents during the live interaction using a radio earphone. This model is based on the premise that changing parent–child interaction patterns will break the cycle of parent and child behaviors that maintain abusive behavior, and replace it with more nurturing and supportive interactions. Although PCIT has been shown to be effective, additional treatments are likely to be needed for parents with mental health problems such as depression or substance use. CPC-CBT is designed to help parents to develop more positive strategies with their children and to help children to cope more effectively with their past abuse and to learn more positive interactions with parents. Therapeutic techniques used with parents include motivational interviewing, psychoeducation, adaptive coping skills, and better problem solving when difficult situations arise. Therapeutic strategies used with children focus on the development of positive coping, anger management, and gradual exposure through the use of a developmentally appropriate trauma narrative. Parents and children participate together in sessions in which the parent is able to convey complete responsibility for their abusive behavior, and then, the parent and child collaborate on a new joint family plan that promotes safety and more positive relationships. Therapeutic sessions with the child and parent together appear to add to the effectiveness of treatment. Children who have been maltreated are at increased risk for further maltreatment according to studies of child victims of abuse and maltreatment. Studies have shown that four factors were most consistently identified as predictors of future maltreatment: number of previous episodes of maltreatment; neglect as the form of maltreatment; parental conflict; and parental psychiatric illness. Maltreated children were found to be about six times more likely to experience recurrent maltreatment, and the risk of recurrence was highest within 30 days of the index experience. This underscores the importance of a careful examination of the protective factors in the home environment and the early initiation of therapeutic sessions. REFERENCES Bernet W. Child maltreatment. In: Sadock BJ, Sadock VA, Ruiz P, eds. Kaplan & Sadock’s Comprehensive Textbook of Psychiatry. 9th ed. Vol. 2. Philadelphia: Lippincott Williams & Wilkins; 2009:3792. Carr CP, Severei CM, Stingel AM, Lemgruber VB, Juruena MF. The role of early life stress in adult psychiatric disorders. A systematic review according to childhood trauma subtypes. J Nerv Ment Dis. 2013;201:1007–1020. Centers for Disease Control and Prevention, National Center for Injury Prevention and Control, Division of Violence Prevention, Preventing Child Maltreatment Through the Promotion of Safe, Stable, and Nurturing Relationships Between Children and Caregivers. (January 18, 2013.) Coelho R, Viola TW, Walss-Bass C, Brietzke E, Grassi-Olveira R. Childhood maltreatment and inflammatory markers: A systematic review. Acta Psychiatr Scand. 2013. Cummings M, Berkowitz SJ. Evaluation and treatment of childhood physical abuse and neglect: A review. Curr Psychiatry Rep. 2014;16:429–439. Currie J, Widom CS. Long-term consequences of child abuse and neglect on adult economic well-being. Child Maltreatment. 2010;15:111–120. Heyman RE, Smith Slep AM. Creating and field-testing diagnostic criteria for partner and child maltreatment. J Fam Psychol. 2006;20:397. Hinkdley N, Ramchandani PG, Jones DP. Risk factors for recurrence of maltreatment: A systematic review. Arch Dis Child. 2006;91:744. Hussey JM, Chang JJ, Kotch JB. Child maltreatment in the United States: Prevalence, risk factors and adolescent health consequences. Pediatrics. 2006;118:933. Norman RE, Byambaa M, De R, Butchart A, Scott J, Vos T. The long-term health consequences of child physical abuse, emotional abuse, and neglect: A systematic review and meta-analysis. PLoS Med. 2012. Runyon MK, Deblinger E, Steer R. Comparison of combined parent-child and parent-only cognitive-behavioral treatments for offending parents and children in cases of child physical abuse. Child Family Behav Ther. 2010;32:196–218. Runyon MK, Deblinger E, Schoreder C. Pilot evaluation of outcomes of combined parent-child cognitive-behavioral therapy group for families at risk for child physical abuse. Cogn Behav Prac. 2009;16:101–118. Swenson CC, Schaeffer CM, Henggler SW, Faldowski R, Mayhew AM. Multisystemic therapy for child abuse and neglect: A randomized effectiveness trial. J Fam Psychol. 2010;24:497–507. Teicher MH, Samson JA, Polcari A, McGreenery CE. Sticks, stones, and hurtful words: Relative effects of various forms of maltreatment. Am J Psychiatry. 2006;163:993. 54 - 31.19d Impact of Terrorism on Children 31.19d Impact of Terrorism on Children Teicher MH, Tomoda A, Andersen SL. Neurobiological consequences of early stress and childhood maltreatment: are results from human and animal studies comparable? Ann N Y Acad Sci. 2006;1071:313. Widom CS, Brzustowicz LM. MAOA and the “cycle of violence:” Childhood abuse and neglect, MAOA genotype, and risk for violent and antisocial behavior. Biol Psychiatry. 2006;60:684. Wilson KR, Hansen DJ, Li M. The traumatic response in child maltreatment and resultant neuropsychological effects. Aggress Violent Behav. 2011;16:87–97. Young SE, Smolen A, Hewitt JK, Haberstick BC, Stallings MC, Corley RP, Crowley TJ. Interaction between MAO-A genotype and maltreatment in the risk for conduct disorder: Failure to confirm in adolescent patients. Am J Psychiatry. 2006;163:951. 31.19d Impact of Terrorism on Children In recent years, exposure to mass trauma and terrorism has become an increasing concern regarding the well-being of youth. Mass trauma has occurred directly and by witness through highly publicized traumatic events globally and in the United States, pertaining to terrorism, war, mass killings, and natural disasters. On April 15, 2013, the first major terrorist attack in the United States since 9/11 occurred at the finish line of the Boston Marathon in the mid-afternoon. Two “improvised explosive devices” (IEDs), that is, homemade bombs, detonated 8 minutes apart in the middle of a densely packed crowd of thousands of marathon runners and bystanders, killing three people and injuring about 264 others. Within moments after the blasts, the crowd’s panic and chaos turned to purposeful attention to help others get to emergency medical teams arriving on the scene. Courageously, bystanders ran toward others to give aid rather than dispersing away from the scene in all directions. Runners tore off their own shirts to apply pressure to fellow runners who were bleeding, or to use them as tourniquets. Boston’s emergency response medical teams worked quickly, efficiently, and tirelessly to transport injured runners to hospitals and into operating rooms in order to save limbs, and stop bleeding. The fact that almost all the injured were saved is a tribute to the emergency preparedness and collaboration of law enforcement, medical, and surgical teams carrying out an emergency plan that they had previously been briefed on, as a matter of course. Additional situations in which youth are exposed to severe trauma and terror involve armed conflict around the world, multiple mass school shootings that have taken place across the United States in recent years, and hurricanes, devastating storms, and tsunamis. Of course, more than a decade ago, the youth in the United States experienced the large-scale domestic terrorist attack on September 11, 2001, on the World Trade Center in New York City and the Pentagon in Washington, DC. There is an increasing body of literature on the impact of terrorism on children as well as a variety of other forms of trauma. One predominant and near universal symptom in children in response to these stimuli is anxiety. Young children may cling excessively to their parents, whereas older children may become preoccupied with fear about unrelated issues. Some youth express overt anger, and others experience a sense of hopelessness, lack of control, and/or depression. Severe traumas, such as experiencing a terrorist event, may be more likely to result in posttraumatic stress syndromes among exposed youth, compared to less severe forms of trauma. The number of traumas experienced by a child, degree of family support provided after the exposure, and the reactions of parents are all important factors in a child’s reaction. According to a national survey after the terrorist attack of September 11th, stress reactions to that disaster were increased by watching repeated media coverage of those events. A similar study evaluating the impact of media versus direct exposure to collective trauma, on acute stress response was undertaken 2 to 4 weeks after the Boston Marathon bombings through surveying 846 people from Boston, 941 people from New York City, and 2,888 people through Internet means. Direct exposure, defined as being at or near the bombings, was compared to media exposure, including footage on television and bombing related stories on the radio, in print, online, and other social media coverage. Because acute stress responses appear within weeks of a traumatic event, this study was able to capture acute stress difference between the two groups. The study found that trauma related to media exposure was associated with acute stress reactions in people from all over the United States who were not directly exposed to the event in Boston. Furthermore, respondents reporting exposure to media coverage of the bombings for six or more hours daily in the week following the bombings were nine times more likely to report high acute stress than those who had minimal exposure to media coverage of the events. In fact, the group who engaged in extensive media coverage had higher levels of acute stress than respondents who had direct exposure in Boston, but who had minimal exposure to media coverage of the bombings. These findings suggest that prolonged media exposure to collective traumatic events may have a strong negative impact on psychological symptoms and acute stress syndromes. However, the study noted substantial resilience in the surveyed population. Researchers have suggested that the effectiveness of Boston’s medical and law enforcement teams in response to the terrorist bombings may have promoted some degree of resilience in the population. A unique aspect of exposure to terrorist-related trauma, as well as school shootings, is the psychological effects of knowing that the trauma was both consciously and purposely perpetrated, and yet also random. The random nature of terrorist attacks appears to lead to especially adverse reactions in children. School shootings are among the most tragic of traumatic events involving youth. On December 14, 2012, in the village of Sandy Hook, in Newtown, Connecticut, a 20-year-old male in black clothing carrying his mother’s rifle, shot his way through a glass window at the front of the Sandy Hook Elementary School, rampaged the school, shooting and killing 20 first-grade students from multiple classrooms and 6 school personnel, and then shot and killed himself. He had shot and killed his mother before arriving at the school. The psychological impact of this massacre on children who survived is moderated by age, gender, and family reactions. Younger children appear to be at higher risk for posttraumatic stress disorder, somatic symptoms, depression, and distress than older children and adolescents. Gender also has been found to influence behavioral symptoms after exposure to severe trauma or terrorism, with girls experiencing higher levels of posttraumatic stress syndromes and depression, while boys exhibit more external behavior problems. Although the United States has launched a series of initiatives in response to the threats and consequences of terrorism in the form of an act of Congress in 2002 called the Public Health Security and Bioterrorism Preparedness and Response Act, children and adolescents continue to view media exposure to terrorist events throughout the world that reinforces a sense of danger. The concept of terrorist acts is characterized by three distinct features: (1) They produce a societal atmosphere of extreme danger and fear, (2) they inflict significant personal harm and destruction, and (3) they undermine the expectation of citizens that the state is able to protect them. Child and adolescent reactions to exposure to terrorism are mediated by numerous factors, including personal appraisal of persisting danger, the likelihood of recurrent attack, and the perception of the relative safety of one’s family and close friends. Children’s responses to terrorist exposure are influenced by how their parents cope with the trauma and resulting turmoil and how well they understand the situation. PTSD has been studied in adolescents, with and without learning disabilities, who have been exposed to terror attacks. Findings from this study revealed that personal exposure to terror, past personal life-threatening events, and history of anxiety all contributed to the development of posttraumatic stress reactions. In addition, adolescents with learning disabilities who had difficulties in cognitively processing the traumatic events were at higher risk of developing PTSD when this was combined with the other high risk factors, such as being personally exposed to the traumatic events. Table 31.19d-1 identifies the relationship between objective features of danger and subjective features related to exposure to terrorist acts. Table 31.19d-1 Experience of Danger Consequent to Terrorist Acts The following summarizes data collected after the terrorist attack of the World Trade Center on September 11, 2001. SEPTEMBER 11, 2001 ATTACKS The U.S. Department of Education, through Project SERV, supported the New York City Board of Education in conducting a needs assessment of New York City schoolchildren. A total of 8,000 randomly selected students were surveyed 6 months after the September 11, 2001 attacks. Striking differences were seen among students in the vicinity of Ground Zero as compared with students in the rest of the city, in exposure to smoke and dust, fleeing for safety, problems getting home, and smelling smoke in the days and weeks after September 11. Approximately 70 percent of all children, however, were exposed to one of these factors. Interpersonal exposure through direct victimization of a family member was greater among children attending schools outside the Ground Zero vicinity as compared with those attending school in this area. Media exposure was extensive and prolonged. Signs of heightened security were visible throughout the city. The study used several scales of the Diagnostic Interview Schedule for Children (DISC). Three sets of findings stand out from this study. First a significant degree of persistent separation anxiety was seen, especially among school-age children, but also among adolescents. Second, reflecting an age-related vulnerability to incident-specific new fears (e.g., subways and buses) and avoidant behavior of school-age children, a nearly 25 percent rate of agoraphobia was reported among 4th- and 5th-graders. Care must be taken, however, not to misrepresent incident-specific new fears as agoraphobia, because the course of recovery and intervention strategies may differ. Third, an enormous reservoir of prior traumatic experiences (more than one half of the total sample) was associated with severity of current PTSD symptoms, emphasizing the need to attend to prior trauma in conducting needs assessments, surveillance, and intervention strategies. Other risk factors, in addition to younger age, included female gender and Hispanic ethnicity. The finding of age-related increases in rates of conduct disorder also needs to be interpreted in light of adolescent response to an ecology of danger in which overly aggressive, reckless, and risk-taking behaviors are well documented and associated with posttraumatic stress reactions. A major strength of this study was the inclusion of selfreported impairment as well as symptoms, setting an important standard for future studies. J. Stuber and colleagues conducted a telephone survey of a random sample of adult residents of Manhattan 1 to 2 months after the September 11th attacks. The sample included more than 100 parents who were asked to describe the experiences and reactions of their children. Not surprisingly, given the time of the incident, most children were at school or day care when the disaster occurred. Many of the parents recalled concern about their children’s safety at the time, and most were not reunited with their children for more than 4 hours. More than 20 percent of the parents studied reported that their children had received counseling related to the disaster. Receiving counseling was associated with male gender, parental posttraumatic stress, and having at least one sibling living in the household. Also using parent report in a New York City telephone survey, researchers assessed predictors of posttraumatic stress reactions in children between the ages of 4 and 17 years, 4 to 5 months after the attacks. Almost 20 percent of children were reported by their parents to have experienced severe or very severe posttraumatic stress reactions, and approximately two thirds had moderate posttraumatic stress reactions. Parental reactions and viewing three or more graphic images of the disaster on television were associated with severe or very severe posttraumatic stress reactions in children. Another study reported that 27 percent of children with severe or very severe posttraumatic stress reactions received some mental health care 4 to 5 months after September 11th. Two surveys of representative samples of adults were conducted after the September 11th attacks; the first between 4 and 5 months and the second between 6 and 9 months after the attacks. Behavior problems were related to the child’s race or ethnicity, family income, living in a single-parent household, disaster event experiences, and parental reactions to the attacks. The results of these surveys were examined in light of findings from a representative survey conducted before September 11th. The rate of behavior problems was lower in the first post-September 11th survey (4 to 6 months after the attacks) than rates in the pre-September 11th survey, but problems returned to preSeptember 11th levels by the second post-September 11th study (6 to 9 months after the attacks). Consistent with findings in studies of Hurricane Andrew, these results suggest that behavior problems may decrease in the months after a disaster or that parents may be insensitive to them, but that they return to pre-disaster levels over time. Media coverage of the September 11th attacks brought renewed debate about its impact, especially on children, even children with no direct exposure. One study reported extensive exposure to television coverage in children throughout the nation, using a representative survey of adults conducted in the first days after the attacks. Approximately one third of the parents surveyed attempted to limit or to prevent their children’s viewing, but, among those whose parents made no attempt to restrict viewing, the number of hours of disaster coverage watched was related to the number of reported stress symptoms. Using a Web-based, nationally representative sample of adults, another study examined distress in children 1 to 2 months after the attacks by asking parents if their children were upset by the events. Among the children perceived as most upset, 20 percent had trouble sleeping, 30 percent were irritable or easily upset, and 27 percent feared separation from their parents. The mean age of children perceived as most upset was 11 years, with no statistically significant gender differences. The proportion of parents reporting at least one child upset did not differ by community in analysis of data from the New York City metropolitan area, Washington, DC, other major metropolitan areas, and the rest of the country. A strength of these surveys was their examination of representative samples, but earlier work points to concern about assessing children by interviewing their parents. Furthermore, as with the Oklahoma City studies, the samples were composed mainly of indirectly exposed children, and the clinical significance of the findings is unclear. Nine-year-old Jason endured the traumatic loss of his father on the first plane into the World Trade Center. Jason’s father was on board American Airlines Flight No. 11 on a business trip. Jason and his siblings were preparing to leave for school when he, his mother and his siblings learned of the event. Jason watched his mother nearly collapse when she confirmed the presence of his father aboard the aircraft. Jason observed the recurring video segments of the second plane crashing into the second tower several times that morning before his mother limited television access. Jason, the oldest child in his family, had enjoyed an exceptionally close relationship with his father. Almost immediately after the terrorist attacks, Jason’s mother became worried that he was despondent, suicidal and unable to function, just preoccupied with the grisly nature of his father’s death. He was becoming increasingly agitated as he talked constantly about the gruesome way that his father had died. Jason’s mother sought immediate psychological treatment for him, during which he began to ask a continual series of questions about his father’s death, including aspects of burning, fragmentation, pain, blood, and the exact moment of his father’s deaths in comparison with what he had initially observed on television. This became the main theme of Jason’s early treatment, in which he ruminated (i.e., whether this father had been “blown up in a thousand pieces” and the sequence of fire, burning, pain, and death). Jason developed nightmares within days in which he awakened and called for his mother at least three times a night. Jason was unwilling to discuss the content of his dreams with his mother, given his observations of her own serious distress. Jason began to express fears that the “hijackers” would hurt his mother and siblings. He became focused on the concept that “half our freedom is gone,” and he was concerned that one half of New York City was destroyed. He was preoccupied with enacting in play, repetitive crashing down of creating the World Trade Center. Although after 3 months, he was able to resume sleeping through the night, he reported new troubling dreams with themes of ghosts “popping out” and “everyone is killed, and then I’m killed.” This worsened after the onset of the war in Afghanistan, and his mother had to constantly reassure him that the war was not near their home. Jason told his therapist of his wish that could find a time machine and be transported back in time on board his father’s flight before it crashed. While his therapist could fly the plane, he would overpower the “hijackers” and throw them off the plane, and then the plane would land safely in Boston. Jason continued his wish that after landing, his father and the other passengers tell him “thank you,” and be very happy. After expressing his wish verbally, he appeared to be somewhat comforted and he began to recall many positive activities with his father, and a series of happy, highly detailed memories of his father, which then caused him to suddenly become tearful with profound sadness at the realization that these would be no more. In therapy, Jason alternately expressed rage and anger and confusion about the actions of “Osama Bin Laden.” Over many months, Jason was able to remember and speak about the good things he remembered about his father without immediately breaking down in tears. Jason become a helpful big brother, who often tried to care for his younger siblings, and his mother often told him how proud she was of him. (Adapted from Robert S. Pynoos, M.D. M.P.H., Merritt D. Schreiber, Ph.D., Alan M. Steinberg Ph.D., Betty Pfefferbaum, M.D., and J.D.) To respond to the mental health needs of children and adolescents who have been exposed to terrorism either through personal experience or through exposure to media depicting world-wide terrorism, the adverse psychological reactions listed in Table 31.19d-2 must be considered. Table 31.19d-2 Psychological Disorders Associated with Terrorism COMPONENTS OF MECHANISMS FOR RECOVERY FROM EXPOSURE TO TERRORISM In order to begin the process of recovery from exposure to mass trauma, an assessment of a child’s current coping must be done. Numerous instruments to measure coping exist. These include COPE, a self-report questionnaire which has 52 items that can be used with children, adolescents and adults; Children’s Coping Strategies Checklist (CCSC), a self-report questionnaire with 45 general coping items used with children 9 to 13 years of age; and How I Coped Under Pressure (HICIPS), which has 45 event specific questions for children in the 4th to 6th grade. Once this assessment has been determined, the next steps can be taken to begin the road to recovery. Perception of Safety The notion of perceived safety is an important protective factor as well as a component of recovery for a child, adolescent, or adult who has been exposed to terrorism. A recent report of symptoms of PTSD, depression, and perceived safety in disaster workers 2 weeks after the September 11th terrorist attacks found that lower perceived safety was associated with increased symptoms of hyperarousal and intrusive fearful thoughts, but not avoidance. An expected diminished sense of safety was found in those individuals who had personally been in greater physical danger, or who had worked with dead bodies compared with others who were physically less exposed. To regain a sense of security, reestablishment of a perception of safety is a necessary first step. Reestablishment or Maintenance of Daily Routines Although it is clearly not always possible to maintain usual daily routines amidst war or exposure to terrorism, a study of Israeli adolescents found that those whose families were able to maintain their usual activities, such as attending school and family functions, were at lower risk for the development of posttraumatic reactions. Proactive Interventions to Enhance Resilience Perceived personal resilience has been shown to be protective against symptoms of posttraumatic stress development. 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