Cerebral palsy

Cerebral palsy

Cerebral palsy (CP) is caused by a non-progressive insult to the developing brain in the perinatal period; in most cases only risk factors, such as prematurity , rather than specific causes, such as hypoxia (hypoxic ischaemic encephalopathy [HIE]), can be identified. The e ff ects of CP may only become apparent as the child grows and fails to reach developmental milestones. Investigations may identify the aetiology and predict the pattern of the CP: premature babies may show evidence of periventricular leukomalacia (PVL) on MRI, associated with a spastic diplegia and relative preservation of intellectual function. , 1806–1875, neurologist, worked successively in Boulogne and Paris, France, but never

Aim of treatment Restores joint range (but results in relative muscle weakness) correction of /f_i xed deformity Restores mechanical alignment and allows muscles to work in a more ef /f_i cient manner Improves posture/function; reduces pain Reduce spasticity – not useful in dystonia Improve lower limb mechanics

/uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF In general, the pattern of involvement can be classified according to the anatomical site involved and the e ff ect on muscle tone ( Table 44.16 ). The prognosis for walking can be predicted by identifying evidence of neurological develop ment, i.e. gaining motor skills and losing primitive reflexes. The age-related Gross Motor Function Classification System (GMFCS) has five categories that relate to mobility and prog nosis (GMFCS /uni00A0 I – near-normal versus GMFCS /uni00A0 V – wheel chair based). Many children with GMFCS /uni00A0 V CP have multiple associated problems and a short life expectancy . An important aspect of management is the control of high tone. Tone can be reduced with drugs (e.g. diazepam and baclofen). Alternatively , neuromuscular blockers such as botulinum to xin allow a focal reduction in tone by preventing acetylcholine release at the neuromuscular junction. The e ff ect is temporary , giving a ‘window’ during which the physio therapists can stretch agonists and strengthen antagonists. It is important to di ff erentiate between dynamic and fixed contractures; the latter will not respond to tone management or splinting. The classic CP gait patterns demonstrate flexor spasticity The child with spastic diplegia has problems at all levels of the lower limb. Single-event multilevel sur gery (SEMLS) is pop ular, and gait analysis (both observational and computerised) contributes to the selection of an appropriate management plan for an individual patient. Computerised analysis provides objective evidence of joint movement and mechanics in mul tiple planes ( Figure 44.37 ). Appropriate bone and soft-tissue procedures can then be planned. Botulinum toxin helps with postoperative pain and spasm. In the child with total body involvement (TBI) and high muscle tone, hip subluxation leading to dislocation is common ( Figur e 44.38 ). Current thinking is that symmetry and pelvic position are important so the hips should be kept in joint with early surgical intervention if necessary . Aggressive management of a spinal deformity initially concentrates on seating position and subsequently emphasises spinal bracing or surgery . Overall, it is important to remember that, in adulthood, independent mobility , even if in a wheelchair, and e ff ective communication are the most important requirements. - Summary box 44.20 - - Cerebral palsy /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF -

Characteristics Tone Commonest type of abnormality; due to pyramidal system damage Spastic (‘high’) Velocity-dependent increased muscle tone and brisk re /f_l exes Dyskinetic Dystonic Increased tone but reduced activity – stiff movements Choreoathetoid Low tone but increased activity – uncoordinated jerky movements Due to damage in the extrapyramidal system Generalised low tone, loss of muscle coordination Ataxia Due to cerebellar damage No one tone/movement disorder predominatesw Mixed Combination of spasticity and dystonia is common Hypotonia Usually a phase (which may last years) before the features of spasticity develop Site Unilateral Hemiplegia Arm more affected than leg Bilateral Diplegia Legs more affected than arms Total body involvement Often signi /f_i cant intellectual impairment and associated dif /f_i culties Brain injury is non-progressive Classi /f_i ed as unilateral or bilateral involvement: hemiplegia, diplegia or TBI Tone may be high, low or variable but there is always a generalised, relative muscle weakness In ambulant children, gait analysis may be used to plan management In TBI, primary concerns are hip subluxation and spinal deformity

Cerebral palsy

Cerebral palsy (CP) is caused by a non-progressive insult to the developing brain in the perinatal period; in most cases only risk factors, such as prematurity , rather than specific causes, such as hypoxia (hypoxic ischaemic encephalopathy [HIE]), can be identified. The e ff ects of CP may only become apparent as the child grows and fails to reach developmental milestones. Investigations may identify the aetiology and predict the pattern of the CP: premature babies may show evidence of periventricular leukomalacia (PVL) on MRI, associated with a spastic diplegia and relative preservation of intellectual function. , 1806–1875, neurologist, worked successively in Boulogne and Paris, France, but never

Aim of treatment Restores joint range (but results in relative muscle weakness) correction of /f_i xed deformity Restores mechanical alignment and allows muscles to work in a more ef /f_i cient manner Improves posture/function; reduces pain Reduce spasticity – not useful in dystonia Improve lower limb mechanics

/uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF In general, the pattern of involvement can be classified according to the anatomical site involved and the e ff ect on muscle tone ( Table 44.16 ). The prognosis for walking can be predicted by identifying evidence of neurological develop ment, i.e. gaining motor skills and losing primitive reflexes. The age-related Gross Motor Function Classification System (GMFCS) has five categories that relate to mobility and prog nosis (GMFCS /uni00A0 I – near-normal versus GMFCS /uni00A0 V – wheel chair based). Many children with GMFCS /uni00A0 V CP have multiple associated problems and a short life expectancy . An important aspect of management is the control of high tone. Tone can be reduced with drugs (e.g. diazepam and baclofen). Alternatively , neuromuscular blockers such as botulinum to xin allow a focal reduction in tone by preventing acetylcholine release at the neuromuscular junction. The e ff ect is temporary , giving a ‘window’ during which the physio therapists can stretch agonists and strengthen antagonists. It is important to di ff erentiate between dynamic and fixed contractures; the latter will not respond to tone management or splinting. The classic CP gait patterns demonstrate flexor spasticity The child with spastic diplegia has problems at all levels of the lower limb. Single-event multilevel sur gery (SEMLS) is pop ular, and gait analysis (both observational and computerised) contributes to the selection of an appropriate management plan for an individual patient. Computerised analysis provides objective evidence of joint movement and mechanics in mul tiple planes ( Figure 44.37 ). Appropriate bone and soft-tissue procedures can then be planned. Botulinum toxin helps with postoperative pain and spasm. In the child with total body involvement (TBI) and high muscle tone, hip subluxation leading to dislocation is common ( Figur e 44.38 ). Current thinking is that symmetry and pelvic position are important so the hips should be kept in joint with early surgical intervention if necessary . Aggressive management of a spinal deformity initially concentrates on seating position and subsequently emphasises spinal bracing or surgery . Overall, it is important to remember that, in adulthood, independent mobility , even if in a wheelchair, and e ff ective communication are the most important requirements. - Summary box 44.20 - - Cerebral palsy /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF -

Characteristics Tone Commonest type of abnormality; due to pyramidal system damage Spastic (‘high’) Velocity-dependent increased muscle tone and brisk re /f_l exes Dyskinetic Dystonic Increased tone but reduced activity – stiff movements Choreoathetoid Low tone but increased activity – uncoordinated jerky movements Due to damage in the extrapyramidal system Generalised low tone, loss of muscle coordination Ataxia Due to cerebellar damage No one tone/movement disorder predominatesw Mixed Combination of spasticity and dystonia is common Hypotonia Usually a phase (which may last years) before the features of spasticity develop Site Unilateral Hemiplegia Arm more affected than leg Bilateral Diplegia Legs more affected than arms Total body involvement Often signi /f_i cant intellectual impairment and associated dif /f_i culties Brain injury is non-progressive Classi /f_i ed as unilateral or bilateral involvement: hemiplegia, diplegia or TBI Tone may be high, low or variable but there is always a generalised, relative muscle weakness In ambulant children, gait analysis may be used to plan management In TBI, primary concerns are hip subluxation and spinal deformity

Cerebral palsy

Cerebral palsy (CP) is caused by a non-progressive insult to the developing brain in the perinatal period; in most cases only risk factors, such as prematurity , rather than specific causes, such as hypoxia (hypoxic ischaemic encephalopathy [HIE]), can be identified. The e ff ects of CP may only become apparent as the child grows and fails to reach developmental milestones. Investigations may identify the aetiology and predict the pattern of the CP: premature babies may show evidence of periventricular leukomalacia (PVL) on MRI, associated with a spastic diplegia and relative preservation of intellectual function. , 1806–1875, neurologist, worked successively in Boulogne and Paris, France, but never

Aim of treatment Restores joint range (but results in relative muscle weakness) correction of /f_i xed deformity Restores mechanical alignment and allows muscles to work in a more ef /f_i cient manner Improves posture/function; reduces pain Reduce spasticity – not useful in dystonia Improve lower limb mechanics

/uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF In general, the pattern of involvement can be classified according to the anatomical site involved and the e ff ect on muscle tone ( Table 44.16 ). The prognosis for walking can be predicted by identifying evidence of neurological develop ment, i.e. gaining motor skills and losing primitive reflexes. The age-related Gross Motor Function Classification System (GMFCS) has five categories that relate to mobility and prog nosis (GMFCS /uni00A0 I – near-normal versus GMFCS /uni00A0 V – wheel chair based). Many children with GMFCS /uni00A0 V CP have multiple associated problems and a short life expectancy . An important aspect of management is the control of high tone. Tone can be reduced with drugs (e.g. diazepam and baclofen). Alternatively , neuromuscular blockers such as botulinum to xin allow a focal reduction in tone by preventing acetylcholine release at the neuromuscular junction. The e ff ect is temporary , giving a ‘window’ during which the physio therapists can stretch agonists and strengthen antagonists. It is important to di ff erentiate between dynamic and fixed contractures; the latter will not respond to tone management or splinting. The classic CP gait patterns demonstrate flexor spasticity The child with spastic diplegia has problems at all levels of the lower limb. Single-event multilevel sur gery (SEMLS) is pop ular, and gait analysis (both observational and computerised) contributes to the selection of an appropriate management plan for an individual patient. Computerised analysis provides objective evidence of joint movement and mechanics in mul tiple planes ( Figure 44.37 ). Appropriate bone and soft-tissue procedures can then be planned. Botulinum toxin helps with postoperative pain and spasm. In the child with total body involvement (TBI) and high muscle tone, hip subluxation leading to dislocation is common ( Figur e 44.38 ). Current thinking is that symmetry and pelvic position are important so the hips should be kept in joint with early surgical intervention if necessary . Aggressive management of a spinal deformity initially concentrates on seating position and subsequently emphasises spinal bracing or surgery . Overall, it is important to remember that, in adulthood, independent mobility , even if in a wheelchair, and e ff ective communication are the most important requirements. - Summary box 44.20 - - Cerebral palsy /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF -

Characteristics Tone Commonest type of abnormality; due to pyramidal system damage Spastic (‘high’) Velocity-dependent increased muscle tone and brisk re /f_l exes Dyskinetic Dystonic Increased tone but reduced activity – stiff movements Choreoathetoid Low tone but increased activity – uncoordinated jerky movements Due to damage in the extrapyramidal system Generalised low tone, loss of muscle coordination Ataxia Due to cerebellar damage No one tone/movement disorder predominatesw Mixed Combination of spasticity and dystonia is common Hypotonia Usually a phase (which may last years) before the features of spasticity develop Site Unilateral Hemiplegia Arm more affected than leg Bilateral Diplegia Legs more affected than arms Total body involvement Often signi /f_i cant intellectual impairment and associated dif /f_i culties Brain injury is non-progressive Classi /f_i ed as unilateral or bilateral involvement: hemiplegia, diplegia or TBI Tone may be high, low or variable but there is always a generalised, relative muscle weakness In ambulant children, gait analysis may be used to plan management In TBI, primary concerns are hip subluxation and spinal deformity