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24.16 Diseases of the peripheral nerves 6176 Robert D.M. Hadden ESSENTIALS Disorders of peripheral nerve function can be categorized in terms of the site of the primary disturbance. (1) Neuronopathies (sensory or autonomic neuronopathies are also known as gang...

24.17 Inherited neurodegenerative diseases 6197 Swati Sathe ESSENTIALS Many disorders of the nervous system, especially the degenerative conditions, have a genetic basis, which is usually due to a mutated gene resulting in decreased production of a critical st...

24.18 Disorders of the neuromuscular junction 6295 David Hilton- Jones and Jacqueline Palace ESSENTIALS Two fundamentally different pathological processes are associated with disease at the neuromuscular junction: (1) acquired disorders in which autoantibodies...

24.19 Disorders of muscle 6304 24.19.1 Structure and function of muscle 6304 Michael G. Hanna and Enrico Bugiardini 24.19 Disorders of muscle CONTENTS 24.19.1 Structure and function of muscle  6304 Michael G. Hanna and Enrico Bugiardini 24.19.2 Muscular dyst...

24.19.2 Muscular dystrophy 6310 Kate Bushby and Chiara Marini- Bettolo section 24  Neurological disorders 6310 FURTHER READING Hughes BW, Kusner LL, Kaminski HJ (2006). Molecular architecture of the neuromuscular junction. Muscle Nerve, 33, 445–​61. Huxley HE,...

24.19.3 Myotonia 6328 David Hilton- Jones section 24  Neurological disorders 6328 entering clinical trials. Gene transfer experiments in animal models have proved the general feasibility of this approach to these genetic diseases, at least on a small scale. Mo...

24.19.5 Mitochondrial disease 6343 Patrick F. Chinnery and D.M. Turnbull 24.19.5  Mitochondrial disease 6343 is failure of substrate utilization or supply when energy demands increase during exercise or starvation. In other disorders, there is disruption of th...

24.2 Mind and brain: Building bridges between neurology, psychiatry, and psychology 5778 Adam Zeman ‘The great regions of the mind correspond to the great regions of the brain’ Paul Broca ‘ . . . the master unsolved problem of biology: how the hundred million ...

24.21 Acquired metabolic disorders and the nervous system 6368 Neil Scolding ESSENTIALS Many systemic disorders of metabolism have neurological conse- quences. Conditions of particular note include the following: Major organ diseases Anoxic brain damage—​cardi...

24.23 Paraneoplastic neurological syndromes 6384 Jeremy Rees ESSENTIALS Paraneoplastic neurological syndromes are disorders caused by the presence of an underlying tumour, but not due to either direct or metastatic invasion, or to recognized metabolic or endoc...

24.24 Autoimmune encephalitis and Morvan’s syndrome 6393 Camilla Buckley and Angela Vincent ESSENTIALS Autoimmune limbic encephalitis—​typical presentation is with acute or subacute onset of short-​term memory loss, seizures, and dis- orientation. MRI characte...

24.3 Clinical investigation of neurological disease 5781 24.3.1 Lumbar puncture 5781 R. Rhys Davies and Andrew J. Larner

24.3.1 Lumbar puncture

24.3.2 Electrophysiology of the central and peripheral nervous systems 5785 Christian Krarup

24.3.3 Imaging in neurological diseases 5802 Andrew J. Molyneux, Shelley Renowden, and Marcus Bradley

24.3.4 Investigation of central motor pathways: Magnetic brain stimulation 5817 K.R. Mills 24.3.4  Investigation of central motor pathways: Magnetic brain stimulation 5817 represent a major revolution in the management of patients with aneurysmal SAH and other...

24.4 Higher cerebral function 5821 24.4.1 Disturbances of higher cerebral function 5821 Peter J. Nestor CONTENTS 24.4.1 Disturbances of higher cerebral function  5821 Peter J. Nestor 24.4.2 Alzheimer’s disease and other dementias  5830 Jonathan M. Schott 24....

24.4.2 Alzheimer’s disease and other dementias 5830 Jonathan M. Schott SECTION 24  Neurological disorders 5830 frontal cortex. Although devastating in their effects on social func- tion, such lesions are notoriously difficult to detect using standard psychomet...