Rett Syndrome
Rett Syndrome Alan K. Percy Rett syndrome is a pervasive developmental disorder affecting predominantly females. After a period of apparently normal development, affected children experience a plateau in development and…
Leukodystrophies
Leukodystrophies Alan K. Percy The leukodystrophies are a group of progressive inherited neurodegenerative disorders affecting myelin formation in the central and (in some instances) peripheral nervous systems. As such, they…
Metabolic Encephalopathies
Metabolic Encephalopathies Edward R. B. McCabe Many of the metabolic disorders include among their clinical features abnormalities of the central nervous system (CNS). A significant portion of these disorders can…
Slow Virus Infections and Transmissible Spongiform Encephalopathies of the Central Nervous System
Slow Virus Infections and Transmissible Spongiform Encephalopathies of the Central Nervous System William J. Britt Transmissible central nervous system (CNS) diseases with unusually long incubation periods and prolonged clinical syndromes…
Hereditary and Acquired Types of Myopathy
Hereditary and Acquired Types of Myopathy Darryl C. De Vivo Salvatore DiMauro Molecular genetics has revolutionized our current understanding of neuromuscular diseases. The inherited conditions embrace all patterns of heredity,…
Diseases of the Neuromuscular Junction
Diseases of the Neuromuscular Junction Julie Thorne Parke Numerous conditions may interfere with the transmission of the electrical impulse across the neuromuscular junctions, which consist of the terminal portion of…
Peripheral Neuropathies
Peripheral Neuropathies Julie Thorne Parke Involvement of the peripheral nerves may occur in a variety of different disorders, including systemic diseases, infections, and poisonings. In addition, degeneration of the peripheral…
Disorders of the Anterior Horn Cell
Disorders of the Anterior Horn Cell Julie Thorne Parke The anterior horn cells may be involved selectively in a number of acquired and inherited diseases. Certain viruses, particularly poliomyelitis, demonstrate…
Reye Syndrome
Reye Syndrome Penelope Terhune Louis Reye syndrome, first described in 1963, is an acute, life-threatening, postinfectious, metabolic encephalopathy that affects predominantly school-aged children, occasionally infants, and rarely adults. Over the…
The Comatose Child
The Comatose Child James Owens Daniel G. Glaze DEFINITION AND PATHOPHYSIOLOGY Coma is not a specific disorder, but a sign of central nervous system (CNS) dysfunction. It may be caused…
