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Yet, the conditions represent collectively over onefourth of all admissions to pediatric neurology services.<\/jats:p><jats:p>Five\u2010year samples of admission characteristics of 1218 patients from two medical centers over twenty\u2010two years permit an estimate of the frequency of the neurodegenerative diseases. The six most\u2010encountered diagnoses, in declining order, were: subacute sclerosing panencephalitis; neuronal ceroid lipofuscinosis; tuberous sclerosis with degeneration; West disease, or idiopathic degenerative encephalopathy associated with infantile spasms; Werdnig\u2010Hoffmann disease, and hereditary spastic paraplegia.<\/jats:p><jats:p>A classification is offered grouping the neurodegenerative disorders into five major categories: polioencephalopathies, leukoencephalopathies, corencephalopathies, spinocerebellopathies, and diffuse encephalopathies. Disorders in each subgroup may be either genetic or nongenetic. Neurodegenerative diseases have multiple causes, including metabolic, viral, immunopathic, environmental, and epileptogenic. 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