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spongiform encephalopathy


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Any one of a group of rapidly progressive degenerative neurological diseases that include scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle, and kuru, Creutzfeldt-Jakob disease, and Gerstmann-Straussler-Scheinker syndrome in humans. In humans the spongiform encephalopathies are characterized by rapidly progressive dementia associated with myoclonic jerks (see myoclonus); on pathological examination the brains of affected individuals show a characteristic cystic degeneration. The diseases are thought to be caused by unconventional transmissible agents (see prion).

Subjects: Medicine and Health.


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