spongiform encephalopathy (spunji-form) n. any one of a group of rapidly progressive degenerative neurological diseases that include scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle, and Creutzfeldt-Jakob disease in humans. Human spongiform encephalopathies are characterized by rapidly progressive dementia associated with myoclonic jerks (see myoclonus). The diseases are thought to be caused by abnormal transmissible proteins (see prion).
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