Akademik

spongiform encephalopathy n any of a group of degenerative diseases of the brain characterized by the development of spongiform lesions and by deterioration in neurological functioning see BOVINE SPONGIFORM ENCEPHALOPATHY, TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY

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).