prion disease

any of a group of fatal, transmissible neurodegenerative diseases caused by abnormalities of prion protein metabolism, which may result from mutations in the prion protein gene or from infection with pathogenic isoforms of the protein (see prion). Characteristics include neuronal loss, gliosis, and extensive vacuolation of the cerebral cortex. Prion diseases may be sporadic, inherited as an autosomal dominant trait, or acquired. Human diseases include Creutzfeldt-Jakob disease, Gerstmann-Strдussler syndrome, fatal familial insomnia, and kuru; animal diseases include scrapie, bovine spongiform encephalopathy, and mink encephalopathy. Called also transmissible neurodegenerative disease and subacute spongiform or transmissible spongiform encephalopathy.