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prion diseases

transmissible and genetic neurodegenerative diseases of humans and animals caused by prions. The diseases are usually characterised by vacuolation in the gray matter and result in ataxia, motor disturbances, dementia, and progression to a fatal outcome. They include creutzfeldt-jakob syndrome, gerstmann-straussler syndrome, kuru, scrapie, fatal familial insomnia, bovine spongiform encephalopathy (encephalopathy, bovine spongiform), transmissible mink encephalopathy, and chronic wasting disease of mule deer and elk. The literature has sometimes referred to these as unconventional slow virus diseases.