Prion Diseases

Abstract Prion diseases, or transmissible spongiform encephalopathies, are degenerative disorders of the central nervous system and are of sporadic, familial or acquired nature. They include Creutzfeldt–Jakob disease (CJD), Gerstmann–Sträussler–Scheinker syndrome, kuru and fatal familial insomnia in humans, as well as all prion encephalopathies of animals. The latter comprise scrapie in sheep, bovine spongiform encephalopathy (BSE), or ‘mad cow disease’, and chronic wasting diseases of mule, deer and exotic ungulates. In the last few years, a great body of circumstantial evidence has accumulated indicating that BSE prions can provoke new variant CJD.