title: [Transmission of spongiform encephalopathies (prion diseases)].
abstract: The transmissible spongiform encephalopathies (TSE), or prion diseases, constitute a form of degenerative disorders of the central nervous system, which are characterized by a typical spongiform histological pattern and a fatal course. According to Prusiner's theory, its agent consists of a protein without any nucleic acid, the "proteinaceous infectious agent", or prion. This is a pathologically folded form of the normal prion protein (PrPC), and then called PrPSc. TSE are observed in different mammals including humans. In humans, they include Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker syndrome (GSS), fatal familial insomnia (FFI), Kuru and the new variant of the Creutzfeldt-Jakob disease (vCJD). The longest known TSE is scrapie in sheep. Since 1984 the bovine spongiform encephalopathy (BSE) and since 1996 the variant Creutzfeldt-Jakob disease have been documented. This paper gives an overview on the pathogenesis, epidemiology and the clinical features of the different prion diseases.
authors.affiliation: Klinik und Poliklinik für Hals-Nasen-Ohren-Heilkunde, Universitätsklinken des Saarlandes, Kirrberger Strasse, 66421 Homburg/Saar. P.Federspil@uniklinik-saarland.de
authors.forename: P
authors.initials: P
authors.lastname: Federspil
authors.forename: P A
authors.initials: PA
authors.lastname: Federspil
mesh_terms: D000818:Animals; D001921:Brain / Q000473:pathology; D002417:Cattle; D006801:Humans; D017096:Prion Diseases / Q000473:pathology / Q000635:transmission*; D011328:Prions / Q000032:analysis; D012307:Risk Factors; D012756:Sheep
chemical_list: D011328:Prions
vernacular_title: Ubertragbare spongiforme Enzephalopathien (Prionkrankheiten).