NR ADXB
AU Federspil,P.; Federspil,P.A.
TI [Transmission of spongiform encephalopathies (prion diseases)]
OT Ubertragbare spongiforme Enzephalopathien (Prionkrankheiten)
QU HNO 2002 Apr; 50(4): 316-26
PT journal article; review; review, tutorial
AB 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.
ZR 51
MH Animal; Brain/pathology; Cattle; English Abstract; Human; Prion Diseases/pathology/*transmission; Prions/analysis; Risk Factors; Sheep
AD Klinik und Poliklinik für Hals-Nasen-Ohren-Heilkunde, Universitätsklinken des Saarlandes, Kirrberger Strasse, 66421 Homburg/Saar. P.Federspil@uniklinik-saarland.de
SP deutsch
PO Deutschland