NR AULB
AU Krebs,P.P.
TI Transmissible spongiform encephalopathies - historical and clinical aspects of prion protein disorders
QU American Journal of Electroneurodiagnostic Technology 1996; 36(3): 182-9
PT Article
AB The transmissible spongiform encephalopathies are comprised of a group of human syndromes including kuru, an endemic disease of the Fore tribe in Papua, New Guinea; Creutzfeldt-Jakob disease (CJD), a rare, sporadic, pre-senile encephalopathy; and Gerstmann-Sträussler-Scheinker syndrome (GSS), which resembles kuru and CJD but is genetically transmissible; as well as scrapie, visna, bovine spongiform encephalopathy (BSE), and transmissible mink encephalopathy (TME) among others in animals. While reference to the animal encephalopathies may be made, the scope of this paper will address the human varieties with a particular focus on Creutzfeldt-Jakob disease. Until recently, CJD, kuru, and GSS were included with subacute sclerosing panencephalitis (SSPE), progressive rubella panencephalitis, and progressive multifocal leukoencephalopathy in the category of slow virus infections. While the latter of these diseases have all been attributed to classic viruses that have been visualized by electron microscopy, found to be antigenic in natural and experimental hosts, and able to produce cytolytic infection in some cell cultures, no agent has been positively identified to be the cause of CJD, GSS, or kuru. Currently, the widely held theory is that transmissible spongiform encephalopathies (TSE) are associated with a mutation of a single cell glycolipoprotein, named a prion protein (PrP).
ZR 17
SP englisch