NR ALVP
AU Udaka,F.; Fujisawa,M.; Kameyama,M.
TI [Creutzfeldt-Jakob disease(CJD) and Gerstmann-Sträussler-Scheinker syndrome(GSS)]
QU Nippon Rinsho. Japanese Journal of Clinical Medicine 1997 Apr; 55(4): 972-7
PT journal article; review; review literature
AB Prion diseases are now a focus of attention since the diseases are known to disseminate beyond expectation. They are various types of Gerstmann-Sträussler-Scheinker syndrome(GSS), fatal familial insomnia, iatrogenic Creutzfeldt-Jakob disease(CJD), and variant CJD which suspected to have disseminated from bovine spongiform encephalopathy. Abnormal prion protein deposits as amyloid structure in the brain of the patients with these diseases. Mutation of the prion protein gene exists in a part of these diseases. Depending on their kind, patients represent a variety of clinical and neuropathological manifestations. They can be clinically divided into two general groups of the CJD type and the GSS type. For clinical diagnosis of the GSS type prion diseases, analysis of prion protein gene is useful.
ZR 24
MH Animal; Cattle; Cattle Diseases/transmission; *Creutzfeldt-Jakob Syndrome/diagnosis; English Abstract; *Gerstmann-Sträussler-Scheinker Disease/diagnosis; Human
AD Department of Neurology, Sumitomo Hospital.
SP japanisch
PO Japan