NR AENB
AU Ghorayeb,I.; Series,C.; Parchi,P.; Sawan,B.; Guez,S.; Laplanche,J.L.; Capellari,S.; Gambetti,P.; Vital,C.
TI Creutzfeldt-Jakob disease with long duration and panencephalopathic lesions: molecular analysis of one case.
QU Neurology 1998 Jul; 51(1): 271-4
PT journal article
AB A 49-year-old woman presented with isolated aphasia followed by dementia and ataxia with a duration of 4 years. Histopathologically there was panencephalic involvement, status spongiosus, and kuru-type plaques. Molecular analysis showed heterozygosity at codon 129 in the prion protein (PrP) gene, and type 2 protease-resistant PrP. The comparison between this case and those previously reported suggests that the panencephalopathic variant of Creutzfeldt-Jakob disease (CJD) is an aspecific end-stage condition displayed by most if not all CJD variants in individual patients with an unusually prolonged course.
MH Biopsy; Brain/*pathology; Case Report; Creutzfeldt-Jakob Syndrome/*genetics/*pathology; Female; Genotype; Human; Kuru/genetics/*pathology; Middle Age; Nerve Fibers/pathology; Prions/genetics; Support, Non-U.S. Gov't; Support, U.S. Gov't, P.H.S.
AD Department of Internal Medicine, Victor Segalen University, Bordeaux, France.
SP englisch
PO USA