Creutzfeldt-Jakob disease with long duration and panencephalopathic lesions: Molecular analysis of one case
- 1 July 1998
- journal article
- case report
- Published by Wolters Kluwer Health in Neurology
- Vol. 51 (1) , 271-274
- https://doi.org/10.1212/wnl.51.1.271
Abstract
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.Keywords
This publication has 1 reference indexed in Scilit:
- Creutzfeldt-Jakob disease with extensive degeneration of white matterActa Neuropathologica, 1980