Creutzfeldt-Jakob disease with long duration and panencephalopathic lesions: Molecular analysis of one case

I. Ghorayeb, C. Series, P. Parchi, B. Sawan, S. Guez, J. L. Laplanche, S. Capellari, P. Gambetti, Claude Vital

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
Pages (from-to)271-274
Number of pages4
JournalNeurology
Volume51
Issue number1
Publication statusPublished - Jul 1998

ASJC Scopus subject areas

  • Neuroscience(all)

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