Creutzfeldt-Jakob disease with long duration and panencephalopathic lesions: molecular analysis of one case.
case_report · Level V
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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.
Medical subject headings
- Brain
- Creutzfeldt-Jakob Syndrome
- Kuru