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

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

Istituto Scienze Neurologiche

Research output: Contribution to journal › Article › peer-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 language	English
Pages (from-to)	271-274
Number of pages	4
Journal	Neurology
Volume	51
Issue number	1
Publication status	Published - Jul 1998

ASJC Scopus subject areas

Neuroscience(all)

Cite this

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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.",

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AU - Ghorayeb, I.

AU - Series, C.

AU - Parchi, P.

AU - Sawan, B.

AU - Guez, S.

AU - Laplanche, J. L.

AU - Capellari, S.

AU - Gambetti, P.

AU - Vital, Claude

PY - 1998/7

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N2 - 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.

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.

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

Abstract

ASJC Scopus subject areas

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