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[Gerstmann-Sträussler-Scheinker disease. Pathologal and genetic study]. / Maladie de Gerstmann-Sträussler-Scheinker. Etude pathologique et généalogique.
Genthon, R; Gray, F; Salama, J; Duyckaerts, C; Belin, C; Brucher, J M; Baron, H; Delaporte, P.
Afiliación
  • Genthon R; Service de Neurologie, Hôpital Avicenne, Bobigny.
Rev Neurol (Paris) ; 148(5): 335-42, 1992.
Article en Fr | MEDLINE | ID: mdl-1448647
Gerstmann-Sträussler-Scheinker's disease is a familial spongiform encephalopathy whose pathological hallmark is the existence--especially in the cerebellum--of numerous amyloid plaques. We report here the third clinicopathological case in a French family. Brain tissue from one of its members--initially described as familial Creutzfeldt-Jakob's disease--has been reported as successfully inoculated to monkeys. We present the currently accumulating data favouring the hypothesis of a common etiology for familial Creutzfeldt-Jakob's disease and Gerstmann-Sträussler-Scheinker's disease. The familial characteristics, resulting in different durations of incubation and evolution, could lead to different clinical and histological expressions.
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Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Enfermedad de Gerstmann-Straussler-Scheinker Tipo de estudio: Diagnostic_studies Límite: Humans / Male / Middle aged Idioma: Fr Revista: Rev Neurol (Paris) Año: 1992 Tipo del documento: Article Pais de publicación: Francia
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Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Enfermedad de Gerstmann-Straussler-Scheinker Tipo de estudio: Diagnostic_studies Límite: Humans / Male / Middle aged Idioma: Fr Revista: Rev Neurol (Paris) Año: 1992 Tipo del documento: Article Pais de publicación: Francia