[Gerstmann-Sträussler-Scheinker disease. Pathologal and genetic study]. / Maladie de Gerstmann-Sträussler-Scheinker. Etude pathologique et généalogique.
Rev Neurol (Paris)
; 148(5): 335-42, 1992.
Article
em Fr
| MEDLINE
| ID: mdl-1448647
ABSTRACT
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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Base de dados:
MEDLINE
Assunto principal:
Doença de Gerstmann-Straussler-Scheinker
Tipo de estudo:
Diagnostic_studies
Limite:
Humans
/
Male
/
Middle aged
Idioma:
Fr
Ano de publicação:
1992
Tipo de documento:
Article