[Gerstmann-Sträussler-Scheinker disease. Pathologal and genetic study]

Autor: R, Genthon, F, Gray, J, Salama, C, Duyckaerts, C, Belin, J M, Brucher, H, Baron, P, Delaporte
Jazyk: francouzština
Rok vydání: 1992
Předmět:
Zdroj: Revue neurologique. 148(5)
ISSN: 0035-3787
Popis: 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.
Databáze: OpenAIRE