NR AEMB
AU Genthon,R.; Gray,F.; Salama,J.; Duyckaerts,C.; Belin,C.; Brucher,J.M.; Baron,H.; Delaporte,P.
TI [Gerstmann-Sträussler-Scheinker disease. Pathologal and genetic study]
OT Maladie de Gerstmann-Sträussler-Scheinker. Etude pathologique et genealogique.
QU Revue Neurologique 1992; 148(5): 335-42
PT journal article; review; review of reported cases
AB 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.
ZR 47
MH Amyloidosis/pathology; Brain/pathology; Case Report; Cerebellum/pathology; Creutzfeldt-Jakob Syndrome/diagnosis; Diagnosis, Differential; English Abstract; Gerstmann-Sträussler-Scheinker Disease/genetics/*pathology; Human; Kuru/diagnosis; Male; Middle Age; Pedigree
AD Service de Neurologie, Hopital Avicenne, Bobigny.
SP französisch
PO Frankreich