[Inherited human prion diseases].

Abstract excerpt

Familial prion diseases (familial Creutzfeldt-Jakob disease: Gerstmann-Sträussler-Scheinker disease, Fatal familial insomnia) are rare, but are also at present one of the most fascinating chapters of Neurology because of their double transmissibility. They are hereditary diseases of adults with a dominant autosomal transmission and an almost complete penetrance. They result most often of a point mutation of the...

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