Experimental transmission of human subacute spongiform encephalopathy to small rodents. IV. Positive transmission from a typical case of Gerstmann-Sträussler-Scheinker's disease.
Spongiform encephalopathy was transmitted to mice from a patient belonging to the "Sch" family with Gerstmann-Sträussler-Scheinker's disease (GSS). Incubation periods in the first passage were much shorter than those in mice infected with Creutzfeldt-Jakob disease. Clinical and pathologic findings of mice infected with both diseases were almost identical. This is the first successful transmission from a typical GSS case without severe spongiform change which suggests the possible transmissible nature of this disorder.
D000818:Animals; D001921:Brain / Q000473:pathology; D002524:Cerebellar Ataxia / Q000235:genetics / Q000473:pathology / Q000635:transmission*; D007562:Creutzfeldt-Jakob Syndrome / Q000473:pathology; D003704:Dementia / Q000235:genetics* / Q000473:pathology; D006801:Humans; D051379:Mice; D009410:Nerve Degeneration; D013577:Syndrome