[Gerstmann-Sträussler-Scheinker disease].
In three of six observations of familial dementia in adult age neuropathological examination revealed Gerstmann-Sträussler-Scheinker's disease characterized from the morphological aspect by amyloid plaques and a spongious state of the gray matter of the brain. As to the latter characterization the disease is close to Jacob-Creutzfeld's disease. The presence of amyloid plaques signalizes extreme production of protein material which cumulates. It is obviously different from amyloid of senile plaques in Alzheimer's disease and corresponds probably to infectious protein material-prions.
D000328:Adult; D001921:Brain / Q000473:pathology*; D003704:Dementia / Q000235:genetics / Q000473:pathology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree
Gerstmannova-Sträusslerova-Scheinkerova nemoc.