NR AGAU

AU Jirasek,A.; Holy,V.; Chodura,V.; Cutka,F.; Sulc,M.; Koukolik,F.

TI Gerstmann-Sträussler-Scheinker disease

QU Czechoslovak Medicine 1988; 11(1): 49-56

PT journal article

AB In three out of six investigated cases of familially occurring dementia in adult age, the neuropathological examination disclosed Gerstmann-Sträussler-Scheinker disease, morphologically characterized by the presence of amyloid plaques and spongy condition of the brain grey matter. The spongy condition makes this disease related to Creutzfeldt-Jakob disease. The presence of the amyloid plaques signalizes an extreme production of the protein material which accumulates in them and which will be different from the amyloid in the senile plaques in Alzheimer's disease, and probably corresponds with infectious protein substances - prions or protein-like particles (PLP).

MH Adult; Female; Human; Male; Middle Age; Pregnancy; Slow Virus Diseases/genetics/*pathology

AD School of Medicine, Charles University, Prague.

SP englisch

PO Tschechoslowakei

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