Gerstmann-Straüssler-Scheinker disease.

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-Jacob 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).