NR AHHQ
AU Liberski,P.P.; Budka,H.
TI Ultrastructural pathology of Gerstmann-Sträussler-Scheinker disease
QU Ultrastructural Pathology 1995 Jan-Feb; 19(1): 23-36
PT journal article
AB We report a detailed ultrastructural study based on a large series of samples from a recent case of Gerstmann-Sträussler-Scheinker disease from the original Austrian family. Numerous PrP-immunopositive plaques dominated light microscopic neuropathology. Ultrastructurally, several types of plaques were observed: unicentric "kuru," multicentric, and neuritic. Dystrophic neurites accompanied amyloid plaques to differing degrees. Plaques were enveloped by astrocytic processes and invaded by microglial cells. A prominent astrocytic reaction accompanied abundant spongiform change. Unusual crystalloids were observed in mitochondria while another type of crystalloid was seen within lysosomes. We conclude that Gerstmann-Sträussler-Scheinker disease is distinct also at the ultrastructural level.
ZR 47
MH Amyloid/analysis/immunology; Astrocytes/ultrastructure; Austria; Cerebral Cortex/chemistry/*ultrastructure; Gerstmann-Sträussler-Scheinker Disease/metabolism/*pathology; Human; Immunohistochemistry; Inclusion Bodies/ultrastructure; Microglia/ultrastructure; Support, Non-U.S. Gov't; Vacuoles/ultrastructure
AD P.P. Liberski, Medical Academy Lodz, Tumor Biol Labs, Laboratory of Electron Microscopy and Neuropathology, Chair Oncol, 4 Paderewski St, PL-93509 Lodz, Poland
SP englisch
PO USA