title: Ultrastructural pathology of Gerstmann-Sträussler-Scheinker disease.
abstract: 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.
authors.affiliation: Department of Oncology, Medical Academy Lodz, Poland.
authors.forename: P P
authors.initials: PP
authors.lastname: Liberski
authors.forename: H
authors.initials: H
authors.lastname: Budka
mesh_terms: D000682:Amyloid / Q000032:analysis / Q000276:immunology; D001253:Astrocytes / Q000648:ultrastructure; D001317:Austria; D002540:Cerebral Cortex / Q000737:chemistry / Q000648:ultrastructure*; D016098:Gerstmann-Straussler-Scheinker Disease / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D002479:Inclusion Bodies / Q000648:ultrastructure; D017628:Microglia / Q000648:ultrastructure; D014617:Vacuoles / Q000648:ultrastructure
chemical_list: D000682:Amyloid