ULTRASTRUCTURAL PATHOLOGY OF GERSTMANN-STRAUSSLER-SCHEINKER DISEASE

被引：20

作者：

LIBERSKI, PP

BUDKA, H

机构：

[1] POLISH MOTHER MEM HOSP,DEPT PATHOL,ELECTRON MICROSCOP LAB,LODZ,POLAND

[2] UNIV VIENNA,INST NEUROL,VIENNA,AUSTRIA

来源：

ULTRASTRUCTURAL PATHOLOGY | 1995年 / 19卷 / 01期

关键词：

CREUTZFELDT-JAKOB DISEASE; GERSTMANN-STRAUSSLER-SCHEINKER DISEASE; PRION DISEASES;

D O I：

10.3109/01913129509014600

中图分类号：

TH742 [显微镜];

学科分类号：

摘要：

We report a detailed ultrastructural study based on a large series of samples from a recent case of Gerstmann-Straussler-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-Straussler-Scheinker disease is distinct also at the ultrastructural level.

引用

页码：23 / 36

页数：14

共 47 条

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