Liberski P P, Budka H
Department of Oncology, Medical Academy Lodz, Poland.
Ultrastruct Pathol. 1995 Jan-Feb;19(1):23-36. doi: 10.3109/01913129509014600.
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.
我们报告了一项基于来自原始奥地利家族近期1例格斯特曼-施特劳斯勒-申克病大量样本的详细超微结构研究。众多朊蛋白免疫阳性斑块在光镜神经病理学中占主导地位。在超微结构上，观察到几种类型的斑块：单中心“库鲁”型、多中心型和神经炎性型。营养不良性神经突不同程度地伴随淀粉样斑块。斑块被星形细胞突起包绕，并被小胶质细胞侵入。显著的星形细胞反应伴随大量海绵状改变。在线粒体中观察到异常晶体，而在溶酶体内可见另一种类型的晶体。我们得出结论，格斯特曼-施特劳斯勒-申克病在超微结构水平上也有其独特之处。
