Liberski P P
Department of Oncology Medical Academy, Lódź.
Pol J Pathol. 1995;46(1):33-41.
Gerstmann-Sträussler-Scheinker (GSS) syndrome is one of the most rare human neurodegenerative disease. In this report I summarise the ultrastructural pathology of amyloid plaques in two cases of this rare disease. The first type, unicentric "kuru" plaques consisted of stellate arrangements (stars or cores) of amyloid bundles emanating from a densely interwoven centre. Amyloid stars were surrounded by astrocytic processes and invaded by microglial cells. The amyloid bundles were attached to microglial cell membranes. Of note, robust basement membranes lined with electron dense astrocytic anchorages material were observed at the periphery of almost all amyloid plaques. Dystrophic neurites (DN) were seen only rarely. Occasionally, clusters composed of several kuru plaques were found; those are interpreted as intermediate forms to multicentric plaques which consisted of several merging stellate cores. In contrast to kuru plaques associated with a limited number of DN, numerous such structures were seen at the periphery of multicentric plaques. Identical DN were observed not associated with any plaques. The last and the rarest type of plaques, were purely neuritic plaques. These consisted of large areas filled with DN of different sizes and shapes (sometimes bizarre) but not amyloid bundles. Analogously to kuru and multicentric plaques, astrocytic processes were observed at the periphery.
格斯特曼-施特劳斯勒-谢inker综合征（GSS）是人类最罕见的神经退行性疾病之一。在本报告中，我总结了两例这种罕见疾病中淀粉样斑块的超微结构病理学。第一种类型，单中心“库鲁”斑块由从紧密交织的中心发出的淀粉样蛋白束的星状排列（星或核心）组成。淀粉样蛋白星被星形胶质细胞突起包围，并被小胶质细胞侵入。淀粉样蛋白束附着于小胶质细胞膜。值得注意的是，在几乎所有淀粉样斑块的周边都观察到有电子致密的星形胶质细胞锚定物质衬里的强大基底膜。营养不良性神经突（DN）很少见。偶尔会发现由几个库鲁斑块组成的簇；这些被解释为多中心斑块的中间形式，多中心斑块由几个合并的星状核心组成。与与有限数量的DN相关的库鲁斑块不同，在多中心斑块的周边可见大量此类结构。观察到相同的DN与任何斑块均无关。最后也是最罕见的斑块类型是纯神经炎性斑块。这些斑块由充满不同大小和形状（有时怪异）的DN但没有淀粉样蛋白束的大面积区域组成。与库鲁斑块和多中心斑块类似，在周边观察到星形胶质细胞突起。
