Incipient intranuclear inclusion body disease in a 78-year-old woman
Incipient intranuclear inclusion body disease in a 78-year-old woman
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DOI：
10.1111/j.1440-1789.2010.01150.x
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发表时间：
2011-04-01
期刊：
影响因子：
2.3
通讯作者：
Wakabayashi, Koichi
中科院分区：
文献类型：
--
作者：
Mori, Fumiaki;Miki, Yasuo;Wakabayashi, Koichi
我们报告一个78岁的女性初期的核内包涵体病（INIBD）。在临床过程中没有发现明显的神经系统症状。尸检发现，在中枢和外周神经系统的神经元和神经胶质细胞以及内脏器官的实质细胞中广泛存在嗜酸性核内包涵体。神经胶质细胞中的包涵体多于神经元细胞。超微结构上，内含物由颗粒状和丝状物质组成。免疫组化结果显示，包涵体中泛素、泛素相关蛋白（NEDD8终极终结者1、小泛素修饰物1、小泛素修饰物2和p62）、早幼粒细胞白血病蛋白和异常扩增的多聚谷氨酰胺均呈阳性。与以前的研究一致，绝大多数的包含轴承胶质细胞的星形胶质细胞。此外，p25 α阳性少突胶质细胞很少含有核内包涵体。这些结果表明，INIBD可能发生在非痴呆的老年人，少突胶质细胞也参与了INIBD的疾病过程。
We report an incipient case of intranuclear inclusion body disease (INIBD) in a 78-year-old woman. No apparent neurological symptoms were noticed during the clinical course. Post mortem examination revealed widespread occurrence of eosinophilic intranuclear inclusions in neuronal and glial cells of the central and peripheral nervous systems, as well as in parenchymal cells of the visceral organs. The inclusions were observed more frequently in glial cells than in neuronal cells. Ultrastructurally, the inclusions consisted of granular and filamentous material. Immunohistochemically, the inclusions were positive for ubiquitin, ubiquitin-related proteins (NEDD8 ultimate buster 1, small ubiquitin modifier-1, small ubiquitin modifier-2 and p62), promyelocytic leukemia protein and abnormally expanded polyglutamine. Consistent with previous studies, the vast majority of inclusion-bearing glial cells were astrocytes. Furthermore, p25 alpha-positive oligodendrocytes rarely contained intranuclear inclusions. These findings suggest that INIBD may occur in non-demented elderly individuals and that oligodendrocyte is also involved in the disease process of INIBD.