首页> 外文期刊>Neuropathology: official journal of the Japanese Society of Neuropathology >Incipient intranuclear inclusion body disease in a 78-year-old woman.
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Incipient intranuclear inclusion body disease in a 78-year-old woman.

机译:一名78岁女性的初期核内包涵体疾病。

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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, p25alpha-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.
机译:我们报告了一名78岁女性的核内包涵体疾病(INIBD)的早期病例。在临床过程中未发现明显的神经系统症状。验尸后发现,嗜酸性核内包裹物广泛存在于中枢神经系统和周围神经系统的神经元和神经胶质细胞中,以及内脏器官的实质细胞中。在神经胶质细胞中比在神经元细胞中更频繁地观察到内含物。在超微结构中,夹杂物由颗粒状和丝状材料组成。免疫组织化学检测,包涵体呈阳性,涉及泛素,泛素相关蛋白(NEDD8终极抑制物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展的聚谷氨酰胺。与先前的研究一致,绝大多数包含包涵体的神经胶质细胞是星形胶质细胞。此外,p25alpha阳性少突胶质细胞很少包含核内包裹体。这些发现表明,INIBD可能发生在非痴呆的老年人中,少突胶质细胞也参与了INIBD的发病过程。

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