Corticobasal degeneration: etiopathological significance of the cytoskeletal alterations
Corticobasal degeneration: etiopathological significance of the cytoskeletal alterations
复制标题
皮质基底节变性：细胞骨架改变的病因病理学意义
作者：
K. Wakabayashi;K. Oyanagi;T. Makifuchi;F. Ikuta;A. Homma;Y. Homma;Y. Horikawa;S. Tokiguchi
对3例皮质基底节变性（CBD）患者的脑组织进行了组织学、超微结构和免疫组化研究。大脑皮质神经元肿胀，黑质变性严重，基底节和脑干广泛分布弱嗜碱性神经纤维缠结（NFTs）。超微结构检查表明，NFT包括特征性的15 nm宽的直小管，其显示出阳性免疫组化染色与抗tau蛋白抗体，但不是泛素。在大脑皮层和皮层下核团中也发现了无NFT的tau免疫反应神经元细胞体，主要在脑干中，并且最大数量的tau阳性胶质细胞包涵体发生在前中央回和后中央回的大脑灰质和白色物质中。这些包涵体由直径约15 nm的管状结构组成，位于少突胶质细胞的胞质和突起中。这些结果表明，CBD和进行性核上性麻痹之间存在密切的细胞骨架病理关系。
We have studied brain tissues from three patients with corticobasal degeneration (CBD) histologically, ultrastructurally and immunohistochemically. Ballooned neurons in the cerebral cortex and severe degeneration of the substantia nigra were observed in them all and weakly basophilic neurofibrillary tangles (NFTs) were distributed widely in the basal ganglia and brain stem. Ultrastructural examination demonstrated that the NFTs comprised characteristic 15-nm-wide straight tubules, which showed positive immunohistochemical staining with an antibody against tau, but not ubiquitin. Tau-immunoreactive neuronal cell bodies without NFTs also were found in the cerebral cortex and subcortical nuclei, predominantly in the brain stem, and the greatest number of tau-positive glial inclusions occurred in the cerebral gray and white matter of the pre- and post-central gyri. These inclusions comprised tubular structures with diameters of about 15 nm and were localized in the oligodendroglial cellular cytoplasm and processes. These findings indicate that there is a close cytoskeletal pathological relationship between CBD and progressive supranuclear palsy.