Takahashi T, Amano N, Hanihara T, Nagatomo H, Yagishita S, Itoh Y, Yamaoka K, Toda H, Tanabe T
Division of Neurology and Psychiatry, Kanagawa Rehabilitation Center, Atsugi, Japan.
J Neurol Sci. 1996 Jun;138(1-2):66-77. doi: 10.1016/0022-510x(95)00347-5.
A 57-year-old man had exhibited cortical sensory disturbance, rigidity, spasticity, dementia, alien hand, grasp reflex, supranuclear ophthalmoplegia, pseudobulbar palsy, and neck dystonia for 4 years. Histological examination of autopsied specimens revealed neuronal loss in the cerebral cortex, with ballooned neurons, subthalamic nucleus, substantia nigra, basal ganglia, midbrain tegmentum, and the thalamus. There were neurofibrillary tangles in the subthalamic nucleus and the substantia nigra. Gallyas-Braak silver impregnation demonstrated numerous argentophilic tangles, threads, and a few argentophilic glia in the cerebral cortex, subcortical white matter, particularly in the precentral gyrus, subcortical nuclei, and the brainstem. These argentophilic structures were largely positive for tau, and negative for ubiquitin, paired helical filaments, and phosphorylated neurofilament. Ultrastructurally, 15-nm-wide straight tubules were observed in the neurons of the substantia nigra, globus pallidus, and the precentral cortex, coexisting with a few twisted tubules periodically constricted at 160- to 230-nm intervals. It was conclusively shown that Gallyas- and tau-positive cytoskeletal abnormalities occurred widely in brain of corticobasal degeneration. Both distribution and morphology of abnormal phosphorylated tau protein in corticobasal degeneration appear to resemble these features in progressive supranuclear palsy. These findings suggest a common cytoskeletal etiopathological significance in corticobasal degeneration and progressive supranuclear palsy.
一名57岁男性出现皮质感觉障碍、强直、痉挛、痴呆、异己手、抓握反射、核上性眼肌麻痹、假性延髓麻痹和颈部肌张力障碍达4年。尸检标本的组织学检查显示大脑皮质神经元丢失，伴有气球样神经元、丘脑底核、黑质、基底神经节、中脑被盖和丘脑。丘脑底核和黑质存在神经原纤维缠结。Gallyas-Braak银浸染显示大脑皮质、皮质下白质，尤其是中央前回、皮质下核团和脑干中有大量嗜银缠结、细丝和少量嗜银胶质细胞。这些嗜银结构tau蛋白大多呈阳性，泛素、双螺旋丝和磷酸化神经丝呈阴性。超微结构上，在黑质、苍白球和中央前皮质的神经元中观察到15纳米宽的直小管，与一些每隔160至230纳米周期性收缩的扭曲小管共存。最终表明，Gallyas和tau阳性的细胞骨架异常在皮质基底节变性的大脑中广泛存在。皮质基底节变性中异常磷酸化tau蛋白的分布和形态似乎与进行性核上性麻痹中的这些特征相似。这些发现提示皮质基底节变性和进行性核上性麻痹在细胞骨架病因学上具有共同意义。
