Wakabayashi Koichi, Miki Yasuo
Department of Neuropathology, Hirosaki University Graduate School of Medicine, Japan.
Brain Nerve. 2013 Jan;65(1):9-18.
Focal asymmetric cortical atrophy with ballooned neurons, nigral degeneration, and tau-positive neuronal and glial lesions in both the gray and white matter, particularly astrocytic plaques in the affected cerebral cortex, are characteristic features of corticobasal degeneration (CBD). Since the clinical manifestations of CBD are diverse, several neurological diseases can present with clinical findings that are thought to be specific to CBD. These clinical conditions are referred to as corticobasal syndrome (CBS). CBS includes Alzheimer's disease, progressive supranuclear palsy, Pick's disease, Lewy body disease, frontotemporal lobar degeneration, and Creutzfeldt-Jakob disease. In cases of clinically diagnosed CBS, the consistent histopathological finding appears to be asymmetric frontal and parietal cortical degeneration.
伴有气球样神经元的局灶性不对称皮质萎缩、黑质变性以及灰质和白质中tau阳性神经元和胶质细胞病变，尤其是受累大脑皮质中的星形胶质细胞斑，是皮质基底节变性（CBD）的特征性表现。由于CBD的临床表现多样，几种神经系统疾病可能会出现被认为是CBD特有的临床症状。这些临床情况被称为皮质基底节综合征（CBS）。CBS包括阿尔茨海默病、进行性核上性麻痹、匹克病、路易体病、额颞叶变性和克雅氏病。在临床诊断为CBS的病例中，一致的组织病理学发现似乎是不对称的额叶和顶叶皮质变性。
