Wakabayashi Koichi, Takahashi Hitoshi
Department of Neuropathology, Institute of Brain Science, Hirosaki University School of Medicine.
Rinsho Shinkeigaku. 2002 Nov;42(11):1155-7.
Corticobasal degeneration (CBD) is a sporadic neurodegenerative disorder of late life with a range of clinical presentations such as progressive asymmetrical rigidity and apraxia, progressive aphasia or dementia. Focal cortical atrophy, ballooned neurons and degeneration of the substantia nigra and globus pallidus have been emphasized in previous descriptions. Recent immunohistochemical studies revealed that tau-positive neuronal and glial lesions in both gray and white matter, especially astrocytic plaques in the affected cerebral cortex, are the characteristic features in CBD. While cortical involvement is also recognized in progressive supranuclear palsy, ballooned neurons are sparse and limited to the paralimbic areas and tufted astrocytes are abundant in the precentral gyrus and striatum. From a neuropathological viewpoint, CBD is distinct from other sporadic tauopathies.
皮质基底节变性（CBD）是一种散发性的老年神经退行性疾病，具有一系列临床表现，如进行性不对称性强直和失用、进行性失语或痴呆。以往的描述中强调了局灶性皮质萎缩、气球样神经元以及黑质和苍白球的变性。最近的免疫组织化学研究表明，灰质和白质中tau阳性的神经元和胶质病变，尤其是受影响的大脑皮质中的星形胶质细胞斑块，是CBD的特征性表现。虽然进行性核上性麻痹也存在皮质受累情况，但气球样神经元稀少且局限于边缘旁区域，中央前回和纹状体中则有丰富的簇状星形胶质细胞。从神经病理学角度来看，CBD与其他散发性tau蛋白病不同。
