Mizusawa H
Department of Neurology, Tokyo Medical and Dental University.
Rinsho Shinkeigaku. 1997 Dec;37(12):1131-3.
Corticobasal degeneration (CBD) was first reported by Rebeiz et al as corticodentatonigral degeneration with neuronal achromasia in 1967. After Gibb et al described 7 cases including 4 cases from the literature under the term of corticobasal degeneration, CBD has become widely recognized. The disease starts mainly in one's fifties and sixties with the duration of 6 to 7 years. The clinical features include asymmetric parkinsonism, cerebral cortical signs, and others. Typically, patients present with unilateral clumsiness with akinetic-rigid syndrome and limb-kinetic apraxia. Postural instability, gait disturbance and involuntary movements such as dystonia are not uncommon. The parkinsonism is DOPA-resistant. BEsides apraxia, alien limb syndrome, cortical sensory disturbances, frontal lobe-release signs, and dementia are representative cortical signs. Other clinical features include dysarthria, pyramidal tract signs and supranuclear gaze palsy. MRI, SPECT or PET reveals asymmetric atrophy, decrease in blood flow or reduction in metabolism of the frontal parietal region around the central sulcus. Electrophysiological and magnetic stimulation studies demonstrated increase in excitability of the cerebral cortex. Myoclonus in CBD is cortical in origin but without any preceding potential or giant somatosensory evoked potential. Neuropathologically CBD is characterized by involvement of the particular cortices and substantia nigra. Other structures such as the putamen, pallidum, thalamus, subthalamus, cerebellar dentate nucleus and brainstem are affected to various extents. Histological features include achromatic, ballooned neurons as well as tau and Gallyas positive neuronal and glial intracytoplasmic inclusions. Astrocytic plaque is considered to be a form of glial inclusions specific to CBD. Diagnosis of typical cases of CBD appears easy but atypical cases were reported with showed dementia or aphasia as a main feature, or were devoid of the asymmetry of signs and symptoms. CBD, progressive supranuclear palsy and Pick's disease share both clinical and neuropathological features to some extent while they are clearly distinct among typical cases. The etiology and pathomechanism of CBD remain to be elucidated.
皮质基底节变性（CBD）于1967年由雷贝兹等人首次报道为伴有神经元色素脱失的皮质齿状核黑质变性。在吉布等人以皮质基底节变性之名描述了7例病例（包括4例文献报道病例）后，CBD已被广泛认可。该病主要在五六十岁起病，病程为6至7年。临床特征包括不对称性帕金森综合征、大脑皮质体征等。典型表现为单侧笨拙，伴有运动不能-强直综合征和肢体运动性失用。姿势不稳、步态障碍和诸如肌张力障碍等不自主运动也并不少见。帕金森综合征对左旋多巴耐药。除失用外，异己肢体综合征、皮质感觉障碍、额叶释放体征和痴呆是典型的皮质体征。其他临床特征包括构音障碍、锥体束征和核上性凝视麻痹。MRI、SPECT或PET显示中央沟周围额顶叶区域不对称萎缩、血流减少或代谢降低。电生理和磁刺激研究表明大脑皮质兴奋性增加。CBD中的肌阵挛起源于皮质，但无前驱电位或巨大体感诱发电位。神经病理学上，CBD的特征是特定皮质和黑质受累。其他结构如壳核、苍白球、丘脑、下丘脑、小脑齿状核和脑干也有不同程度的受累。组织学特征包括无色、气球样神经元以及tau和加利亚斯染色阳性的神经元和胶质细胞胞浆内包涵体。星形细胞斑被认为是CBD特有的一种胶质细胞包涵体形式。典型CBD病例的诊断似乎容易，但也有非典型病例报道，这些病例以痴呆或失语为主要特征，或无体征和症状的不对称性。CBD、进行性核上性麻痹和皮克病在一定程度上具有共同的临床和神经病理学特征，但在典型病例中它们明显不同。CBD的病因和发病机制仍有待阐明。
