Huang Kai-Ju, Lu Ming-Kuei, Kao Albert, Tsai Chon-Haw
Neuroscience Laboratory, Department of Neurology , China Medical University Hospital, Taichung, Taiwan.
Acta Neurol Taiwan. 2007 Mar;16(1):13-21.
Corticobasal degeneration (CBD) is a rare neurodegenerative disorder characterized by distinctive clinical manifestations including asymmetric akinetic-rigid syndrome and higher cortical dysfunctions. We characterized the clinical, electrophysiological and imaging presentations in four patients with CBD. All patients exhibited unilateral hand dystonia, rigidity and apraxia, but showed no significant response to levodopa therapy. Surface electromyography demonstrated short duration and stimulus-sensitive myoclonus in three of the four patients. On the other hand, there was no "giant" SEPs (somatosensory evoked potentials), and the backaveraged electroencephalography did not show any jerk-locked cortical potentials. Brain magnetic resonance imaging showed asymmetrical cortical atrophy. [99mTc]HMPAO single-photon emission computed tomography (SPECT) revealed decreased regional cerebral blood flow in the frontoparietal areas and thalamus opposite to the more severely affected limb. [99mTc]TRODAT-1 SPECT showed decreased uptake in the striatum of the affected hemisphere. These data supported that there are abnormal cortical excitability and asymmetric pathological change of the affected hemisphere in the patients with CBD.
皮质基底节变性（CBD）是一种罕见的神经退行性疾病，其特征为独特的临床表现，包括不对称性运动不能-强直综合征和高级皮质功能障碍。我们对4例CBD患者的临床、电生理和影像学表现进行了特征分析。所有患者均表现为单侧手部肌张力障碍、强直和失用症，但对左旋多巴治疗无明显反应。表面肌电图显示4例患者中有3例出现短时限和刺激敏感的肌阵挛。另一方面，未发现“巨大”体感诱发电位（SEP），反向平均脑电图也未显示任何抽搐锁定的皮质电位。脑磁共振成像显示不对称性皮质萎缩。[99mTc]HMPAO单光子发射计算机断层扫描（SPECT）显示，与受影响更严重肢体相对侧的额顶叶区域和丘脑局部脑血流量减少。[99mTc]TRODAT-1 SPECT显示受影响半球纹状体摄取减少。这些数据支持CBD患者存在异常的皮质兴奋性和受影响半球的不对称性病理改变。
