THE CLINICAL AND NEURO-ELECTROPHYSIOLOGIC STUDY ON CORTICOBASAL DEGENERATION

目的:探讨皮质基底节变性(CBD)患者的临床和神经电生理特征。方法:对29例临床诊断为CBD患者,系统分析其临床症状、体征、常规化验检查、脑CT/MRI、SPECT以及神经电生理改变,并与其它疾病相对比。结果:结果显示,29例患者均出现不对称性帕金森样表现,皮层功能受损占79.3％,其它功能障碍占37.9％。21例患者脑CT或MRI显示不对称性脑萎缩,SPECT显示额顶区低灌注表现,以受损肢体的功能区较明显。EEG显示19例患者出现不对称性慢波,伴有肌阵挛的15例病人,表面肌电图显示患肢随意运动时可诱发巨大电位,皮层反应增强,SEP无特殊变化,运动诱发电位检查,26例患者中枢运动传导时间正常。结论:CBD是一种累及皮层和基底节、病程以缓慢进展为特征的变性性疾病。大部分患者可出现帕金森综合征样表现,皮层及其他运动功能障碍。脑影象学显示额顶叶萎缩。神经电生理检查表现为皮层兴奋性增强,而锥体束传导功能正常。

Objective: To explore the clinical and neuroelectrophysiologic features of patients with cortical basal degeneration (CBD) . Methods: Twenty-nine patients with clinical diagnosis of CBD were examined in detail in symptoms, sings, CT/MRI, SPECT and electrophysiology and compared with other diseases in clinical features. Results: The results showed that asymmetrical Parkinson's manifestations were presented in all cases, cortical dysfunction in 23 cases (79.3%), other disorders in 11 cases (37.9%). Twenty-one cases showedasymmetrical atrophy in CT/MRI and 25 patients were hypoperfused in the fronto-parietal regions in SPECT and affected contralateral limbs. There were asymmetrical slow waves in 19 cases in EEG. In surface EMG, fifteen cases with myoclonus showed enhanced responses in their myoclonus arm or leg, cortical reflexes enhanced and with normal somatosensory evoked potential (SEP) , Twenty - six patients showed normal CMCT in MEPs. Conclusion: CBD is characterized by a slow progressive disease with both cortical and basal ganglionic dysfunction . The most manifestation of CBD was asymmetric Parkinson's syndrome, cortical dysfunction and other movement disorders, Brain imaging showed fronto-parietal atrophy. The study of electrophysiology showed that cortical excitability enhanced and pyramidal tract was intact.