橄榄-桥脑-小脑萎缩的临床神经电生理学研究

目的 了解橄榄－桥脑－小脑萎缩（OPCA）患者的临床特点与神经电生理改变。方法 对16例OPCA患者进行多种形式的电生理检查，并结合临床表现进行分析。结果 发现OPCA患者视觉诱发电位（VEP）、听觉诱发电位（BAEP）的体感诱导电位（SEP）有较高的异常率，而事件相关电位P300、肌电图（EMG）、周围神经传导速度（NCV）和脑电图（EEC）无明显异常变化；临床以共济失调为核心症状伴随多种形式的神经功能异常，诱发电位检查可以发现一些亚临床病灶。结论 神经系统病理损害的广泛性是OPCA患者的主要特征，电生理检查对确定亚临床病灶有重要价值。

Clinical electrophysiology of the patients with olivo-ponto-cerebellar atrophy

Objective To observe the clinical features and electrophysiology changes of the patients with olivo-ponto-cerebellar atrophy(OP- CA). Mothods Various electrophysiologic study(EPS) were tested in 16 patients with OPCA. The changes of EPS were analyzed in comparison with the clinical appearance. Results It was found that all patients exhibited abnormal changes in at least one of the EPS. There were high abnor- mal rates on visual evoked potential (VEP) test, brain-stem auditory evoked potential(BAEP) test and somatosensory evoked potential(SEP)test. The distinctive changes were not found on electromyography, nerve coduction velocity, electroencephalography (EEG) and event-related potentials P300. Ataxia was the main clinical manifestations coexisting multiple neurological symptoms. Conclusion Patients with OPCA have extentive damages in nervous system. Multimodal EPS have great value in determining the subclinical pathological conditions of OPCA.