目的 :观察中药肝豆片Ⅰ号对肝豆状核变性 (HLD)患者神经电生理的影响。方法 :32例HLD患者治疗前检测脑电图 (VEEG)、肌电图 (EMG)及脑干听觉诱发电位 (BAEP) ,于口服肝豆片Ⅰ号治疗4周后 ,对治疗前有异常神经电生理改变的患者进行复查。结果 :HLD患者治疗后在临床症状明显改善的同时 ,VEEG、EMG及BAEP的有效率依次为 71 4 3% (5 /7例 )、70 0 0 % (7/10例 )、6 6 6 7% (2 0 /30例 ) ,其有效率差异无显著性 (P >0 0 5 )。HLD患者治疗前Ⅲ、Ⅴ波潜伏期 (PL)及Ⅰ Ⅲ、Ⅲ Ⅴ、Ⅰ Ⅴ波峰间期(IPL)均较健康对照组延长 (P <0 0 5 ,P <0 0 1) ;治疗后Ⅲ Ⅴ、Ⅰ Ⅴ波IPL均有改善。结论 :神经电生理检查对HLD有辅助诊断及疗效判断价值 ,肝豆片Ⅰ号能改善HLD患者异常的神经电生理。 Objective: To observe the effect of Gadou No.1 on the electrophysiology of hepatolenticular degeneration (HLD). Methods: EEG, electromyogram (EMG) and brainstem auditory evoked potentials (BAEP) were measured in 32 HLD patients before treatment. After oral administration of Gudou Tablet Ⅰ for 4 weeks, Patients with electrophysiological changes are reviewed. Results: The clinical symptoms of HLD patients were significantly improved after treatment, while the effective rates of VEEG, EMG and BAEP were 71 4 3% (70 cases), 70 0 0 cases (7/10 cases), 6 6 6 7 % (20/30), the effective rate was not significantly different (P> 0.05). The latency of PL and Ⅲ, Ⅲ Ⅴ and Ⅰ Ⅴ peak interval (IPL) in patients with HLD before treatment were longer than those in healthy controls (P <0.05, P <0.01). After treatment, Ⅲ Ⅴ, Ⅰ Ⅴ wave IPL have improved. Conclusion: The neuroelectrophysiological examination has the value of adjuvant diagnosis and therapeutic effect on HLD. HeDou Tablet No.1 can improve the abnormal electrophysiology of HLD patients.