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65例肝豆状核变性(HLD)患者驱铜治疗前与治疗2个月后,分别进行脑干听觉诱发电位(BAEP)检测,观察其潜伏期(PL)和峰间期(IPL)的变化,并选用38例健康人为对照组。结果表明,HLD患者BAEP的PL和IPL较正常对照组明显延长,主要表现为Ⅲ、Ⅴ波PL及Ⅲ-Ⅴ、Ⅰ-ⅤIPL延长,从而提示铜离子在HLD患者脑内沉积损害了脑干听觉传导通路。经驱铜治疗后,临床症状有明显的改善,同时BAEP亦显示各波PL和IPL较疗前显著好转。男女患者治疗前的异常率分别为22%和19%,治疗后降至9%和7%(P<0.05,P<0.01),BAEP的好转与临床症状的改善一致。
Sixty-five patients with hepatolenticular degeneration (HLD) were treated with copper for two months before treatment and brainstem auditory evoked potentials (BAEP) were measured to observe the change of latency and peak interval (IPL) 38 healthy people were chosen as the control group. The results showed that the PL and IPL of BAEP in HLD patients were significantly longer than those in normal controls, which were mainly manifested by the increase of Ⅲ and Ⅴ wave PL and the prolongation of Ⅲ-Ⅴ and Ⅰ-ⅤIPL, which suggested that the deposition of copper ions in brain of HLD patients impaired brainstem auditory Conduction pathway. After copper-driven treatment, clinical symptoms were significantly improved, while BAEP also showed that the wave PL and IPL significantly improved compared with before treatment. The rates of abnormalities before and after treatment in both men and women were 22% and 19%, respectively, and decreased to 9% and 7% after treatment (P <0.05, P <0.01). The improvement of BAEP was consistent with the improvement of clinical symptoms.