Butinar D, Trontelj J V, Khuraibet A J, Khan R A, Hussein J M, Shakir R A
Institute of Clinical Neurophysiology, University Medical Center, Ljubljana, Yugoslavia.
J Neurol Sci. 1990 Feb;95(2):163-9. doi: 10.1016/0022-510x(90)90239-j.
Twelve patients with Wilson's disease, aged 11-25 years, underwent brainstem auditory evoked potential (BAEP) study. The results were correlated to clinical, neuroradiological and laboratory data. Ten had prominent to severe neurological manifestations, suggestive of involvement of one or several CNS structures, and 2 were neurologically free. All had evidence of abnormal copper metabolism, and 8 had CT scan evidence of brain atrophy, or hypodense areas in basal ganglia, or both. The 2 patients without neurological manifestations as well as one with neurological signs had normal BAEP. One patient with neurological signs had increased N1 latency due to cochlear hearing loss, but normal interpeak intervals, while 8 of 10 patients with prominent neurological symptoms and signs had abnormal BAEPs (prolongation of NIII-NV interpeak interval). The value of NIII-NV interpeak interval correlated with the number of different neurological signs (neurological score) attributable to involvement of different CNS structures (r = 0.64 at P = 0.001). Abnormal BAEPs do not seem to be an early finding in Wilson's disease.
12例年龄在11至25岁的威尔逊病患者接受了脑干听觉诱发电位（BAEP）检查。研究结果与临床、神经放射学及实验室数据进行了关联分析。其中10例有明显至严重的神经学表现，提示一个或多个中枢神经系统结构受累，2例无神经学症状。所有患者均有铜代谢异常的证据，8例有脑部萎缩的CT扫描证据，或基底节区低密度影，或两者皆有。2例无神经学表现的患者以及1例有神经学体征的患者BAEP正常。1例有神经学体征的患者因耳蜗性听力损失N1潜伏期延长，但峰间期正常，而10例有明显神经学症状和体征的患者中有8例BAEP异常（NIII-NV峰间期延长）。NIII-NV峰间期的值与因不同中枢神经系统结构受累所致不同神经学体征的数量（神经学评分）相关（r = 0.64，P = 0.001）。BAEP异常似乎并非威尔逊病的早期表现。
