Wilson病患者及其杂合子亲属的脑电图研究

目的:探讨Wilson病及其杂合子的脑电图改变与临床的关系。方法:记录43例Wilson患者及其60例杂合子亲属的脑电图。结果:43例Wilson病患者脑电图异常率达83.9％,以轻、中度异常改变为主,三组患者间异常率差异不明显(P>0.05);杂合子亲属中脑电图异常率达60.0％,子女组脑电图异常率及异常程度均高于同胞父母组,差异显著(P<0.05和0.01);杂合子亲属脑电图改变有年龄依赖性。Wilson病及杂合子脑电图改变均以弥漫性慢波化、阵发性慢活动和癫痫样放电等为主要表现。结论:1.脑电图可以作为判断肝损型及无症状型患者中枢神经系统临床下损害的有效指征;2.Wilson病杂合子脑电图改变与其年龄密切相关。

A STUDY ON EEG IN PATIENTS WITH WILSON' S DISEASE AND THEIR HETEROZYGOUS RELATIVES

Objectives: To study the correlation of clinical and EEG findings in patients with Wilson's disease and their heterozygous relatives. Methods: EEGs were recorded in 43 patients with Wilson's disease and their 60 heterzygors relatives. Results : The abnormal rate of EEG was 83.9% (59-6% was mild, 34.6% was moderate) in Wilson's disease. No significant differences were found in patients with different types of Wilson's disease (P>0. 05). In the heterozygous relatives, the abnormal rate of EEG was 60. 0%. The abnormal rate and degree of EEG were higher in the heterozygous children than in sibling parents. The most significant EEG changes were seen in the youngest age group. The main changes of EEG in Wilson's disease group and heterozygous group were slow-down of the basic rhythm, paroxysmal slow activities and epileptiform discharges, etc. Conclusions: 1. EEG might be an useful indicat; on in judging the presence of subclini-cal CNS damage in the hepatic and asymptomatic types. 2. The pathological EEG changes were closely related to the age of the heterzygotes.