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Ｗｉｌｔｓｏｎ病患者及其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无显著性（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，差异有显著性（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 Aheir heterozygous relatives. Methods: EEGs were recorded in 43 patients with Wilson's disease and their 60 heterzygous relatives (26 children. 30 parents and 4 siblings). Results: The abnormality rate of EEG was 839%(596% mild, 34.6% moderate) in Wilson's disease. No significant differences were found in patients with different types of Wilson's disease (P>005).As for the heterozygous relatives, the abnormality rate of EEG was 600%, a significant increase in pathological EEG findings was found in the heterozygous children. The most significant EEG changes were seen in the youngest age group. The main changes of EEG in Wilson's disease group and heterzygotes group were a slowdown of the basic rhythm, slow waves increase, paroxysmal slow waves and epiletiform discharges, etc. Conclusions: 1EEG might be an useful indicator in judging the presence of subclinical CNS damage in the hepatic and asymtomatic forms. 2. The pathological EEG changes are closely related to the age of the heterzygotes.