摘要
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35例(81%)，以轻、中度异常改变为主，杂合子亲属中脑电图异常率为60%，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；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、阵发性慢活动和样放电等为主要表现。结论：脑电图可以作为判断肝损型及无症状型Willson病患者中枢神经系统临床下损害的有效指征；Wilson病杂合子脑电图改变与其年龄密切相关。
Objectives: To study the relationship between the clinical signs and the 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 heterzygous relatives.Results:The abnormality rate of EEG was 81%in patients with Wilson's disease, mainly showing mild to moderate abnormality in heterozgous relatives,the abnormality rate of EEG was 60%, EEG changes of heterozgous showed dependance on ages. The main changes of EEG in Wilson's disease group and heterzygotes group were diffuse slowing of the basic rhythm,paroxysmal slow waves and epileptiform discharges,etc.Conclusion:EEG might be an useful indicator in judging the presence of subclinical CNS damage in patients with hepatic damage and without symptoms. The EEG changes of heterzggotes are closely related to the ages. Abstract Wilson's disease;Electroencephalogram;Heterozygotes
