目的 分析Angleman综合征的临床、脑电图特点及分子遗传学检测。方法 回顾性分析4例Angleman综合征患者的临 床资料并复习文献。结果 4例均发育迟缓，有大嘴、枕部扁平、与环境无关的笑容等特征性面容，2例合并癫痫发 作。4例脑电图均有局灶或广泛的高-极高幅慢活动或节律，可夹杂棘波，并在不同部位间游走，睡眠期呈持续状态 发放。结论 脑电图检查有助于Angleman综合征的检出，可进行分子遗传学检查进一步确诊。

Objective To analyze the clinical manifestations, electroencephalography(EEG) and molecular genetic test of Angelman syndrome(AS). Methods The clinical data of four AS patients were analyzed retrospectively. Results Both four patients with delayed motor milestones, intellectual disability,and with dysmorphic facial features included big mouth,flat craniumskull, happy laugh incompatible with the environment,et al. Seizures occurred in two cases. The EEG manifested as rhythmic delta and theta in anterior, posterior and generalized areas mixing spikes, and the slow waves often migrate between the different sites. Conclusion EEG is necessary to diagnosis of AS, and molecular genetic examination can be applied for further confirmation.