婴儿良性癫癎的临床观察和远期随访研究

目的 研究婴儿良性癫痫的发作特征,脑电图及治疗反应,探讨早期诊断方法。方法 对出生后3-24个月内起病,排除热性惊厥,症状性癫痫及发育异常的婴儿惊厥进行临床观察及寻像脑电图（VEEG）监测,并随访治疗效果和远期预后,结果 42例经2年以上随访确诊为婴儿良性癫痫,其中3例有良性婴儿惊厥家族史,19%惊厥伴有轻微腹泻,67%为短期内频繁发作,无癫痫持续状态,3例VEEG监测证实分别为起源于颞区,枕区及多灶性的部分性发作,发作间期脑电图背景正常,24%睡眠中有Rolandic区小棘波,39例接受抗癫痫单药治疗,平均用药时间9个月,3例未用药物治疗,起病1年内发作均消失,结论 起病早期具有以下特征应考虑有婴儿良性癫痫的可能;（1）起病年龄在3-12个月,不超过24个月,可有婴儿良性惊厥家族史;（2）发病前后精神运动发育正常;（3）发作无诱因,或仅有轻度腹泻等非特异性感染;（4）以部分性发作为主,可继发全身性发作,起病时发作可以很频繁,但无癫痫持续状态;（5）发作间期脑电图背景正常,无典型癫痫样放电,可有睡眠期Rolandic区小棘波;（6）神经影像学正常。

Clinical observation and long-term follow-up of benign infantile epilepsy

Objective To investigate clinical characteristics, EEG changes and therapeutic response of benign infantile epilepsy and to study the early diagnostic methods. Methods Clinical observation and Video-EEG monitoring were carried out in babies with convulsions at 3-24 months of age. In these children, febrile convulsion, symptomatic epilepsies and developmental abnormalities were excluded, and the therapeutic effect and long-term outcome were followed up. Results Forty-two babies were diagnosed to have benign infantile epilepsy by two-year follow-up. Three of them had familial history of benign infantile convulsions. Nineteen percent had mild diarrhea during the onset of convulsions, cluster seizures occurred during a short period in 67% of cases and no status epilepticus occurred. Video-EEG monitoring confirmed seizures originating from temporal, occipital or multifocal areas separately in 3 patients with partial seizures. Interictal EEG background was normal and there were Rolandic small spikes during sleep in 24% of patients. Thirty-nine patients were treated with single antiepileptic drugs and the mean treatment course was 9 months. Three cases did not take medicine. All the patients were seizure free within a year. Conclusion Benign infantile epilepsy should be considered when the following characteristics occur in early atage of the disease: (1) convulsions occurring between 3 to 12 month of age and not later than 24 months of age with or without familial history of benign infantile convulsion; (2) normal psychomotor development before and after convulsion occurs; (3) no evoked factors or only mild diarrhea; (4) majority of cases have partial seizures, or secondary generalized seizures. There are often cluster convulsions during the onset stage, but no status epilepticus; (5) normal EEG background and there may be Rolandic small spikes during sleep; (6) normal neuroimaging.