Objective To investigate the clinical, electroencephalograghy (EEG) features and prognosis in children with idiopathic occipital epilepsy of Gastaut (COE-G). Methods A retrospective review was conducted of 13 children patients who were diagnosed as idiopathic occipital epilepsy of gastaut (COE-G). All of them were treated with antiepileptic drugs according to the seizure typies. The prognosis were reviewed. The clinical feature and EEG were analysed. Results In total, 13 children (6 boys and 7 girls) with idiopathic childhood occipital epilepsy of gastaut were enrolled in this study. The median age at seizure onset was 8.7 years. The ictal clinical manifestations involve frequent diurnal visual seizures in all patients, elementary visual hallucinations in nine (69.2％), blindness or blurring of vision in seven (53.8％), headache in five (38.5％), and secondarily generalized tonic-clonic seizures in three (23.1％). Frequent deviation of eyes and head were common, 1 patient had seizures at night also. Interictal EEG showed high-amplitude spikes, spike and wave discharge on the unilateral or bilateral occipital regions which had often been induced by eyes closed, inhibited by eyes open. Posterior temporal spikes occured in 4 cases (30.1％), brief generalized discharges of spike-wave in 1 case (7.7％). Ictal EEG showed continuing low-amplitude sharp wave rhythm originated from one hemisphere of the occipital or posterior temporal lobe, with its amplitude increasing gradually and spread to the former head of the same hemisphere or lateral head of the contralateral hemisphere during progress. 11 cases (84.6％) had seizure free, in half of whom the medication was terminated in late adolescence. Most of them was treated monotherapy, some required combination therapy, 2 cases had poor response to multiple antiepileptic drugs, and 1 case had mild cognitive impairment. Conclusion COE-G had relatively late onset, characterized by prominent diurnal visual seizures, more frequent attacks, occipital spike discharges on EEG. Most of them have good outcome of treatment with antiepileptic drugs, and the prognosis is mostly good.%目的 探讨Gastaut型特发性儿童枕叶癫痫(COE-G)的临床特点、脑电图及预后情况.方法 对2003年6月至2010年3月深圳市儿童医院确诊为COE-G的13例患儿应用抗癫痫药物进行治疗,治疗前及治疗后做EEG检查,随诊分析其预后情况,并进行临床特点总结及脑电图资料分析.结果 发病年龄中位数为8.7岁,男6例,女7例,临床特征为较频繁的日间视觉症状,常有头眼偏斜及偏头痛症状.患儿均有日间发作,1例夜间也有发作.初级视幻觉9例(69.2%),失明或视力模糊7例(53.8%),头痛5例(38.5%),继发强直阵挛发作3例(23.1%).发作间期脑电图显示枕区为主的后头部高幅棘波、棘慢波放电,单侧或双侧枕区出现,左右可不同步,常为闭眼诱发,睁眼抑制;伴同侧后颞区棘波活动4例(30.1%),弥漫性棘波放电1例(7.7%).发作期脑电图为一侧枕区或后颞区起源的低幅棘波节律持续发放,波幅渐增高并向同侧前头部或对侧后头部扩散.大部分单药治疗有效,部分需要联合用药.11例(84.6 %)惊厥缓解,其中一半在青春期晚期终止药物.2例对多种抗癫痫药物反应不佳,1例有轻度认知障碍.结论 COE-G起病较晚,具较确切特征表现,日间视觉症状突出,大多发作频繁,脑电图以枕区棘波放电为特点,抗癫痫药物控制效果较好,预后大多良好.
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