Gastaut型儿童枕叶癫痫临床及脑电图特点分析

The clinical and electroencephalographic features of childhood occipital epilepsy of Gastaut

目的探讨Gastaut型儿童枕叶癫痫(COE-G)的临床及脑电图特点。方法随诊分析2004年6月至2008年6月遵义医学院附属医院儿科7例确诊为COE-G患儿的临床、脑电图特点及对治疗的反应。结果9岁左右起病,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。3例伴偏侧阵挛,仅1例泛化为全身发作:患儿均有日间发作,2例夜间也有发作,脑电图示枕区为主的后头部高幅痫性放电,常为闭眼诱发,睁眼抑制:1例对多种抗癫痫药物反应不佳,2例伴认知问题。结论COE-G起病较晚,较具确切特征表现,良性预后尚值得怀疑。

Objective To characterize the clinical features and electroencephalographic （EEG） features of childhood occipital epilepsy of Gastaut （COE-G）. Methods The clinical and EEG characteristics of 7 children who were diag-nosed as COE-G were identified and followed-up clinically, with sleep and awake EEGs. Results COE-G had a peak age of onset at about 9 years. Visual manifestations were the most common ictal event. Ictal deviation of the eyes associat- ed with ipsilateral turning of the head and migraine-like symptoms were frequent. Hemiconvulsions occurred in 3 chil- dren, and only one child had secondarily generalized tonic-clonic seizures （GTCS）. In all patients the seizures occurred while awake, and 2 also had seizures during sleep. EEG showed that the majority of the patients had occipital spike-wave discharges when the eyes were closed, which disappeared when the eyes were open. One child continued having frequent seizures despite antiepileptic drugs treatment and two cases had subtle cognitive deficits. Conclusion COE-G, a late onset childhood occipital seizures, is a distinctive epilepsy syndrome, and sheds some doubts on the prognosis and longterm evolution.