Yang Zhixian, Li Hui, Xue Jiao, Qian Ping, Liu Xiaoyan, Zhang Yuehua
Department of Pediatrics, Peking University First Hospital, Beijing, China.
Brain Dev. 2017 Nov;39(10):828-835. doi: 10.1016/j.braindev.2017.06.007. Epub 2017 Jul 13.
To investigate the general characteristics and the category of myoclonic epilepsy in infancy (MEI) with or without afebrile generalized tonic-clonic seizures (GTCS).
Thirty-three children were retrospectively recruited from approximately 42,814 video-electroencephalogram (VEEG) recordings monitored in our department over last nearly 10years. Myoclonic seizures (MS) must be identified by VEEG in all patients. The clinical, EEG features and outcome were analyzed among these patients.
The 33 patients (25 boys and 8 girls) were divided into three groups: 11 patients with typical MEI; 16 patients with MEI experienced afebrile GTCS before MS onset; and 6 patients with MEI presented afebrile GTCS occurring concurrently with MS. No significant differences were found among the three groups, including gender distribution, family history, personal history of febrile seizures, the age at seizure onset and control, the duration of MS, the interval between age at onset and seizure control, the age at EEG normalization, the interval between seizure onset age and EEG normalization age and normal psychomotor development at the end of follow-up. More patients in group two and group three were controlled by two or three kinds of antiepileptic drugs compared with those in group one.
In this study, three groups of patients had similar clinical, EEG features and outcome. Afebrile GTCS was associated with a stronger cortical hyperexcitability. It was worth considering whether MEI with preceding or concurrent afebrile GTCS should be recognized as subgroups or different epileptic syndromes independent of MEI.
探讨伴或不伴无热全身性强直阵挛发作(GTCS)的婴儿肌阵挛癫痫(MEI)的一般特征和类别。
回顾性纳入过去近10年在我科监测的约42,814份视频脑电图(VEEG)记录中的33例患儿。所有患者均须经VEEG确诊肌阵挛发作(MS)。分析这些患者的临床、脑电图特征及转归。
33例患者(25例男孩,8例女孩)分为三组:11例典型MEI患者;16例MEI患者在MS发作前出现无热GTCS;6例MEI患者的无热GTCS与MS同时出现。三组之间在性别分布、家族史、热性惊厥个人史、发作起始及控制年龄、MS持续时间、发作起始至控制的间隔时间、脑电图正常化年龄、发作起始年龄至脑电图正常化年龄的间隔时间以及随访结束时正常的精神运动发育等方面均未发现显著差异。与第一组相比,第二组和第三组中更多患者需用两种或三种抗癫痫药物才能控制发作。
本研究中,三组患者具有相似的临床、脑电图特征及转归。无热GTCS与更强的皮质兴奋性过高有关。伴前驱或并发无热GTCS的MEI是否应被视为MEI的亚组或独立的不同癫痫综合征值得考虑。