Abstract: To summarize our experience in the clinical expression and EEG features of patients with juvenile myoclonic epilepsy (JME) in chidren. MethodsForty-four patients had been involved.Clinical data such as seizure type,cause of misdiagnosis,antiepileptic drugs,and EEG and sleep EEG had been analyzed. Results100%of 44 cases showed myoclonic jerks;16 cases(36.4%) also had typical absences;33 cases(75%) also had generalized tonic-clonic seizures;11 cases (25%) had all three seizure types;6 cases (13.6%) only had myoclonic seizures.13 cases had been diagnosed at the first time.31 cases were misdiagnosised.The positive rate for EEG was 50%，and for sleep EEG was 95.5%.39 cases were seizure free.7 cases (15.9%) occurred myoclonic epileptic status. ConclusionJME is a very common form of epilepsy.Physicians should be aware of its characteristics.With the help of sleep EEG，physicians can get correct diagnosis，and choose the reasonable antiepileptic drugs for better control.

Key words: Myoclonic jerks, Sleep, EEG, Generalized tonic-clonic seizure, Typical absences

摘要： 目的总结青少年肌阵挛性癫疒间(JME)的临床及脑电图特点。 方法对2000年11月至2004年11月就诊于复旦大学儿科医院的44例JME患儿的临床资料进行回顾性分析。 结果44例JME患儿100%出现全身性肌阵挛发作，16例(36.4%)伴有失神发作，33例(75%)伴有全身性强直阵挛性发作，11例(25%)3种发作均有，6例(13.6%)只有全身性肌阵挛发作。首次就诊即确诊为JME 13例，有31例曾误诊。清醒脑电图阳性率为50%，睡眠脑电图阳性率为95.5%，3例动态脑电图检查均阳性。JME患儿43例给予药物治疗，除1例死亡外，其中3例偶有肌阵挛性发作，余39例发作均控制。治疗过程中出现肌阵挛持续状态(JMES)者有7例，占15.9%。 结论JME是儿童时期较常见的癫疒间综合征，临床医生在诊断中要多询问病史，结合规范的睡眠及动态脑电图检查，以提高检出率，并合理选择抗癫疒间药物。

关键词: 青少年肌阵挛性癫疒间, 全身性强直阵挛性发作, 失神发作, 肌阵挛性发作, 睡眠脑电图, 丙戊酸钠