青少年肌阵挛性癫疒间的临床与脑电图44例分析

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。

Clinical expression and EEG features of 44 cases of juvenile myoclonic epilepsy.

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.