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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JME13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。
Objective To summarize the clinical and electroencephalographic features of juvenile myoclonic epilepsy (JME). Methods The clinical data of 44 patients with JME who visited the Pediatric Hospital of Fudan University from November 2000 to November 2004 were retrospectively analyzed. RESULTS: Forty-four patients with JME developed systemic myoclonic seizures in 100%, 16 (36.4%) with absence seizures, 33 (75%) with generalized tonic clonic seizures and 11 (25% ) Had 3 episodes, and 6 (13.6%) had generalized myoclonic seizures. The first visit was diagnosed as JME13 cases, 31 cases had been misdiagnosed. The positive rate of sober EEG was 50%, the positive rate of sleep EEG was 95.5%, and 3 cases of EEG were positive. 43 cases of JME were given drug treatment, except one case died, of which 3 cases of occasional myoclonic seizures, more than 39 cases were controlled. Seven cases (15.9%) had myoclonus status (JMES) during treatment. Conclusion JME is a more common childhood epilepsy syndrome. Clinicians should ask more medical history in the diagnosis, combined with standard sleep and dynamic electroencephalography to improve the detection rate, and the rational selection of anti-epileptic drugs.