青少年肌阵挛性癫87例临床及治疗特点分析  

Features of clinical and therapy in juvenile myoclonic epilepsy: an analysis of 87 cases

作　　者：汤业磊[1] 张进[1] 丁美萍[1] 楼敏[1] 周祥琴[2] 

机构地区：[1]浙江大学医学院附属第二医院神经内科,杭州310009 [2]中国医学科学院中国协和医科大学北京协和医院神经内科

出　　处：《中华神经科杂志》2006年第5期316-319,共4页Chinese Journal of Neurology

基　　金：国家自然科学基金资助项目(30370386);浙江省医药卫生科学研究基金项目(2005B083)

摘　　要：目的分析青少年肌阵挛性癫的临床及治疗特点。方法对87例青少年肌阵挛性癫患者进行回顾性分析,包括家族史、热性惊厥史、发病规律、临床表现、脑电图、变化及治疗效果。结果10例(11.5%)患者亲属中有癫史,12例(13.8%)患者有热性惊厥。肌阵挛发作起病年龄(13.1±3.4)岁;伴发强直阵挛发作平均起病年龄(14.3±3.8)岁;伴失神发作平均起病年龄(10.0±3.3)岁。平均延误诊断时间2.2年。睡眠诱发、闪光诱发刺激脑电图检查可提高性放电检出阳性率。16例患者在抗癫治疗中出现了癫发作次数或强度的增加。给予丙戊酸钠单药治疗的45例(75%)患者癫发作可得到控制。结论临床工作中对该病认识不足,极易误诊,造成疾病治疗迁延不愈,甚至出现治疗中因抗癫药物选择不合理而引起癫发作增加;小剂量丙戊酸钠治疗有效。Objective To analyze clinical characteristics of patients with juvenile myoclonic epilepsy in China. Methods Eighty-seven patients were retrospectively studied in the aspects of family history, febrile seizures, clinical features, EEG, treatment effect. Results There was a female preponderance of incidence, In contrast to the earlier studies we found a high incidence of febrile seizures and a low incidence of family history, myoclonic seizures began at age of （13.1 ±3.4 ） years. That combined with generalized tonoclonic seizures began at age （ 14. 3 ±3. 8 ） years. Absence seizures began at age （ 10. 0±3, 3 ） years. The correct diagnosis was delayed at a mean of 2.2 years from onset of the disease, The incidence of abnormal EEG discharge could be enhanced by hyperventilation, photic stimulation and sleep, Sixteen patients who had received carbamazepine or phenytoin were experienced aggravation of seizures, Forty-five patients who received monotherapy with sodium valproate remained seizure-free in a follow-up longer than 0, 5 years. Conclusions Failure to recognize JME may result in uncontrolled seizures, and even aggravated of seizures by using antiepilepsy drugs. Effective treatment was achieved with small doses of sodium valproate.

关 键 词：癫痫 肌阵挛性 脑电描记术 丙戊酸 

分 类 号：R742.1[医药卫生—神经病学与精神病学] R644[医药卫生—临床医学]