儿童肌阵挛失神癫痫脑电图五例  

Electroencephalogram of myoclonic absence epilepsy in five children

作　　者：赵绚 贾宇 张礼萍[1] 王诗雨 魏荣[1] 戚小红[1] 王玉平[2] Zhao Xuan;Jia Yu;Zhang Liping;Wang Shiyu;Wei Rong;Qi Xiaohong;Wang Yuping(Department of Pediatrics,Xuanwu Hospital,Capital Medical University,Beijing 100053,China)

机构地区：[1]首都医科大学宣武医院儿科,北京100053 [2]首都医科大学宣武医院神经内科,北京100053

出　　处：《脑与神经疾病杂志》2023年第3期133-137,共5页Journal of Brain and Nervous Diseases

基　　金：北京市医院管理中心儿科学科协同发展中心儿科专项一般项目(XTYB201810)。

摘　　要：目的总结儿童肌阵挛失神癫痫(MAE)的临床表现,视频脑电图(VEEG)特征,治疗方案及预后转归等特点。方法分析2010年1月至2019年12月于首都医科大学宣武医院收治的5例MAE患儿的临床资料和特点。结果5例患儿中2例男孩,3例女孩。发病年龄中位数9(3~11)岁。临床表现中5例患者均以MA为突出表现,其中4例为双上肢节律性肌阵挛抽动,另1例为口轮匝肌及双上肢同时受累。发作具有突发突止的特点且发作频繁,每日可发作数次至10余次。住院期间所有患儿均进行VEEG检查,记录到5例患儿均存在典型的双侧对称同步的3Hz棘慢复合波阵发,同步肌电记录到与肌阵挛有锁时关系的肌电爆发。5例患儿在诊断明确后进行抗癫痫治疗,所有患儿均在治疗后癫痫发作频率减少,随访2~6年,5例患儿均无临床发作,智力运动发育正常,EEG复查均无癫痫样异常放电。结论MAE以肌阵挛失神为突出表现,EEG特点为双侧对称同步的3Hz棘慢复合波阵发,同步肌电图记录到与肌阵挛有锁时关系的肌电爆发。尽早合理的应用抗癫痫药物可有效的控制患儿发作,改善预后。Objective This study aimed to summarize the clinical manifestations,video electroencephalogram(VEEG)features,imaging findings,treatments,and prognosis of children with myoclonic absence epilepsy(MAE).Methods The clinical data of 5 children with MAE admitted to Xuanwu Hospital,Capital Medical University from August 2010 to August 2019,were collected and analyzed,retrospectively.Results Among 5 cases with MAE,3 were male and 2 were female.The median onset age was 9(3-11)years.All patients developed MAE as the main characteristic during the disease course.Four patients had rhythmic myoclonus on bilateral upper limbs and the rest had orbicularis oris muscles involvement characterized as onset began and ended abruptly.The frequency of seizures was several times to more than 10 times per day.During the hospitalization,all 5 patients were recorded by VEEG,the ictal EEG showed bilateral synchronous and symmetrical 3Hz spike and slow-wave complex,and the strict relation with myoclonus was observed on electromyography.All patients were given anti-seizure medications(ASMs)after diagnosis.After followed up for 3 to 6 years,all patients achieved seizure-free,and no epileptic discharge was observed in the EEG review.Conclusion MAE was rarely reported in patients with the epileptic syndrome.Detailed analysis on the features of seizures and VEEG needed to be emphasized in clinical works.Reasonable application of ASMs could reduce the frequency of seizures and improve the prognosis of patients.

关 键 词：肌阵挛失神癫痫 3Hz棘慢复合波 肌电描记 丙戊酸钠 预后 

分 类 号：R742.1[医药卫生—神经病学与精神病学]