目的探讨托吡酯对儿童肌阵挛癫(JME)的临床疗效和耐受性。方法 JME患儿31例,随机分为治疗组16例,对照组15例。治疗组给予托吡酯0.5~1.0 mg·kg-1·d-1,po,qd,每周或每2周增加1.0~2.0 mg·kg-1·d-1,当加到完全控制发作剂量或目标剂量(5.0~8.0 mg·kg-1·d-1)时,可以每天分2次服药;对照组给予丙戊酸钠,初始剂量10~20 mg·kg-1·d-1,po,qd,每周或每2周增加5~10 mg·kg-1·d-1,当加到完全控制发作剂量(20~30 mg·kg-1·d-1)时,可以每天分2或3次服用。观察治疗6,10,12个月临床疗效及药物不良反应。结果治疗组患儿每日肌阵挛发作疗效为:经6个月治疗,完全控制6例,显效4例,有效、无效各3例;经10个月治疗,完全控制、显效各5例,有效2例,无效4例;经12个月治疗,完全控制6例,显效、有效各3例,无效4例。对照组患儿每日肌阵挛发作疗效为:经6个月治疗,完全控制6例,显效、有效、无效各3例;经10个月治疗,完全控制5例,显效、有效各3例,无效4例;经12个月治疗,完全控制7例,显效、无效各3例,有效2例。治疗组患儿认知功能智商值治疗前和治疗12个月分别为(98.81±6.95),(98.56±5.40)分。对照组患儿认知功能智商值治疗前和治疗12个月后分别为(95.73±5.76),(95.27±5.50)分。治疗组和对照组不能耐受不良反应分别有1,6例。结论托吡酯对JME疗效肯定,且不影响患儿认知能力,耐受性好。 Objective To investigate the clinical efficacy and tolerability of topiramate in children with myoclonic epilepsy (JME). Methods Thirty-one children with JME were randomly divided into treatment group (n = 16) and control group (n = 15). The treatment group was given topiramate 0.5 ~ 1.0 mg · kg-1 · d-1, po, qd, weekly or every 2 weeks increased by 1.0 ~ 2.0 mg · kg-1 · d-1, when added to complete control of seizure dose or target The patients in the control group were given sodium valproate at initial dose of 10-20 mg · kg-1 · d-1, po, qd at the dose of 5.0 ~ 8.0 mg · kg-1 · d-1 , Increased 5 ~ 10 mg · kg-1 · d-1 every week or every 2 weeks. When added to the complete control seizure dose (20 ~ 30 mg · kg-1 · d-1) Take it. The clinical efficacy and adverse drug reactions of 6, 10 and 12 months after treatment were observed. Results The curative effect of daily myoclonic seizures in treatment group was: after 6 months of treatment, 6 cases were completely controlled, 4 cases markedly effective, 3 cases effective and 3 cases ineffective. After 10 months of treatment, 5 cases were completely controlled and markedly effective, 2 cases were effective and 4 cases were ineffective. After 12 months of treatment, 6 cases were completely controlled, 3 cases were markedly effective and 3 cases were ineffective, and 4 cases were ineffective. The control group of children with daily myoclonus curative effect as follows: after 6 months of treatment, 6 cases were completely controlled, markedly effective, effective and ineffective in 3 cases; after 10 months of treatment, complete control of 5 cases, markedly effective and effective in 3 cases , 4 cases were ineffective; after 12 months of treatment, 7 cases were completely controlled, 3 cases were markedly effective and 3 cases were ineffective, and 2 cases were effective. The IQ of children in the treatment group before and 12 months of treatment were (98.81 ± 6.95) and (98.56 ± 5.40) points respectively. Control group children with cognitive function IQ before treatment and 12 months after treatment were (95.73 ± 5.76), (95.27 ± 5.50) points. The treatment group and the control group can not tolerate adverse reactions were 1,6 cases. Conclusion Topiramate had a positive curative effect on JME without affecting the cognition and tolerance of children.