左乙拉西坦添加治疗大田原综合征的疗效

Efficacy of Levetiracetam as Adjunctive Therapy in Patients with Ohtahara Syndrome

目的了解左乙拉西坦(LEV)添加治疗大田原综合征(OS)的疗效。方法采用开放性自身对照研究,对确诊OS且当抗癫药物治疗无效的患儿给予添加LEV口服治疗。共入组11例。男7例,女4例;年龄29～87 d。治疗剂量从10 mg·kg-1·d-1开始,每1～2周日剂量增加10 mg·kg-1,至维持剂量40～50 mg·kg-1·d-1,分2次口服。治疗前1周和治疗后1个月及每3个月分别评价临床发作及视频脑电图。治疗期间检测肝肾功能及血常规,密切随访不良反应。结果 1例(9.1%)因出现明显的烦躁症状,在服药2周停药,停药2 d后烦躁症状完全缓解;其余10例患儿平均随访时间16.3个月(7～28个月)。1例(9.1%)发作完全控制,7例(63.6%)发作减少>50%,2例(18.1%)无效,总有效率为72.7%。6例患儿(54.5%)精神运动发育滞后有不同程度减轻。坚持服药的10例患儿均未出现明显不良反应,治疗期间未见肝、肾功能及血常规有异常改变。结论 LEV添加治疗OS具有良好疗效及安全性,可以在临床中进一步推广,但尚需多中心随机双盲安慰剂对照试验进一步支持。

Objective To evaluate the efficacy of adjunctive levetiracetam （LEV） therapy in patients with ohtahara syndrome （OS）. Methods Eleven infants （7 male and 4 female） at age of 29 to 87 days with OS were enrolled. LEV was administered in 2 equal daily doses of 10 mg· kg-1. The dose was increased by 10 mg· kg-1 every 1 -2 weeks up to the target dose（40 -50 mg·kg-1·d-1）. Before and after the LEV treatment, the seizure frequency and video electroencephalogram were evaluated. In the follow -up period,the side effects were eva- luated in the blood tests and clinical symptoms. Results One patient（9.1% ） discontinued LEV treatment because of hyperexcitability at 2 weeks. During an average follow - up period of 16.3 months（7 -28 months） ,7 patients （63.6%） had 〉 50% decrease in seizure frequency and 1 patient（9.1% ） became seizure - free. Two children（ 18.1% ） developed into West syndrome and did not respond to the treatment. LEV treatment was effective in 8 （72.7%） patients in total. The psychomotor development of 6 patients（54.5% ） improved dramatically. No side effect was found in the rest of 10 patients. Conclusions LEV as adjunctive therapy is effective and well - tolerated in treating infants with OS. But this needs more muhicentre, randomized, double -blind. placebo- controlled trials to support.