左乙拉西坦对促皮质素治疗失败婴儿痉挛症的疗效和安全性

Efficacy and Safety of Levetiracetam for Patients with West Syndrome Refractory to Adrenocorticotrophic Hormone Treatment

目的评价左乙拉西坦(LEV)对促皮质素(ACTH)治疗失败婴儿痉挛症的疗效和安全性。方法选择中南大学湘雅医院儿科2007年9月-2008年10月接受ACTH治疗的21例婴儿症挛症患儿,对其中8例ACTH治疗失败后进行序贯LEV治疗的婴儿痉挛症患儿进行开放性自身对照随访研究。依据下列标准判断疗效,(1)完全控制:发作频率减少100%,VEEG未见高度失律;(2)显效:发作频率减少≥75%,VEEG清醒状态下未见高度失律;(3)有效:发作频率减少≥50%且<75%,VEEG清醒状态下可见间断高度失律;(4)无效:发作频率减少<50%,VEEG未见明显改善;(5)加重:发作频度增加>25%,VEEG未见明显改善。结果 8例患儿均随访4～12个月。总有效率为87.5%(7/8例),4例(50.0%)完全控制,2例(25.0%)有效,显效、无效各1例(各12.5%)。5例出现不良反应,包括乏力3例,嗜睡、激惹2例,主要表现为激惹。不良反应主要出现在治疗4周内,未见肝肾功能损害或血液学改变等严重不良事件发生。结论 LEV能有效改善ACTH治疗失败的婴儿痉挛症患儿的癫发作,且具有良好的安全性。

Objective To evaluate the efficacy and safety of levetiracetam（LEV） therapy for patients with West syndrome refractory to adrenocorticotrophic hormone（ACTH） treatment.Methods In this self-controlled,open-label research,8 patients with West syndrome refractory to ACTH treatment admitted to the Xiangya Hospital,Central South University from Sep.2007 to Oct.2008 were treated with LEV sequential therapy.The baseline seizure frequency and long term videoelectroencephalogram（VEEG） monitored results were compared,the responses to LEV treatment were classified into 5 categories：seizure free（100% seizure control without hypsarryhthmia）;good（decreasing in seizure frequency more than 75 % without hypsarryhthmia in the awake stage VEEG）;minimal（decreasing in seizure frequency more than 50%,and less than 75% with fragmented hypsarryhthmia in the awake stage VEEG）;unmodified（less than 50% seizure reduction without VEEG improvement）;worsening（increasing seizure frequency to more than 25% without VEEG improvement）.Adverse events were assessed according to clinical observation and laboratory investigations.Results Eight cases were all given a 4-12 months follow-up visit.Seven patients（87.5%） were responders.Specifically,4 cases（50.0%） children became free of seizure,2 cases（25.0%） had a minimal response,1 case（12.5%） had a good response,and 1 case（12.5%） had an unmodified response.Adverse events included asthenia in 3 cases,somnolence and behavioral problems（mainly irritability）in 2 cases of patients during the first 4 weeks of treatment.The majority of adverse events were mild in severity and transient.There were no significant laboratory abnormalities in liver function,renal function,or hemato-logy.Conclusions LEV is safe and effective for West syndrome refractory to ACTH treatment.A prospective,controlled clinical trial of LEV in patients with West syndrome should be considered.