氨己烯酸在结节性硬化症并婴儿痉挛症患儿中的治疗效果分析  

Analysis of Safety and Efficacy of Treatment of Vigabatrin in Infantile Spasms in Tuberous Sclerosis Complex Patients

作　　者：张新颜[1] 张帅[2] 邓欣[3] 姜涛[2,3] 

机构地区：[1]北京市海淀医院北京大学第三医院海淀院区急诊科,北京100080 [2]北京市神经外科研究所,北京100050 [3]首都医科大学附属北京天坛医院神经外科,北京100050

出　　处：《临床误诊误治》2016年第4期75-79,共5页Clinical Misdiagnosis & Mistherapy

基　　金：国家科技支撑计划项目(2013BAI09B03)

摘　　要：目的研究氨己烯酸在结节性硬化症（tuberous sclerosis complex,TSC）并婴儿痉挛症（infantile spasms,IS）患儿中的治疗效果及安全性。方法收集2013年2月—2015年7月TSC并IS且服用氨己烯酸（500-1750 mg/d）治疗患儿10例,对其治疗情况进行回顾性分析,观察其临床疗效及不良反应。结果本组累积应用氨己烯酸时间为1-49个月,中位数时间10个月。确诊后2例首选氨己烯酸治疗,余在其他药物治疗不满意后加用氨己烯酸。其中8例痉挛消失或接近消失,1例未好转,1例加用促肾上腺皮质激素后好转。6例1周内起效,15 d和60 d起效各1例,平均起效时间5 d。10例药物耐受性较好,不良反应轻微。结论氨己烯酸治疗TSC并IS具有较好的效果,起效时间快,耐受性好,可作为一线治疗药物。Objective To investigate the efficacy and safety of treatment of infantile spasms in tuberous sclerosis complex. Methods From February 2013 to July 2015,10 patients were given oral vigabatrin（ 500-1750 mg / d）. Seizure outcome was followed up retrospectively. We investigated medical history and electroencephalograph of all the cases. Results Range of epilepsy onset was 1 to 49 months with a median time of 10 months. 8 out of 10 patients were responders according to ＂ all-or-none＂ criteria. One nonresponder was seizure free after added treatment of ACTH. One responder had epilepsy recurrence six months later and was seizure-free after operation in our hospital. Most patients responded in one week except one patient in 15 days and another patient in 60 days. Side effects of vigabatrin were minor in our cases. Conclusion Vigabatrin is very effective and well-tolerable in treatment of TSC patients. It should be the first line treatment approach in infantile spasms in tuberous sclerosis complex.

关 键 词：结节性硬化症 痉挛 婴儿 氨己烯酸 癫痫 治疗结果 

分 类 号：R741[医药卫生—神经病学与精神病学]