Kankirawatana Pongkiat, Raksadawan Nattee, Balangkura Kampanad
Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
J Med Assoc Thai. 2002 Aug;85 Suppl 2:S778-83.
To determine the clinical outcome and side effects of vigabatrin (VGB) in the treatment of infantile spasms (IS) and its long-term outcome.
All children with IS treated with vigabatrin were studied. Clinical data regarding age of onset, duration of IS before therapy started, recurrence of IS, types of seizures that relapse, clinical outcome and side effects were monitored.
36 children (17 girls, 19 boys) with IS participated in the study. The mean age of onset of IS was 115.55 +/- 67.3 days old (range, 15 to 300 days). Six were cryptogenic IS and 30 were symptomatic IS. The etiologies of symptomatic IS in this study were tuberous sclerosis, hypoxic ischemic encephalopathy (HIE)/periventricular leukomalacia, porencephaly, partial agenesis of corpus callosum, hemimegalencephaly, cortical dysplasia, and microcephaly. 66.67 per cent (24 of 36) of patients responded to VGB within a mean 2.95 +/- 2.25 days (range, 1 to 7 days). In those who responded to VGB, 3 patients developed recurrent IS within 69.3 +/- 46.7 days (range, 30 to 121 days). Five patients developed epilepsy with different types of seizure during long-term follow-up. The mean duration of subsequent epilepsy after cessation of IS was 16.4 months (range, 5 months to 3 years 10 months). The mean duration of follow-up was 2.74 years (range, 1.09 years to 5.76 years). 10 patients were successfully weaned off VGB after a mean IS free period of 22.5 +/- 5.5 months (range, 12 to 27 months). Transient drowsiness was seen in 4 patients. Three patients had transient abnormal sleep patterns and irritability. Visual field abnormalities were not found but difficult to assess fully in this study.
VGB therapy has a high response rate for the control of IS and is well tolerated in most children. All patients who responded to VGB and were spasm free for more than one year were successfully weaned off VGB therapy. Because serious side effects such as visual field abnormalities are difficult to monitor, the authors propose that VGB could be withdrawn or switched to another AED after a spasm-free period of more than one year.
确定氨己烯酸(VGB)治疗婴儿痉挛症(IS)的临床疗效、副作用及其长期预后。
对所有接受氨己烯酸治疗的婴儿痉挛症患儿进行研究。监测有关发病年龄、治疗开始前婴儿痉挛症的持续时间、婴儿痉挛症复发情况、复发癫痫发作类型、临床疗效和副作用的临床数据。
36例婴儿痉挛症患儿(17例女孩,19例男孩)参与了该研究。婴儿痉挛症的平均发病年龄为115.55±67.3天(范围15至300天)。6例为隐源性婴儿痉挛症,30例为症状性婴儿痉挛症。本研究中症状性婴儿痉挛症的病因包括结节性硬化症、缺氧缺血性脑病(HIE)/脑室周围白质软化症、脑穿通畸形、胼胝体部分发育不全、半侧巨脑症、皮质发育异常和小头畸形。66.67%(36例中的24例)患者在平均2.95±2.25天(范围1至7天)内对氨己烯酸产生反应。在对氨己烯酸产生反应的患者中,3例在69.3±46.7天(范围30至121天)内出现婴儿痉挛症复发。5例患者在长期随访中出现不同类型发作的癫痫。婴儿痉挛症停止后后续癫痫的平均持续时间为16.4个月(范围5个月至3年10个月)。平均随访时间为2.74年(范围1.09年至5.76年)。10例患者在平均无婴儿痉挛症22.5±5.5个月(范围12至27个月)后成功停用氨己烯酸。4例患者出现短暂嗜睡。3例患者有短暂异常睡眠模式和易激惹。本研究未发现视野异常,但难以全面评估。
氨己烯酸治疗对控制婴儿痉挛症有较高的有效率,且大多数患儿耐受性良好。所有对氨己烯酸产生反应且无痉挛超过一年的患者均成功停用氨己烯酸治疗。由于难以监测视野异常等严重副作用,作者建议在无痉挛超过一年后停用氨己烯酸或换用另一种抗癫痫药物(AED)。