Villeneuve N, Soufflet C, Plouin P, Chiron C, Dulac O
Service de neuropédiatrie, hôpital Saint-Vincent-de-Paul, Paris, France.
Arch Pediatr. 1998 Jul;5(7):731-8. doi: 10.1016/s0929-693x(98)80053-5.
Steroids (hydrocortisone or ACTH) still remain the usual treatment for infantile spasms (IS). However, since 1990, some authors have reported the efficacy of vigabatrin (VGB), especially in cases related to tuberous sclerosis.
Seventy children with infantile spasms were treated by VGB first line monotherapy. Modalities of treatment and monitoring were the same for all children. VGB was given at the daily dose of 100 mg/kg during 1 week. If spasms persisted, the daily VGB dose was increased to 150 mg/kg during a 2nd week. In case of persistence of IS, on the 15th day, hydrocortisone was then added to VGB. Of the 70 infants, 39 were symptomatic and 31 cryptogenic.
On VGB, 37 children (54%) stopped having IS within a mean 3.5 days. Response to VGB was different according to etiology. Among cryptogenic cases, 22 infants (71%) definitively stopped having spasms and only one relapsed. Among symptomatic cases, only 15 infants (38%) stopped having IS, and half (8/15) relapsed. VGB mean daily dose at cessation of spasms was 114 mg/kg. Side effects were transient drowsiness (27%) and agitation (12%). Mean follow-up was 10 months (1-24 months). Seventy-five percent of the infants presenting with a focus of spike after the 1st month of treatment relapsed.
Infants with cryptogenic spasms have a good response to VGB monotherapy. When mental retardation is noticed before IS, and MRI is normal, there is no efficacy. In these cases, the best treatment seems to be prolonged corticotherapy associated with VGB.
类固醇(氢化可的松或促肾上腺皮质激素)仍然是婴儿痉挛症(IS)的常用治疗方法。然而,自1990年以来,一些作者报告了氨己烯酸(VGB)的疗效,尤其是在与结节性硬化症相关的病例中。
70例婴儿痉挛症患儿接受VGB一线单一疗法治疗。所有患儿的治疗和监测方式相同。VGB在1周内每日剂量为100mg/kg。如果痉挛持续存在,在第2周将VGB每日剂量增加至150mg/kg。如果婴儿痉挛症持续存在,则在第15天将氢化可的松添加到VGB治疗中。这70例婴儿中,39例为症状性,31例为隐源性。
使用VGB治疗后,37例患儿(54%)在平均3.5天内停止发作婴儿痉挛症。根据病因,对VGB的反应有所不同。在隐源性病例中,22例婴儿(71%)最终停止痉挛发作,只有1例复发。在症状性病例中,只有15例婴儿(38%)停止发作婴儿痉挛症,其中一半(8/15)复发。痉挛停止时VGB的平均每日剂量为114mg/kg。副作用为短暂嗜睡(27%)和烦躁不安(12%)。平均随访时间为10个月(1 - 24个月)。治疗第1个月后出现尖波灶的婴儿中有75%复发。
隐源性痉挛婴儿对VGB单一疗法反应良好。如果在婴儿痉挛症之前就已发现智力发育迟缓且MRI正常,则VGB治疗无效。在这些情况下,最佳治疗方法似乎是延长皮质激素治疗并联合VGB治疗。