Faculty of Medicine, Department of Paediatrics, University of Colombo, Colombo, Sri Lanka.
Faculty of Medicine, Department of Community Medicine, University of Colombo, Colombo, Sri Lanka.
Pediatr Neurol. 2021 Jun;119:22-26. doi: 10.1016/j.pediatrneurol.2021.01.008. Epub 2021 Mar 8.
This article explores the role of initial treatment on control of spasms and other epilepsies at four years in children previously treated for West syndrome.
The Sri Lanka Infantile Spasm Study is a prospective clinical trial evaluating response to intra-muscular adrenocorticotropic hormone (ACTH) versus oral prednisolone. A previous report documented response through age 12 months. This article provides four-year follow-up data.
At age four years, 65 of the original 97 were available for follow-up; another 13 had died, and 19 moved and could not be contacted. Of the 65 children, 37 (57%) continued to have seizures and 28 were seizure free. In the 37 children with ongoing epilepsy, 32.4% continued to have spasms, either alone or in combination with other seizure types. The epilepsy types seen in these children were focal epilepsy (59.4%), mixed focal and generalized epilepsy (24%), generalized epilepsy only (10.8%), and uncertain (5%). The majority of those still having epilepsy (66.7%) were controlled on medication. There was no significant difference in the rate of epilepsy or spasms or their control by medication between those treated with ACTH or oral prednisolone. Spasm control at day 14 did not influence the four-year spasm or epilepsy outcome.
A majority of children diagnosed with West syndrome continued to have seizures at age four years, although most were controlled on antiseizure medication. The long-term risk of developing epilepsy or its control was the same, regardless of whether ACTH or prednisolone was initially used as treatment.
本文探讨了在曾接受 West 综合征治疗的儿童中,初始治疗对控制痉挛和其他癫痫发作的作用,随访时间为四年。
斯里兰卡婴儿痉挛症研究是一项评估肌内注射促肾上腺皮质激素(ACTH)与口服泼尼松龙疗效的前瞻性临床试验。之前的报告记录了至 12 个月龄时的反应。本文提供了四年随访的数据。
在四岁时,97 例原始患儿中有 65 例可进行随访;另外 13 例死亡,19 例搬迁无法联系。在 65 例儿童中,37 例(57%)仍有发作,28 例无发作。在持续癫痫发作的 37 例儿童中,32.4%仍有痉挛发作,无论是单独发作还是与其他发作类型同时发作。这些儿童的癫痫类型为局灶性癫痫(59.4%)、局灶性和全面性癫痫混合(24%)、全面性癫痫(10.8%)和不确定(5%)。大多数仍有癫痫发作的儿童(66.7%)通过药物治疗得到控制。接受 ACTH 或口服泼尼松龙治疗的儿童癫痫或痉挛发作的发生率及其药物控制率无显著差异。第 14 天痉挛控制情况对四年后痉挛或癫痫结局无影响。
大多数被诊断为 West 综合征的儿童在四岁时仍有发作,但大多数通过抗癫痫药物得到控制。无论最初使用 ACTH 还是泼尼松龙治疗,长期发生癫痫或控制癫痫的风险是相同的。