Cohort study of infantile epileptic spasms syndrome: etiological analysis and treatment of corticosteroids

作者全名："Jiang, Yu; Zou, Nan; Luo, Yuanyuan; Cheng, Min; Liao, Shuang; Hong, Siqi; Liang, Xiaohua; Zhong, Min; Li, Tingsong; Jiang, Li"

作者地址："[Jiang, Yu; Zou, Nan; Zhong, Min; Li, Tingsong] Chongqing Med Univ CHCMU, Dept Rehabil, Childrens Hosp, Chongqing, Peoples R China; [Jiang, Yu; Zou, Nan; Luo, Yuanyuan; Cheng, Min; Liao, Shuang; Hong, Siqi; Liang, Xiaohua; Zhong, Min; Li, Tingsong; Jiang, Li] Minist Educ, Natl Clin Res Ctr Child Hlth & Disorders, Int Sci & Technol Cooperat Base Child Dev & Crit D, Chongqing Key Lab Pediat,Key Lab Child Dev & Disor, Chongqing, Peoples R China; [Jiang, Yu; Zou, Nan; Luo, Yuanyuan; Cheng, Min; Liao, Shuang; Hong, Siqi; Jiang, Li] CHCMU, Dept Neurol, Chongqing, Peoples R China; [Liang, Xiaohua] CHCMU, Dept Clin Epidemiol & Biostat, Chongqing, Peoples R China; [Li, Tingsong] Dept Rehabil, Bldg 8,Zhongshan Er Rd 136, Chongqing 400014, Peoples R China"

通信作者："Li, TS (通讯作者)，Dept Rehabil, Bldg 8,Zhongshan Er Rd 136, Chongqing 400014, Peoples R China."

来源：SEIZURE-EUROPEAN JOURNAL OF EPILEPSY

ESI学科分类：NEUROSCIENCE & BEHAVIOR

WOS号：WOS:000862844300008

JCR分区：Q2

影响因子：3

年份：2022

卷号：101

期号：　

开始页：120

结束页：126

文献类型：Article

关键词：Infantile epileptic spasms syndrome; Etiology; Classification; Prednisone; Corticosteroids

摘要："Background: Infantile epileptic spasms syndrome (IESS) is the most common type of severe epilepsy in infants. However, etiological frequency and optimized therapy, particularly corticosteroid regimen and dose, remain unknown.Methods: An ambispective study of an IESS-diagnosed cohort was conducted. Etiologies were evaluated based on the 2017 International League Against Epilepsy classification system. Patients received intravenous dexamethasone or methylprednisolone for 3-5 consecutive days, followed by usual-dose (2 mg/kg/d) oral prednisone for 60-90 days with tapering doses for 1-2 months or high-dose (4 mg/kg/d) oral prednisone for 9-11 days with tapering doses for 2-4 weeks. Treatment responses were compared between the usual and high-dose prednisone groups after propensity score matching. Correlation analysis between treatment responses and underlying etiology was performed.Results: Of the 441 included participants, 218 (49.4%) cases had proven etiologies. The most common etiology of IESS was acquired-structural (23.6%), followed by genetic (15.4%) and congenital-structural (7.0%). Hypoxicischemic encephalopathy (55, 52.8%) was the most common acquired-structural etiology. Among the 242 patients administered corticosteroids, 95 received usual-dose oral prednisone and 147 received high-dose oral prednisone. After propensity score matching, 54 patients were included in the usual-dose and high-dose groups, respectively, and treatment effectiveness was compared. There were no significant differences in seizure freedom at days 13-14 (55.6% vs. 51.9%, p = 0.700) and continued seizure freedom between days 14-42 (29.6% vs. 38.9%, p = 0.311) post corticosteroid administration between the usual- and high-dose prednisone groups. The proportion of children achieving seizure cessation at days 13-14 (chi 2 = 1.470, p = 0.698) and days 14-42 (chi 2 = 0.928, p = 0.836) was similar in the different etiological subgroups. Unknown etiological group showed significantly higher resolution of hypsarrhythmia than other etiological groups (chi 2 = 10.761, p = 0.009). Both usual-dose and high-dose group showed tolerance to full-dose corticosteroids and similar adverse events over the observation period.Conclusion: IESS etiology was primarily related to structural causes. Clinical response in short-term follow-up was independent of prednisone dosage and underlying etiology. Better EEG responses may occur in patients with unknown etiology."

基金机构：Joint Project of Chongqing Health Commission and Science and Technology Bureau [2022GDRC006]; CQMU Program for Youth Innovation in Future Medicine [W0031]

基金资助正文：Funding This study was supported by the Joint Project of Chongqing Health Commission and Science and Technology Bureau (2022GDRC006) and CQMU Program for Youth Innovation in Future Medicine (W0031) .