Jiang Yu, Zou Nan, Luo Yuanyuan, Cheng Min, Liao Shuang, Hong Siqi, Liang Xiaohua, Zhong Min, Li Tingsong, Jiang Li
Department of Rehabilitation, Children's Hospital of Chongqing Medical University(CHCMU), Chongqing, China; Ministry of Education Key Laboratory of Child Development and Disorders, International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of Pediatrics, National Clinical Research Center for Child Health and Disorders, Chongqing, China; Department of Neurology, CHCMU, Chongqing, China.
Ministry of Education Key Laboratory of Child Development and Disorders, International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of Pediatrics, National Clinical Research Center for Child Health and Disorders, Chongqing, China; Department of Neurology, CHCMU, Chongqing, China.
Seizure. 2022 Oct;101:120-126. doi: 10.1016/j.seizure.2022.07.019. Epub 2022 Aug 3.
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.
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.
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%). Hypoxic-ischemic 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 (χ = 1.470, p = 0.698) and days 14-42 (χ = 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 (χ = 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.
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.
婴儿痉挛症综合征（IESS）是婴儿期最常见的严重癫痫类型。然而，病因频率和优化治疗方案，特别是皮质类固醇的治疗方案和剂量，仍不明确。
对一个诊断为IESS的队列进行前瞻性和回顾性研究。根据2017年国际抗癫痫联盟分类系统评估病因。患者连续3 - 5天接受静脉注射地塞米松或甲泼尼龙，随后接受常规剂量（2mg/kg/d）口服泼尼松60 - 90天，并在1 - 2个月内逐渐减量，或接受高剂量（4mg/kg/d）口服泼尼松9 - 11天，并在2 - 4周内逐渐减量。在倾向评分匹配后，比较常规剂量和高剂量泼尼松组的治疗反应。对治疗反应与潜在病因进行相关性分析。
在纳入的441名参与者中，218例（49.4%）病因已明确。IESS最常见的病因是获得性结构性病因（23.6%），其次是遗传性病因（15.4%）和先天性结构性病因（7.0%）。缺氧缺血性脑病（55例，52.8%）是最常见的获得性结构性病因。在242例接受皮质类固醇治疗的患者中，95例接受常规剂量口服泼尼松，147例接受高剂量口服泼尼松。倾向评分匹配后，常规剂量组和高剂量组分别纳入54例患者，并比较治疗效果。常规剂量和高剂量泼尼松组在使用皮质类固醇后第13 - 14天无癫痫发作率（55.6%对51.9%，p = 0.700）和第14 - 42天持续无癫痫发作率（29.6%对38.9%，p = 0.311）无显著差异。在不同病因亚组中，第13 - 14天（χ = 1.470，p = 0.698）和第14 - 42天（χ = 0.928，p = 0.836）实现癫痫停止发作的儿童比例相似。病因不明组的高峰节律紊乱缓解率显著高于其他病因组（χ = 10.761，p = 0.009）。在观察期内，常规剂量组和高剂量组对全剂量皮质类固醇均有耐受性，且不良事件相似。
IESS病因主要与结构性原因有关。短期随访中的临床反应与泼尼松剂量和潜在病因无关。病因不明的患者可能有更好的脑电图反应。
