52例睡眠期癫痫性电持续状态患儿临床特征及预后分析

Clinical characteristics and prognosis analysis of 52 children with electrical status epilepticus during sleep

目的:探讨睡眠期癫痫性电持续状态(ESES)患儿的临床特征并评估治疗模式对预后的影响。方法:回顾性分析2015年12月—2021年12月武汉大学人民医院小儿神经科脑电图数据,提取脑电图结果显示为ESES的患儿资料,查阅His系统住院及门诊医疗随诊记录,收集患儿一般信息、临床表现、癫痫发作特征、发育评估、头颅影像学、脑电图结果、治疗方案等相关信息。结果:共有52名患儿(男29例,女23例)纳入研究。ESES诊断时的年龄范围为28~148月龄(中位年龄为72个月)。癫痫发作是最常见的首发临床表现(75%),所有患儿病程中均出现癫痫发作。脑部影像学MRI结果显示异常20例(38.5%)。27例患儿(51.9%)存在某种形式的发育迟缓。32例ESES消除患儿的有效治疗方案为糖皮质激素[或联合人血丙种免疫球蛋白(IVIG)]免疫治疗19例(59.4%),大剂量安定5例(15.6%),抗癫痫药物3例(12.5%),同时应用大剂量安定和抗癫痫药物5例(15.6%)。年龄越大(≥10岁)ESES达到缓解的趋势越明显。癫痫控制无发作率在ESES消除组较ESES无缓解组高(P=0.024)。不伴有发育迟滞的患儿ESES缓解率(73.9%)较发育迟滞组57.7%高(P=0.027)。ESES患儿脑部影像学正常组(23/30,76.7%)较异常组(9/19,47.4%)更易达到ESES消除(P=0.036)。结论:ESES患儿使用糖皮质激素(或联合IVIG)进行免疫调节治疗,可能有助于促进ESES消除;合并发育迟滞和颅脑结构影像学异常可能是ESES消除的不利因素;ESES消除与癫痫控制无发作和年龄增长(超过10岁)相关。

Objective:To investigate the clinical characteristics of children with electrical status epilepticus during sleep(ESES)and to evaluate the effect of treatment patterns on prognosis.Methods:A retro-spective analysis was conducted on the electroencephalogram database of the Department of Pediatric Neurology,Renmin Hospital of Wuhan University from December 2015 to December 2021.Data on children with ESES were extracted from the electroencephalogram results.Inpatient and outpatient medical follow-up records of His system were consulted.General information,clinical manifesta-tions,characteristics of seizures,developmental evaluation,cranial imaging,electroencephalogram results,treatment plans,and other relevant information were collected.Results:A total of 52 chil-dren(29 males and 23 females)were included in the study.The age at the time of diagnosis for ESES ranged from 28 to 148 months(72 months in median age).Epileptic seizures were the most common first clinical manifestation(75.0%).All children experienced seizures during the disease.Brain MRI showed abnormalities in 20 cases(38.5%).The number of 27 children(51.9%)had some form of de-velopmental retardation.The effective treatment regimen for 32 children with ESES resolution includ-ed glucocorticoid(or combined with human immunoglobulin(IVIG)immunotherapy)in 19 cases(59.4%),high-dose diazepam in 5 cases(15.6%),antiepileptic drugs in 3 cases(12.5%),and combi-nation with high-dose diazepam and antiepileptic drugs in 5 cases(15.6%).The older the age(≥10 years old),the more pronounced the trend of ESES resolution.The rate of seizure-free was higher in the ESES resolution group than in the ESES non-resolution group(P=0.024).The rate of ESES res-olution in children without developmental retardation(73.9%)was higher than that in the developmen-tal retardation group(57.7%)(P=0.027).The ESES resolution rate was higher in children with ES-ES in the normal brain imaging group(23/30,76.7%)compared to the abnormal group(9/19,47.4%)(P=0.036).Conclusion:Immunomodulatory therapy with glucocorticoids(or combined with IVIG)in children with ESES may help promote the resolution of ESES.The combination of de-velopmental retardation and imaging abnormalities of brain structures may be unfavorable factors for the resolution of ESES;ESES resolution is associated with seizure-free and increase in age(age>10 years).