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目的通过总结儿童睡眠中癫癎性电持续状态(ESES)的疾病谱、临床表现及甲泼尼龙治疗的疗效和不良反应,以扩展对ESES疾病谱的了解,探讨甲泼尼龙在ESES治疗中的作用。方法收集2007年6月-2009年8月中南大学湘雅医院儿科脑电图监测过程中发现ESES现象的患儿21例,总结分析其临床资料,并予甲泼尼龙序贯泼尼松治疗,定期随访,并复查脑电图,根据治疗前后临床表现、脑电图变化及药物不良反应进行疗效评价。结果21例中癫癎综合征10例(47.6%),症状性8例(38.1%),病因不明3例(14.3%)。在甲泼尼龙治疗后第21天复查EEG,其中15例(71.4%)ESES完全控制;3例(14.3%)有效,3例(14.3%)无效,6例仍有癫癎发作;6个月时ESES的复发率为36.8%(7/19例),2例仍有癫癎发作;1 a时ESES的复发率为33.3%(5/15例),1例激素依赖,1例复发后再次予甲泼尼龙治疗仍然有效。结论ESES可以出现在多种癫癎性脑病、儿童良性癫癎综合征、各种脑发育畸形、孤独症、继发性脑损伤及脑积水外科手术后。甲泼尼龙对改善ESES短期具有显著疗效,并可以协助控制癫癎发作、促进认知和语言发育,但复发率高。甲泼尼龙对ESES患儿远期预后的影响尚需大规模、多中心的研究证实。
Objective To expand the understanding of ESES disease spectrum by summarizing the spectrum of disease, clinical manifestations, and efficacy and adverse reactions of epileptic electroencephalography (ESES) in childhood sleep, and to explore the role of methylprednisolone in ESES treatment effect. Methods Twenty-one children with ESES were detected during the EEG monitoring of Xiangya Hospital of Central South University from June 2007 to August 2009. The clinical data of the patients were collected. The patients were given methylprednisolone sequential prednisone, Regular follow-up, and review EEG, according to the clinical manifestations before and after treatment, EEG changes and adverse drug reactions to evaluate the efficacy. Results 21 cases of epilepsy syndrome in 10 cases (47.6%), symptomatic in 8 cases (38.1%), etiology in 3 cases (14.3%). EEG was reviewed on day 21 after methylprednisolone treatment, of which 15 (71.4%) were completely controlled by ESES; 3 (14.3%) were effective, 3 (14.3%) were ineffective, 6 had epileptic seizures; The ESES recurrence rate was 36.8% (7/19 cases) and 2 cases still had epileptic seizures. The ESES recurrence rate was 33.3% (5/15 cases) at 1 year, 1 case was hormone-dependent and 1 case relapsed To methylprednisolone treatment is still valid. Conclusion ESES can appear in a variety of epileptic encephalopathy, childhood benign epilepsy syndrome, various brain developmental deformities, autism, secondary brain injury and hydrocephalus after surgery. Methylprednisolone has a significant short-term effect on ESES and may help control epileptic seizures and promote cognitive and speech development, but with a high recurrence rate. The impact of methylprednisolone on the long-term prognosis of children with ES ES is yet to be confirmed by large, multicenter studies.