儿童睡眠中癫癎性电持续状态的疾病谱及甲泼尼龙治疗的疗效  

Disease Spectrum and Efficacy of Methylprednisolone Therapy on Electrical Status Epilepticus during Slow Sleep in Children

作　　者：彭镜[1] 尹飞[1] 柳固[1] 何芳[1] 李波[1] 

机构地区：[1]中南大学湘雅医院儿科,长沙410078

出　　处：《实用儿科临床杂志》2010年第3期203-206,共4页Journal of Applied Clinical Pediatrics

摘　　要：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 summarize the disease spectrum and clinical manifestation,and to evaluate the efficacy and adverse reaction of methylprednisolone therapy on electrical status epilepticus during sleep（ESES） in children.Methods The clinical data of children with ESES obtained from Xiangya Hospital during Jun.2007 to Aug.2009 were analyzed.All children were given methylprednisolone and prednisone sequential therapy.In comparison with baseline clinical characteristics and long term electroencephalogram（EEG） monitor result to eva-luate the efficacy and safety of the treatment.Adverse events assess combining clinical observation with laboratory investigations.Results Ten cases（47.6%）had epileptic syndrome,8 cases had symptomatic epilepsy and the remaining 3 cases had uncertain etiology.Long term EEG evaluation after 21 days on methylprednisolone showed ESES resolution in 15 cases（71.4%） accompany significant improvement in cognition and behavior,75% decrease of the spike-wave index in 3 cases（14.3%）,and lack of response in 3 cases（14.3%）.Relapse rate at the end of 6 months and 12 months was 36.8%（7/19）and 33.3%（5/15）respectively,1 case became hormone dependent,1 case was given methylprednisolone after relapsed and still effectual.Conclusions ESES evolve from several different etiologies including epileptic encephalopathies,benign partial epilepsy of childhood,brain malformation,autism,secondary brain insults and hydrocephalus.Most children have improvement in ESES,seizures,cognition and language after short term methylprednisolone treatment,but the relapse rate is really high.The long term effect of methylprednisolone treatment on children with ESES still needs to be confirmed by large multicenter studies.

关 键 词：睡眠中癫癎性电持续状态 甲泼尼龙 疾病谱 儿童 

分 类 号：R742.1[医药卫生—神经病学与精神病学]