儿童睡眠中癫痫性电持续状态的临床分析及甲泼尼龙冲击治疗的随访研究

A clinical analysis of electrical status epilepticus during sleep in children and a follow-up study of methylprednisolone pulse therapy

摘要:

目的 探讨儿童睡眠中癫痫性电持续状态（ESES）的临床特点，以及甲泼尼龙冲击治疗对ESES患儿的疗效。方法 对78例ESES患儿的临床资料进行回顾性分析。其中56例抗癫痫药物治疗失败患儿给予甲泼尼龙15~20 mg/（kg·d）冲击治疗，每疗程用3 d，停3 d，期间予口服泼尼松1~2 mg/（kg·d），连续3个疗程，分析甲泼尼龙冲击治疗对消除ESES现象、控制临床发作及改善智力、行为方面的作用。结果 78例患儿平均癫痫发病年龄为6.8±2.4岁，首次出现ESES年龄为7.6±2.5岁。ESES患儿的智力发育落后于正常儿童，部分行为问题评分高于正常儿童。甲泼尼龙冲击治疗对癫痫发作控制的总有效率为73%（41/56）；治疗后脑电图棘慢波指数改善的总有效率为70%（39/56）。治疗后的言语智商、操作智商及总智商较治疗前均有提高，学习问题、冲动-多动及多动指数3个因子评分均明显降低（P < 0.05）。随访1年以后的总复发率为29%（11/38）。结论 ESES多在学龄期前后出现，可对患儿的智力及行为产生损伤。甲泼尼龙冲击治疗对控制ESES患儿临床发作及对脑电图放电的改善均有明显疗效，并可改善患儿的智力及行为发育，但有较高的复发率。

Abstract:

Objective To study the clinical features of electrical status epilepticus during sleep (ESES) in children, as well as the clinical effect of methylprednisolone pulse therapy in children with ESES. Methods A retrospective analysis was performed using the clinical data of 78 children with ESES. Among these children, 56 children who had had the failure of antiepileptic drugs were treated with methylprednisolone pulse therapy at a dose of 15-20 mg/(kg·d) for three courses. Each course of treatment was 3 days, followed by oral prednisone[1-2 mg/(kg·d)] for 3 days. The role of methylprednisolone pulse therapy in eliminating ESES, controlling clinical seizures, and improving intelligence and behaviors was analyzed. Results The mean age of onset of epilepsy in 78 children was 6.8±2.4 years, and the mean age for the first occurrence of ESES was 7.6±2.5 years. Compared with normal children, children with ESES had delayed intelligence development and higher scores of some behavior problems. Methylprednisolone pulse therapy had an overall response rate of 73% (41/56) on clinical seizures, and the overall response rate on electroencephalography (EEG)/spike-wave index was 70% (39/56) after treatment. There were significant improvements in verbal intelligence quotient, performance intelligence quotient and full intelligence quotient, and significant reductions in the scores of learning problems, impulse-hyperactivity and hyperactivity index after treatment (P < 0.05). The overall recurrence rate after 1-year follow-up was 29% (11/38). Conclusions ESES often presents around school age and impairs children's intelligence and behaviors. Methylprednisolone pulse therapy has a marked efficiency in reducing clinical seizures and EEG discharges in children with ESES and can improve intelligence and behavior development, but the recurrence rate remains high.