甲基泼尼松龙冲击治疗Lennox-Gastaut综合征合并睡眠期癫癎性脑电持续状态

目的：LennoxGastaut综合征(LGS)为一种难治性的儿童癫癎综合征,部分患儿合并睡眠期癫癎性电持续状态(electricalstatusepilepticusduringsleep,ESES)增加了治疗的难度。该文观察了大剂量甲基泼尼松龙冲击治疗LGS合并ESES的近期疗效。方法：22例经视频脑电图或动态脑电图证实合并ESES的LGS患儿,给予甲基泼尼松龙冲击治疗以及口服泼尼松维持治疗。冲击治疗后两周评价临床发作并复查脑电图。结果：22例患者中,15例临床发作减少,16例癫癎样放电减少,未见严重不良反应。结论：甲基泼尼松龙冲击治疗可减少LGS患儿发作及脑电图放电,在抗癫癎药物治疗无效时可考虑使用。

Methylprednisolone pulse therapy of Lennox-Gastaut syndrome with electrical status epilepticus during sleep

Objective Lennox-Gastaut syndrome (LGS) is a refractory epilepsy in children. Some are accompanied by electrical status epilepticus during sleep (ESES)rendering treatment more difficult. This study examined the short-term effect of intravenous methylprednisolone (MP) pulse therapy on LGS with ESES. Methods Twenty-two children with LGS, who were confirmed with ESES by electroencephalograph (EEG), received MP intravenous pulse therapy followed by prednisone administration. Results Fifteen patients had partial remission of seizures (68.2%)and the EEG showed reduced epileptiform discharges in 16 cases (72.7%). No severe adverse effects were found. Conclusions MP pulse therapy can alleviate the symptoms and ESES in children with LGS. If anti-epileptic drug treatment is not effective, the therapy should be considered.