伴中央颞区棘波小儿良性癫痫变异型临床分析

doi:10.7504/ek2014070611

摘要/Abstract

摘要：

目的探讨伴中央颞区棘波小儿良性癫痫（BECT）变异型临床表现、神经电生理特点、治疗及预后。方法对2001年10月至2012年12月北京大学第一医院儿科神经科门诊或住院的67例BECT变异型儿童临床资料、视频脑电图（VEEG）特点、治疗反应及预后进行2～10年的随访及总结。结果患儿首次出现癫痫发作的年龄为1岁6个月至10岁，出现BECT变异型临床表现的年龄为2岁6个月至10岁6个月。所有患儿脑电图（EEG）均表现为Rolandic区放电，其中25例（37.3%）为非快速眼动睡眠（NREM）期放电指数≥85%，42例（62.7%）NREM期放电指数为50%～＜85%。51例（76.1%）监测到负性肌阵挛发作。应用口服抗癫痫药物治疗10例发作控制，5例发作减少＞50%， 52例无效。单独应用口服抗癫痫药物无效患儿中 50 例联合应用皮质激素治疗， 2 ~ 10年随访，其中 21例（42.0%）发作控制， 25例（50.0%）发作减少＞50%，4例发作未控制。14例（28.0%）于甲基泼尼松龙治疗后1个月至1年2个月复发。激素治疗后6个月评估言语智商（VIQ）及操作智商（PIQ）均有提高（P＜0.05）。长期随访的学龄期患儿60例中30例（50.0%）学习成绩差，对其中25例进行神经心理评估，20例（80.0%）有不同程度认知功能受损。结论 BECT变异型除发作频繁及出现新的发作形式外，常伴认知受损、EEG恶化。多数患儿口服抗癫痫药物难以控制发作，激素治疗对大部分患儿有效，6个月治疗使临床症状、认知和EEG显著改善，但复发率较高。部分BECT变异型远期预后不理想，遗留认知功能下降及行为异常。

关键词: 伴中央颞区棘波小儿良性癫痫变异型, 脑电-临床特点, 治疗

Abstract:

Abstract： Objective To study the electro-clinical features and prognosis of the variants of benign childhood epilepsy with centro-temporal spikes（BECT）. Methods Sixty-seven patients with BECT variants were diagnosed in the hospital. They have been followed up for 2 to 10 years.The clinical information, including clinical manifestation，video-EEG，the response to the medical treatment， and the prognosis of the patients, was studied. Results The onset of the seizures in these patients was from 1 year and 6 months to 10 years， and the median age was 4 years and 5 months. The symptoms of BECT variants presented from 2 years and 6 months to 10 years and 6 months，the median age was 6 years. The EEGs of all patients showed abundance of spike and waves in Rolandic areas during wake-up and sleep.In 25 cases （37.3%），the index of discharge in non-rapid eye movements（NREM）was more than 85%.In 42 cases（62.7%），the index was between 50% and 85%.Epileptic negative myoclonus（ENM）was detected in 51 cases（76.1%）by the outstretched arm tests during the EEG recording.All the patients were treated with antiepileptic drugs（AED），only 10 cases were seizure free， and the seizure frequency decreased more than 50% in 5 cases.There were no responses in the other 52 cases.Then the corticosteroid was administered to 50 cases who were resistant to AED. Among them，21 cases were seizure free; in 25 cases，the seizure frequency decreased more than 50%， and only 4 cases had no response to this treatment. Fourteen cases（28%）relapsed 1 month to 1 year and 2 months after corticosteroid therapy.Six months after the corticosteroid therapy， verbal intelligence quotient（VIQ）and performance intelligence quotient（PIQ）increased（P＜0.05）.A long time follow-up of 60 school-aged children showed that 30 cases（50%）had poor school performance. Neuropsychological assessment was performed on 25 cases， and the cognitive decline was observed in 20 cases（80%）. Conclusion BECT variants should be considered in patients with BECT when the seizure frequency increases and new types of seizures present.EEG deterioration and cognitive decline often accompany simultaneously.Seizures are difficult to control with antiepileptic drugs.Corticosteroid therapy is effective in most of the patients. Clinical manifestations，EEG and cognition improve within 6 months after the therapy, but relapse rate is a little bit higher. The long term prognosis of BECT variants is unsatisfying in cognitive function and behavior.

Key words: variants of benign childhood epilepsy with centro-temporal spikes, electro-clinical features, treatment

中图分类号:

Ｒ72

施晓容，包新华，刘晓燕，侯绍荣，秦炯，吴希如. 伴中央颞区棘波小儿良性癫痫变异型临床分析[J]. 中国实用儿科杂志, DOI: 10.7504/ek2014070611.

SHI Xiao-rong，BAO Xin-hua，LIU Xiao-yan，HOU Shao-rong，QIN Jiong，WU Xi-ru.. Clinical analysis of the variants of benign childhood epilepsy with centro-temporal spikes.[J]. Acta Metallurgica Sinica, DOI: 10.7504/ek2014070611.

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