论文部分内容阅读
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。
Objective To investigate the clinical manifestations, neuroelectrophysiological characteristics, treatment and prognosis of benign epilepsy (BECT) with central temporal spine in children. Methods The clinical data, video EEG (VEEG) features, treatment response and prognosis of 67 children with BECT with outpatient or inpatient department of Peking University First Hospital from October 2001 to December 2012 were studied in 2 to 10 years Follow-up and summary. Results The first onset of seizures in children was from 1 year and 6 months to 10 years old. The age of clinical manifestations of BECT variant appeared to be 2 years 6 months to 10 years 6 months. EEG in all children showed discharge in Rolandic area, of which discharge rate was 85% (37.3%) in non-REME stage, and 85% in 42 cases (62.7%). The discharge index in NREM stage was 50% ~ <85%. Fifty-one patients (76.1%) had negative myoclonic seizures. Oral antiepileptic treatment of 10 cases of seizure control, 5 cases of seizures reduced by> 50%, 52 cases were ineffective. Fifty patients with oral antiepileptic drug alone were treated with corticosteroids, and were followed up for 2 to 10 years. Twenty-one patients (42.0%) underwent seizure control, 25 (50.0%) patients had seizures reduced by> 50% and 4 patients had seizures control. Fourteen patients (28.0%) relapsed from 1 month to 1 year and 2 months after methylprednisolone treatment. Six months after hormone therapy, both verbal IQ (IQQ) and manipulative IQ (PIQ) were improved (P <0.05). Long-term follow-up of 60 cases of school-age children in 30 cases (50.0%) poor academic performance, of which 25 cases of neuropsychological assessment, 20 cases (80.0%) have varying degrees of cognitive impairment. Conclusions In addition to frequent episodes and new seizures, BECT variants often have impaired cognition and worsen EEG. Most children with oral antiepileptic drugs are difficult to control the attack, hormone therapy is effective in most children, 6 months of treatment to clinical symptoms, cognitive and EEG significantly improved, but the recurrence rate higher. Partial BECT variant long-term prognosis is not ideal, legacy cognitive decline and abnormal behavior.