线粒体脑肌病伴高乳酸血症和卒中样发作的癫痫发作及脑电图特点分析  

Epileptic seizures and electroencephalographic findings in patients with mitochondrial myopathy encephalopathy, lactic acidosis and stroke-like episodes

作　　者：张晓[1] 王朝霞[1] 刘凤君[1] 邓馨[1] 于磊[2] 王薇薇[1] 袁云[1] 

机构地区：[1]北京大学第一医院神经内科,100034 [2]北京大学第一医院影像科,100034

出　　处：《中华神经科杂志》2014年第5期336-340,共5页Chinese Journal of Neurology

基　　金：科技部“十二五”重大专项课题资助项目（2011ZX09307-001-07）

摘　　要：目的 探讨线粒体脑肌病伴高乳酸血症和卒中样发作（MELAS）患者的癫痫发作及脑电图特点.方法 收集2007年11月至2013年8月于北京大学第一医院确诊的44例MELAS患者,所有患者均行脑电图及头MRI检查,总结不同时期（卒中样发作期及非发作期）的癫痫发作类型和脑电图改变特点并对其进行统计学分析.结果 44例MELAS患者中有39例（88.6％）出现癫痫发作,26例（66.7％）有1种以上发作类型.卒中样发作期癫痫部分发作患者22例、全面发作患者30例、癫痫持续状态17例;非卒中样发作期癫痫部分发作患者7例、全面发作患者14例、癫痫持续状态2例.简单部分性发作、全面性强直阵挛发作、癫痫持续状态在卒中样发作期和非发作期的出现率分别为47.7％（21/44）、68.2％（30/44）、38.6％（17/44）和13.6％（6/44）、27.3％（12/44）、4.5％（2/44）,差异具有统计学意义（x2=12.022、14.758、15.103,P=0.001、0.000、0.000）.44例患者脑电图结果全部异常,脑电图中α频率慢化、弥漫δ或θ波、癫痫样放电在卒中样发作期的出现率分别为6.8％（3/44）、43.2％ （19/44）和25.0％ （11/44）,在非卒中样发作期出现率分别为31.8％ （14/44） 、59.1％（26/44）和22.7％ （10/44）,α频率慢化在卒中样发作期和非发作期的差异具有统计学意义（x2=8.822,P=0.003）.结论 MELAS患者在卒中样发作期内各种类型的癫痫发作均明显增多,但癫痫波在非卒中样发作期中同样常见.Objective To investigate the features of epileptic seizures and eletroencepalogram （EEG） in patients with mitochondrial myopathy encephalopathy,lactic acidosis and stroke-like episodes （MELAS）.Methods Forty-four patients with MELAS were diagnosed at the First Hospital of Peking University from November 2007 to August 2013.EEG and head MRI were performed on all patients.The types of epileptic seizure and EEG changes were compared between patients in and outside stroke-like episodes.Results Epileptic seizures occurred in 39 of 44 patients （88.6%） with MELAS,while multitype epileptic seizures were presented in 26 cases （66.7%）.In stroke-like episodes,22 patients presented with partial seizures,30 with generalized seizures and 17 with status epilepticus.In nonstroke-like episodes,7 patients presented with partial seizures,14 with generalized seizures and 2 with status epilepticus.The frequency of partial seizures,generalized tonic-clonic seizures,status epilepticus were 47.7% （21/44）,68.2% （30/44）,38.6% （17/44） in stroke-like episodes and 13.6% （6/44）,27.3% （12/44）,4.5% （2/44） in nonstroke-like episodes,which have statistical significance （x2 =12.022,14.758,15.103;P =0.001,0.000,0.000,respectively）.Abnormal EEGs appeared in all patients.The rates of slow alpha frequency,diffuse δ or θ wave,epileptic discharges were 6.8% （3/44）,43.2% （19/44）,25.0%（11/44） in stroke-like episodes and 31.8% （14/44）,59.1% （26/44）,22.7% （10/44） in nonstroke-like episodes,respectively.Slow alpha frequency were significantly different between patients in and outside stroke-like episodes （x2 =8.822,P =0.003）.Conclusions Epileptic seizures with different types are more common during stroke-like episodes in patients with MELAS.While the rates of epileptic discharges are also common outside the stroke-like episodes.

关 键 词：MELAS综合征 癫痫 脑电描记术 

分 类 号：R742.1[医药卫生—神经病学与精神病学]