伴强直性发作癫痫患儿的临床表现和脑电图特征

The clinical manifestations and features of electroencephalogram with tonic seizures

目的通过对67例伴强直性发作癫痫患儿的临床表现及视频脑电图(VEEG)特点分析,提高对该发作类型的诊断水平。结果收集河北省儿童医院神经内科67例伴强直性发作的癫痫患儿的病例资料,分析其临床表现和VEEG特征。结果 67例患儿均监测到明确的临床发作,其中清醒期发作19例(28%),睡眠期发作30例(45%),且容易出现在睡眠I期、II期。发作间期脑电图表现:①背景活动正常37例,慢化者15例;②广泛性棘波节律阵发,易出现在非快速眼动期(NREM期);③广泛性及多灶性慢波、棘慢波或多棘慢波阵发;④一侧或双侧前头部棘波、棘慢波或θ活动发放;⑤单侧或双侧Rolandic区棘慢波发放;⑥高度失律。发作期脑电图表现:①局灶起始的棘波节律发放;②广泛性棘波节律发放;③广泛性慢波阵发,其上复合或其后跟随棘波节律;④广泛性4~6Hz棘慢波发放→广泛性棘波节律阵发;⑤广泛性低波幅棘波节律发放→广泛性高波幅棘慢波阵发。以上表现形式有时会组合出现于同一例患者中。发作持续时间与背景活动的关系:发作持续约1~8s者39例(39/67,58.2%),背景活动慢化者4例(4/39,10.3%);发作持续8~15s,甚者更长者(15s)28例(28/67,41.7%),背景活动慢化者11例(11/28,39.3%)。67例患者随访研究1年,最终诊断为:8例(11.9%)诊断为婴儿痉挛征,7例(10.4%)诊断为Lennox-Gastaut综合征(LGS),3例(4.4%)诊断为额叶癫痫,15例(22.3%)诊断为伴有中央颞区棘波的儿童良性癫痫(BECT),34例(50.7%)仅停留在发作类型的诊断层面。结论强直性发作可单独出现,也可出现在多种癫痫综合征中;VEEG可监测患儿发作期临床表现及脑电图异常波形,为临床诊断及鉴别诊断提供理论依据。

Objective Improve the understanding of seizures in children by analyzing the clinical manifestations and features of video electroencephalogram(VEEG) in 67 children with tonic seizures. Methods Data of 67 cases of tonic seizures were collected from our department, and their clinical manifestations and VEEG characteristics were retrospectively analyzed. Results 67 cases were detected with clear clinical seizures, which 19 cases(28%) in awake episodes, in sleeping stage were 30 patients(45%) appearing in sleep I, II.The manifestations of EEG during the interictal period include: ① 37 cases of normal background activity, 15 cases of moderated;② the paroxysmal generalized spike wave rhythm;③ wide-spread and multi-focal high-extremely high amplitude slow wave, spike slow wave or multi-spike slow wave array;④ one or bilateral forehead spike, spike slow waves or θactivity were distributed;⑤ release of spike slow waves in Rolandic region on one or both sides;⑥ Hypsarrhythmia. The ictal EEG is shown as follows: ① rhythm release of spike waves at the beginning of focal foci;② generalized spike wave rhythm distribution;③ extensively array with a combination of upper and lower amplitude spike-wave rhythms;④ generalized 4-6 Hz slow wave release-generalized rhythm burst;⑤ wide-spread low amplitude spike wave rhythm distribution--extensively high amplitude spike slow wave array. These episodes are sometimes combined in a single patient.Relationship between duration of onset and background activity: 39 cases(39/67,58.2%) with seizures lasting about 1-8 seconds and 4 cases(4/39,10.3%) with moderate background activity;The episode lasted 8-15 seconds, even longer, there were 28 patients(28/67,41.7%), and 11 patients(11/28,39.3%) were moderately active. Sixty-seven patients were followed up for 1 year, The final diagnosis:8 were diagnosed as west syndrome, 7 were diagnosed with LennoxGastaut syndrome(LGS), 3 were diagnosed with frontal lobe epilepsy, and 15 were diagnosed with children with benign childhood epilepsy with centro-temporal spikes(BECT), 34 patients were only stayed at the diagnostic level of the type of seizure. Conclusion Tonic seizures can occur alone or in a variety of epilepsy syndromes;VEEG can monitor the clinical manifestations and abnormal EEG waveforms during the onset of children, providing theoretical basis for clinical diagnosis and differential diagnosis.