学龄儿童病灶性Lennox-Gastaut综合征的临床特征及脑电图

目的分析学龄儿童病灶性Lennox-Gastaut综合征(LGS)的临床特征及脑电图特征,为LGS进一步的临床研究和诊断提供参考。方法选择2011-08—2015-05在我院诊治的LGS学龄儿童32例为LGS组,选择同期在我院进行检查的32例非癫痫正常儿童32例为对照组,记录与调查所有儿童的临床资料、临床表现特征、脑电图特征、智力发育与随访情况等。结果观察组患儿的起病年龄(5.21±2.91)岁;其中8例出生时有异常,8例有惊厥史,9例有癫痫史,6例有智能发育迟滞家族史;强直发作24例,不典型失神11例,肌阵挛发作10例,痉挛发作7例,全面强直-阵挛性癫痫发作21例,失张力发作9例。对照组脑电图均正常,LGS组脑电图背景节律Ⅰ级12例,Ⅱ级16例,Ⅲ级4例;30例清醒或睡眠期可见到12次/s左右的快活动,20例存在一侧性放电波。LGS组智力评分为(61.14±11.84)分,智力障碍发生率为34.4%;对照组智力评分为(34.22±9.93)分,智力障碍发生率为3.1%,组间对比差异有统计学意义(P0.05)。治疗后3个月LGS组的癫痫无发作率为81.3%,6个月无发作率68.8%,1a无发作率为50.0%。结论学龄儿童病灶性LGS的癫痫发作类型多样,脑电图多显示背景ɑ节律异常和棘慢复合波,智能障碍的发生率较高,药物联合治疗的效果较好。

Clinical features and EEG analysis of focal Lennox-Gastaut syndrome in school-age children

Objective To explore and discuss the clinical characteristics and EEG features of focal Lennox-Gastaut syndrome (LGS) in school-age children in order to provide references for further clinical research and diagnosis of LGS.Methods Thirty-two school-age children with focaed LGS from August 2011 to May 2015 in our hospital were selected as LGS group and 32 non-epileptic children undergoing examinations at the same period were selected as control group.We recorded clinical data,clinical manifestations,EEG characteristics,intelligence development and follow-up status.Results In the LGS group,the onset age were (5.21±2.91) years,and there were 8 patients born with abnormality,8 patients with history of seizures,9 patients with history of epilepsy and 6 patients with family history of the delayed intelligence development.In terms of epileptic types,tonic seizures occurred in 24 patients,atypical absence seizures in 11 patients,clonic seizures in 7 patients,generalized tonic-clonic seizures (GTCS) in 21 patients and atonic seizures in 9 patients.The EEG characteristics in the control group were normal,while in the LGS group 12 patients showed grade Ⅰ of EEG background rhythm,16 patients showed Ⅱ grade and 4 patients showed grade Ⅲ,in addition,30 patients presented rapid activity of near 12 times/s when they were awake or at REM state and 20 patients had unilateral discharge waves.The intelligence scores in the LGS group were (61.14±11.84) points and the incidence rate of intellectual disability was 34.4%,while the intelligence scores in the control group were (34.22±9.93) points and incidence rate was 3.1%(P<0.05).The rate of free seizures in the LGS group was 81.3% in three months after treatment,68.8% in six months and 50.0% in one year.Conclusion There are variable epileptic types in school-age children with focal LGS,and EEG displays abnormal αbackground rhythm and spike-slow complex waves.In addition,the incidence rate of intellectual disability is high and the combined therapy of medications may be favorable.