摘要
目的 探讨结节性硬化症（tuberous sclerosis complex,TSC）癫痫发作起始部位与发作间期痫样放电显著部位的关系.方法 对20例经视频脑电图监测到癫痫发作的TSC患儿进行临床资料总结,分析脑电图发作间期显著放电部位与发作期起始部位的一致性.结果 本组女7例,男13例.癫痫起病年龄为生后15 d～6岁,脑电图监测年龄为6个月～11岁.发作间期脑电图表现为高度失律3例,多灶性放电且可区分出显著放电灶12例,广泛性及限局性放电共存2例,限局性放电3例.监测到的发作类型有：癫痫性痉挛、部分性发作、不典型失神、全面及局部肌阵挛,其中部分性发作最多见,其次为癫痫性痉挛.4例癫痫性痉挛发作期为广泛性放电,对应的发作间期脑电图为高度失律或广泛性放电,3例癫痫性痉挛的发作表现及发作期放电均不对称,发作间期显著放电灶与发作期放电部位和侧别2例一致,1例侧别一致但部位不一致.13例仅监测到部分性发作,发作间期显著放电灶与发作期放电侧别和部位均不一致者占15％ （2/13）,均一致性者达62％ （8/13）,部位或侧别之一一致者占15％（2/13）,1例因发作期起始侧别及部位不易确定而无法分析.结论 TSC患儿的发作间期脑电图以多灶性放电为多见,且常有一个显著的放电灶,部分癫痫性痉挛及多数部分性发作时,发作期放电起始部位与发作间期显著放电部位相一致,从而为癫痫外科的术前评估定位提供一定的参考依据.
Objective To analyze the relationship between the ictal onset zone and dominant interictal epileptiform foci in tuberous sclerosis complex （TSC） patients.Method Clinical data of 20 patients with TSC which had epileptic seizures during Video-EEG monitoring was assessed.Consistency and dominance of focal interictal epileptiform activity and the ictal onset zone were identified.Concordance between interictal and ictal findings was analyzed.Result Of the 20 patients,7 were female,and 13 were male.The age of epilepsy onset was from 15 d to 6 years.The Video-EEG monitoring age was from 6 months to 11 years.Family history was found in three cases.Abnormality in neuroimaging existed in 17 of 18 patients who were examined.Interictal EEG showed hypsarrhythmia in 3 patients,multifocal epileptiform activity with a dominant focus in 12 patients,both focal and generalized discharges in 2 patients,and only focal discharges in 3 patients.The seizures types during EEG monitoring included epileptic spasms,partial seizure,atypical absence,and generalized or focal myoclonic seizure.The most common seizure type was partial seizure and then epileptic spasms.EEG in 4 patients with epileptic spasms showed ictal generalized discharges and interictal hypsarrhythmia or generalized discharges.Clinical manifestation of epileptic spasms was asymmetric in 3 patients.Lateralization and location of interictal and ictal discharges were consistent in 2 of the 3 patients,while only lateralization consistency in 1 of the 3 patients.Partial seizures as the only seizure type were monitored in 13 patients.Of the 13 patients,lateralization and location of interictal and ictal discharges were inconsistent in 2 patients （15％）,consistent in 8 patients （62％）,lateralization or location consistent in 2 patients （15％）.One case could not be analyzed because of uncertainty of lateralization and location of seizure onset.Conclusion In the majority of patients with TSC,multifocal interictal epileptiform activity is present,in which a most dominance of focal epileptiform activity could be found.For some epileptic seizures or the majority of partial seizures,the ictal onset zone is concordant with the dominance of focal interictal epileptiform foci.The concordance might have positioning reference significance for preoperative evaluation of epilepsy surgery.
出处
《中华儿科杂志》
CAS
CSCD
北大核心
2014年第4期292-297,共6页
Chinese Journal of Pediatrics
基金
国家科技支撑计划项目（2012BAI03B02）
