Yang Zhixian, Guo Qinghui, Zhuang Jiaxin, Liu Xiaoyan, Xiong Hui, Wu Ye, Wang Shuang, Chang Xingzhi, Zhang Yuehua, Bao Xinhua, Jiang Yuwu, Qin Jiong
Department of Pediatrics, Peking University First Hospital, Beijing.
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Zhonghua Er Ke Za Zhi. 2014 Apr;52(4):292-7.
To analyze the relationship between the ictal onset zone and dominant interictal epileptiform foci in tuberous sclerosis complex (TSC) patients.
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.
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.
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.
分析结节性硬化症（TSC）患者发作期起始区与主要发作间期癫痫样放电灶之间的关系。
对20例在视频脑电图监测期间有癫痫发作的TSC患者的临床资料进行评估。确定局灶性发作间期癫痫样活动与发作期起始区的一致性及优势性。分析发作间期与发作期结果之间的一致性。
20例患者中，女性7例，男性13例。癫痫发作年龄为15天至6岁。视频脑电图监测年龄为6个月至11岁。3例有家族史。18例接受检查的患者中17例存在神经影像学异常。发作间期脑电图显示，3例患者有高度失律，12例患者有多灶性癫痫样活动且有一个主要病灶，2例患者有局灶性和全身性放电，3例患者仅有局灶性放电。脑电图监测期间的发作类型包括癫痫痉挛、部分性发作、非典型失神发作以及全身性或局灶性肌阵挛发作。最常见的发作类型是部分性发作，其次是癫痫痉挛。4例癫痫痉挛患者的脑电图显示发作期全身性放电以及发作间期高度失律或全身性放电。3例癫痫痉挛患者的临床表现不对称。3例患者中有2例发作间期和发作期放电的定侧及定位一致，但3例患者中只有1例定侧一致。13例患者仅监测到部分性发作作为唯一发作类型。在这13例患者中，2例（15%）发作间期和发作期放电的定侧及定位不一致，8例（62%）一致，2例（15%）定侧或定位一致。1例因发作起始定侧和定位不确定而无法分析。
在大多数TSC患者中，存在多灶性发作间期癫痫样活动，其中可发现一个最主要的局灶性癫痫样活动。对于某些癫痫发作或大多数部分性发作，发作期起始区与局灶性发作间期癫痫样放电灶的优势部位一致。这种一致性可能对癫痫手术的术前评估具有定位参考意义。
