Inoue Takeshi, Uda Takehiro, Kuki Ichiro, Yamamoto Naohiro, Nagase Shizuka, Nukui Megumi, Okazaki Shin, Kawashima Toshiyuki, Nakanishi Yoko, Kunihiro Noritsugu, Matsuzaka Yasuhiro, Kawawaki Hisashi, Otsubo Hiroshi
Department of Pediatric Neurology, Osaka City General Hospital, 2-13-22 Miyakojimahondori, Miyakojima-ku, Osaka 534-0021, Japan.
Department of Pediatric Neurosurgery, Osaka City General Hospital, 2-13-22 Miyakojimahondori, Miyakojima-ku, Osaka 534-0021, Japan.
Epilepsy Behav Rep. 2021 Jan 19;15:100429. doi: 10.1016/j.ebr.2021.100429. eCollection 2021.
We present a case of drug-resistant focal motor seizures in which separate cortico-cortical epileptic networks within the supplementary motor area (SMA) proper and primary motor area (PMA) were proven by ictal high-frequency oscillation (HFO) and cortico-cortical evoked potential (CCEP). A 12-year-old girl presented with two types seizures: type A, tonic extension and subsequent clonic movements of the right arm; and type B, tonic and clonic movements of the right leg. MRI was normal and karyotype genetic analysis revealed 46,X,t(X;14)(q13;p12). She underwent placement of chronic subdural electrodes over the left hemisphere. We recorded a total of nine seizures during 10 days of epilepsy monitoring. Type A seizures started from the lower part of the left SMA proper and early spread to the hand motor area of the PMA. Type B seizures started from the upper part of the SMA proper and early spread to the leg motor area of the PMA. CCEPs of both SMA proper and PMA activated two identical routes for evoked potentials correlating with separate pathways. Corticectomy of the left SMA proper and PMA achieved seizure-free without hemiparesis. Within a small homunculus of the SMA proper, separate epileptic networks were proven and validated by seizure semiology, ictal HFO, and CCEP.
我们报告一例耐药性局灶性运动性癫痫病例，其中通过发作期高频振荡（HFO）和皮质-皮质诱发电位（CCEP）证实了辅助运动区（SMA）固有区和初级运动区（PMA）内存在独立的皮质-皮质癫痫网络。一名12岁女孩出现两种类型的癫痫发作：A 型，右臂强直性伸展及随后的阵挛性运动；B 型，右腿强直性和阵挛性运动。MRI 正常，核型基因分析显示 46,X,t(X;14)(q13;p12)。她在左半球放置了慢性硬膜下电极。在 10 天的癫痫监测期间，我们共记录到 9 次癫痫发作。A 型发作始于左 SMA 固有区下部，并早期扩散至 PMA 的手部运动区。B 型发作始于 SMA 固有区上部，并早期扩散至 PMA 的腿部运动区。SMA 固有区和 PMA 的 CCEP 激活了两条相同的诱发电位途径，与不同的传导通路相关。切除左 SMA 固有区和 PMA 后癫痫发作停止，且未出现偏瘫。在 SMA 固有区的一个小运动小人像范围内，通过癫痫发作症状学、发作期 HFO 和 CCEP 证实并验证了独立的癫痫网络。
