Park Jun T, Fernandez-Baca Vaca Guadalupe, Tangen Rachel B, Cohen Mark L, Miller Jonathan P
Section of Pediatric Epilepsy, Department of Pediatrics, Rainbow Babies & Children's Hospital, Cleveland, Ohio, USA; Epilepsy Center, Neurological Institute, Department of Neurology, University Hospitals of Cleveland Medical Center, Cleveland, Ohio, USA; Case Western Reserve University School of Medicine, Cleveland, Ohio, USA.
Epilepsy Center, Neurological Institute, Department of Neurology, University Hospitals of Cleveland Medical Center, Cleveland, Ohio, USA; Case Western Reserve University School of Medicine, Cleveland, Ohio, USA.
World Neurosurg. 2018 Aug;116:268-273. doi: 10.1016/j.wneu.2018.05.182. Epub 2018 Jun 1.
Catastrophic epilepsy results in severe neurodevelopmental delay in infants because of frequent and/or long seizures. Therefore, consideration of early epilepsy surgery is essential for neurodevelopmental outcome. Once an infant with catastrophic seizures is identified as a surgical candidate, it is important that the surgical plan be carefully defined based on detailed presurgical evidence to minimize surgical complications in this age group.
We present 2 infants with catastrophic epilepsy, epileptic spasms, and bihemispheric electroencephalographic abnormalities who underwent one-stage disconnection surgery based on a sound hypothesis of the epileptogenic zone. Each patient underwent an extensive noninvasive presurgical investigation followed by stereotactic disconnection leucotomy in a single stage. After the 2 children were followed for 24-36 months. A seizure reduction by at least 90% (Engel class I) was achieved in both cases with subsequent improvement in neurodevelopmental progress. There were no perioperative complications. Both patients had widespread cortical dysplasia on pathologic evaluation.
Careful consideration of the noninvasive presurgical workup can identify focal onset even in the presence of catastrophic epilepsy with widespread bilateral abnormalities. Single-stage lobar leucotomy for disconnection of the epileptogenic zone can lead to excellent outcome in these patients.
灾难性癫痫因频繁和/或长时间发作导致婴儿严重神经发育迟缓。因此，考虑早期癫痫手术对神经发育结局至关重要。一旦确定患有灾难性癫痫的婴儿为手术候选者，基于详细的术前证据仔细制定手术计划以尽量减少该年龄组的手术并发症就很重要。
我们报告了2例患有灾难性癫痫、癫痫痉挛和双侧半球脑电图异常的婴儿，他们基于癫痫灶的合理假设接受了一期离断手术。每位患者都进行了广泛的非侵入性术前检查，随后在单一阶段进行立体定向离断性白质切除术。对这2名儿童进行了24至36个月的随访。两例患者的癫痫发作均减少了至少90%（Engel I级），随后神经发育进程得到改善。没有围手术期并发症。两名患者在病理评估中均有广泛的皮质发育异常。
即使存在伴有广泛双侧异常的灾难性癫痫，仔细考虑非侵入性术前检查也能识别局灶性发作起始。用于离断癫痫灶的一期脑叶白质切除术可使这些患者获得良好结局。
