Department of Pediatrics, Pediatric Epilepsy Clinics, Severance Children's Hospital, Brain Research Institute, Yonsei University College of Medicine, Seoul, Korea
Pediatrics. 2010 Jan;125(1):e58-66. doi: 10.1542/peds.2009-0566. Epub 2009 Dec 14.
The objective of this study was to evaluate the role of resective pediatric epilepsy surgery for Lennox-Gastaut syndrome (LGS).
We analyzed clinical data of 27 children and adolescents who had LGS and underwent resective epilepsy surgery despite abundant (>30% of preoperative interictal and/or ictal epileptiform discharges) generalized or generalized contralateral maximal and multiregional electroencephalogram abnormalities.
On high-resolution MRI, cerebral lesions were noted in 23 (85.2%) patients but not in 4 (14.8%) patients. The age of patients at the time of surgery was between 1.7 and 17.3 years (mean: 7.8 years). Surgeries were lobar or multilobar resection in 21 (77.8%) patients and hemispherotomy in 6 (22.2%). At a mean of 33.1 months' postoperative follow-up, 16 (59.3%) patients had no seizures and 4 (14.8%) had infrequent seizures. Of 4 patients without brain abnormalities found on MRI, 2 patients became seizure-free after resective surgery was performed on the basis of electrophysiologic studies and concordant results in other multimodal neuroimages. Malformation of cortical development was the most common pathology and was seen in 20 (74.1%) patients, but 2 (7.4% patients) did not show any abnormal pathology. Sixteen (72.7%) patients, including 14 who had no seizures and 2 who had infrequent seizures after surgery, showed an increase in developmental quotient. No clinical profile was significantly associated with postoperative seizure-free rate.
Resective epilepsy surgery should be considered for children with LGS, despite abundant generalized and multiregional electroencephalogram abnormalities.
本研究旨在评估切除性小儿癫痫手术治疗 Lennox-Gastaut 综合征（LGS）的作用。
我们分析了 27 例患有 LGS 且尽管存在广泛（术前 30%以上的间发性和/或发作性癫痫样放电）或广泛对侧最大和多区域脑电图异常，但仍接受切除性癫痫手术的儿童和青少年的临床数据。
在高分辨率 MRI 上，23 例（85.2%）患者存在脑病变，但 4 例（14.8%）患者无病变。手术时患者年龄为 1.7 至 17.3 岁（平均 7.8 岁）。21 例（77.8%）患者行叶切除术或多叶切除术，6 例（22.2%）行半球切开术。在平均 33.1 个月的术后随访中，16 例（59.3%）患者无发作，4 例（14.8%）患者发作频率较低。在 MRI 上未发现脑异常的 4 例患者中，有 2 例根据电生理研究和其他多模态神经影像的一致结果，在进行切除性手术后成为无发作患者。皮质发育畸形是最常见的病理类型，见于 20 例（74.1%）患者，但 2 例（7.4%）患者无任何异常病理。16 例（72.7%）患者，包括 14 例手术后无发作和 2 例发作频率较低的患者，发育商均有所提高。没有任何临床特征与术后无发作率显著相关。
尽管存在广泛的多区域脑电图异常，切除性癫痫手术仍应考虑用于 LGS 患儿。
