Cottier Rachel, Niazi Farbod, Goël Keshav, Korman Catherine, Porte Tiphaine, Ducruet Thierry, Cossu Giulia, Briscoe Christina, Singh Avantika, Harini Chellamani, Ibrahim George M, Fallah Aria, Weil Alexander G, Hadjinicolaou Aristides
Brain and Development Research Axis, Azrieli CHU Ste-Justine Research Center, Montreal, QC, Canada.
Division of Neurosurgery, Department of Surgery, Sainte-Justine University Hospital Centre, Montreal, QC, Canada.
Front Neurol. 2024 Dec 30;15:1518554. doi: 10.3389/fneur.2024.1518554. eCollection 2024.
Epileptic spasms (ES) are a unique seizure type typically presenting in the form of infantile epileptic spasms syndrome (IESS) with characteristic hypsarrhythmia on scalp EEG and a preponderance with developmental delay or regression. While pharmacotherapy is the mainstay of treatment, surgical options, including disconnective or resective procedures, are increasingly recognized as viable therapeutic options for recurrent or persistent ES. However, limited data on safety, effectiveness, and prognostic factors hinder informed decision-making regarding surgery indications, timing, and intervention type. We performed a systematic review and an individual patient data meta-analysis (IPDMA) in accordance with PRISMA guidelines, focusing on surgical interventions for ES and reporting seizure outcomes using the Engel or ILAE scales. Twenty-six studies encompassing 358 ES patients undergoing resection/callosotomy were included. Participants undergoing other approaches (e.g., multiple subpial transections) or multimodality approaches were excluded from analysis. The median age at spasm onset was 6 months (IQR = 3.0-15.6), with a median age at surgery of 37 months (IQR = 17.2-76.8). Most patients (74.1%) exhibited additional seizure types. A total of 136 patients (35.8%) underwent corpus callosotomy (CC), of whom 125 (91.9%) had a complete callosotomy, while 11 (8.1%) had a partial callosotomy. Resective surgery was performed on 222 patients (58.4%). Among those who underwent resection, 109 (49.1%) had both lesional MRI findings and lateralized EEG abnormalities. Overall, 201 patients (56.1%) remained spasm-free at a median postoperative follow-up of 36 months (interquartile range, IQR = 21-60), including 52 (38.2%) from the callosotomy group and 149 (67.1%) from the resective surgery group. In the resective surgery cohort, patients with MRI-confirmed lesions ( = 0.026; HR = 0.53, 95% CI = 0.31-0.93) and those who underwent hemispherectomy ( = 0.026, HR = 0.46, 95% CI = 0.23-0.91) had better seizure outcomes. Only a minority (24.4%) underwent invasive EEG monitoring prior to ES surgery. Surgical treatment of ES proves effective, with two thirds of patients undergoing resective surgery and a third undergoing CC becoming spasm free. Post-operative developmental improvement was observed in 44 participants (65.7% of those with available data). The presence of lesional MRI and more extensive resection/disconnection (e.g., hemispherectomy) emerged as significant prognostic factors for spasm freedom and can inform clinical decision-making.
癫痫性痉挛(ES)是一种独特的发作类型,通常以婴儿癫痫性痉挛综合征(IESS)的形式出现,头皮脑电图具有特征性的高峰节律紊乱,且多伴有发育迟缓或倒退。虽然药物治疗是主要的治疗方法,但手术选择,包括切断性或切除性手术,越来越被认为是复发性或持续性ES的可行治疗选择。然而,关于安全性、有效性和预后因素的数据有限,这阻碍了在手术适应症、时机和干预类型方面做出明智的决策。我们按照PRISMA指南进行了一项系统评价和个体患者数据荟萃分析(IPDMA),重点关注ES的手术干预,并使用恩格尔或国际抗癫痫联盟(ILAE)量表报告发作结果。纳入了26项研究,共358例接受切除/胼胝体切开术的ES患者。接受其他方法(如多处软膜下横切术)或多模态方法的参与者被排除在分析之外。痉挛发作的中位年龄为6个月(四分位间距IQR = 3.0 - 15.6),手术的中位年龄为37个月(IQR = 17.2 - 76.8)。大多数患者(74.1%)还表现出其他发作类型。共有136例患者(35.8%)接受了胼胝体切开术(CC),其中125例(91.9%)进行了完全胼胝体切开术,11例(8.1%)进行了部分胼胝体切开术。222例患者(58.4%)接受了切除性手术。在接受切除术的患者中,109例(49.1%)既有病变性磁共振成像(MRI)表现,又有脑电图侧化异常。总体而言,201例患者(56.1%)在术后中位随访36个月(四分位间距,IQR = 21 - 60)时无痉挛发作,其中胼胝体切开术组52例(38.2%),切除性手术组149例(67.1%)。在切除性手术队列中,MRI证实有病变的患者(P = 0.026;风险比HR = 0.53,95%置信区间CI = 0.31 - 0.93)和接受大脑半球切除术的患者(P = 0.026,HR = 0.46,95% CI = 0.23 - 0.91)发作结果更好。只有少数患者(24.4%)在ES手术前接受了侵入性脑电图监测。ES的手术治疗被证明是有效的,三分之二接受切除性手术的患者和三分之一接受CC的患者无痉挛发作。44例参与者(有可用数据者的65.7%)术后出现发育改善。病变性MRI的存在以及更广泛的切除/切断(如大脑半球切除术)是无痉挛发作的重要预后因素,可为临床决策提供参考。