Samanta Debopam, Aungaroon Gewalin, Fine Anthony L, Karakas Cemal, Chiu Michelle Y, Jain Puneet, Seinfeld Syndi, Knowles Juliet K, Mohamed Ismail S, Stafstrom Carl E, Dixon-Salazar Tracy, Patel Anup D, Bhalla Sonam, Keator Cynthia Guadalupe, Vidaurre Jorge, Warren Aaron E L, Shellhaas Renée A, Perry M Scott
Division of Child Neurology, Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, AR, USA.
Comprehensive Epilepsy Center, Division of Neurology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA; Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA.
Epilepsy Res. 2025 Feb;210:107499. doi: 10.1016/j.eplepsyres.2024.107499. Epub 2025 Jan 2.
Lennox-Gastaut syndrome (LGS) is a severe developmental and epileptic encephalopathy characterized by multiple drug-resistant seizure types, cognitive impairment, and distinctive electroencephalographic patterns. Neuromodulation techniques, including vagus nerve stimulation (VNS), deep brain stimulation (DBS), and responsive neurostimulation (RNS), have emerged as important treatment options for patients with LGS who do not respond adequately to antiseizure medications. This review, developed with input from the Pediatric Epilepsy Research Consortium (PERC) LGS Special Interest Group, provides practical guidance for clinicians on the use of these neuromodulation approaches in patients with LGS. We discuss patient selection criteria, expected seizure and non-seizure outcomes, potential complications, and device management considerations for each technique. The review also covers initiation and titration strategies, ongoing care requirements, and emerging data on combining multiple neuromodulation modalities. While all three approaches can reduce seizure frequency in patients with LGS, with commonly reported responder rates ranging from 50 % to 60 %, their impacts on cognition, behavior and quality of life are more variable. Careful patient selection, individualized programming, and long-term follow-up are essential to optimize outcomes with neuromodulation in this challenging patient population. Further research is needed to identify optimal candidates, determine the ideal timing during patients' clinical course to consider neuromodulation, develop standardized outcome measures, and evaluate the comparative effectiveness and cost-effectiveness of different neuromodulation techniques for LGS.
伦诺克斯 - 加斯托综合征（LGS）是一种严重的发育性和癫痫性脑病，其特征为多种耐药性癫痫发作类型、认知障碍以及独特的脑电图模式。神经调节技术，包括迷走神经刺激（VNS）、深部脑刺激（DBS）和反应性神经刺激（RNS），已成为对癫痫发作药物治疗反应不佳的LGS患者的重要治疗选择。本综述由儿科癫痫研究联盟（PERC）LGS特别兴趣小组提供意见编写而成，为临床医生在LGS患者中使用这些神经调节方法提供实用指导。我们讨论了每种技术的患者选择标准、预期的癫痫发作和非癫痫发作结果、潜在并发症以及设备管理注意事项。该综述还涵盖了启动和滴定策略、持续护理要求以及关于联合多种神经调节方式的新数据。虽然所有这三种方法都可以降低LGS患者的癫痫发作频率，通常报告的有效率在50%至60%之间，但它们对认知、行为和生活质量的影响则更具变异性。在这个具有挑战性的患者群体中，仔细的患者选择、个性化编程和长期随访对于通过神经调节优化治疗效果至关重要。需要进一步研究以确定最佳候选者，确定患者临床过程中考虑神经调节的理想时机，制定标准化的结局指标，并评估不同神经调节技术对LGS的比较有效性和成本效益。
