Neuromodulation Strategies in Lennox‐Gastaut Syndrome: Practical Clinical Guidance from the Pediatric Epilepsy Research Consortium

IF 2 4区医学 Q3 CLINICAL NEUROLOGY

Epilepsy Research Pub Date : 2025-02-01 DOI:10.1016/j.eplepsyres.2024.107499

Debopam Samanta , Gewalin Aungaroon , Anthony L. Fine , Cemal Karakas , Michelle Y. Chiu , Puneet Jain , Syndi Seinfeld , Juliet K. Knowles , Ismail S. Mohamed , Carl E. Stafstrom , Tracy Dixon-Salazar , Anup D. Patel , Sonam Bhalla , Cynthia Guadalupe Keator , Jorge Vidaurre , Aaron E.L. Warren , Renée A. Shellhaas , M. Scott Perry

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引用次数: 0

Abstract

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.

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lenox - gastaut综合征的神经调节策略：儿科癫痫研究联盟的实用临床指导。

lenox - gastaut综合征（LGS）是一种严重的发育性和癫痫性脑病，其特征是多种耐药发作类型、认知障碍和独特的脑电图模式。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），已经成为对抗癫痫药物反应不充分的LGS患者的重要治疗选择。这篇综述是根据儿童癫痫研究联盟（PERC） LGS特别兴趣小组的意见编写的，为临床医生在LGS患者中使用这些神经调节方法提供了实用指导。我们讨论患者的选择标准，预期的癫痫发作和非癫痫发作的结果，潜在的并发症，和设备管理的注意事项，每个技术。综述还包括起始和滴定策略，持续护理要求，以及结合多种神经调节方式的新数据。虽然这三种方法都可以减少LGS患者的癫痫发作频率，通常报道的应答率从50% %到60% %不等，但它们对认知、行为和生活质量的影响却变化较大。在这一具有挑战性的患者群体中，谨慎的患者选择，个性化的规划和长期随访对于优化神经调节的结果至关重要。需要进一步的研究来确定最佳候选者，确定患者临床过程中考虑神经调节的理想时机，制定标准化的结果测量，并评估不同神经调节技术对LGS的比较有效性和成本效益。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Epilepsy Research 医学-临床神经学

CiteScore

0.10

自引率

4.50%

发文量

143

审稿时长

62 days

期刊介绍： Epilepsy Research provides for publication of high quality articles in both basic and clinical epilepsy research, with a special emphasis on translational research that ultimately relates to epilepsy as a human condition. The journal is intended to provide a forum for reporting the best and most rigorous epilepsy research from all disciplines ranging from biophysics and molecular biology to epidemiological and psychosocial research. As such the journal will publish original papers relevant to epilepsy from any scientific discipline and also studies of a multidisciplinary nature. Clinical and experimental research papers adopting fresh conceptual approaches to the study of epilepsy and its treatment are encouraged. The overriding criteria for publication are novelty, significant clinical or experimental relevance, and interest to a multidisciplinary audience in the broad arena of epilepsy. Review articles focused on any topic of epilepsy research will also be considered, but only if they present an exceptionally clear synthesis of current knowledge and future directions of a research area, based on a critical assessment of the available data or on hypotheses that are likely to stimulate more critical thinking and further advances in an area of epilepsy research.