Optimal stimulation of the thalamic centromedian nucleus in children with Lennox-Gastaut syndrome: patient series.

Aaron E L Warren, Audrey Xu, Maria Isabel Barros Guinle, Thomas Johnstone, Prathyusha Teeyagura, Rayann Solidum, Ann Hyslop, Hyunmi Kim, Gerald Grant, Jonathon J Parker, Vivek Buch, Brenda E Porter, Juliet K Knowles
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Abstract

Background: Lennox-Gastaut syndrome (LGS) is a severe developmental and epileptic encephalopathy characterized by multiple seizure types, intellectual disability, and distinctive EEG findings. Deep brain stimulation of the centromedian nucleus (CM-DBS) is an emerging therapy for LGS, but pediatric experience remains limited.

Observations: The authors report a single-center experience with CM-DBS in 6 children and adolescents (ages 12-18 years) with LGS. One patient experienced infection-related device removal after 2 months. The remaining 5 received sustained stimulation for 2.5-5 years. All 5 showed caregiver-reported improvements in seizure burden and alertness, with corroborating Clinical Global Impression-Improvement scores. Three patients exhibited marked seizure reduction following targeted reprogramming toward a previously identified optimal target of stimulation in the anterolateral CM. Pre- and post-DBS scalp EEG recordings were available in 1 patient and showed a reduced burden of interictal discharges.

Lessons: CM-DBS is a promising treatment for pediatric, medically refractory LGS when resective approaches are unsuitable. Benefits were sustained over years. Lead localization and direction of stimulation appear important to optimize clinical benefit. These findings support the feasibility and safety of pediatric CM-DBS and highlight the need for prospective trials incorporating EEG-based outcomes and patient-centered measures including comorbidities and quality of life. Early, network-targeted neuromodulation may improve long-term outcomes. https://thejns.org/doi/10.3171/CASE25478.

lenox - gastaut综合征患儿丘脑中央核的最佳刺激:患者系列。
背景:lenox - gastaut综合征(LGS)是一种严重的发育性和癫痫性脑病,以多种发作类型、智力残疾和独特的脑电图表现为特征。脑深部正中核刺激(CM-DBS)是一种新兴的LGS治疗方法,但儿科经验仍然有限。观察:作者报告了6例LGS儿童和青少年(12-18岁)CM-DBS的单中心经验。一名患者在2个月后经历了感染相关的器械移除。其余5只接受2.5-5年的持续刺激。所有5例患者均显示护理人员报告的癫痫发作负担和警觉性的改善,并证实了临床总体印象改善评分。三名患者表现出明显的癫痫发作减少后,有针对性的重编程,以先前确定的最佳目标刺激前外侧CM。1例患者dbs前后的头皮脑电图记录显示间歇放电负担减轻。经验教训:CM-DBS是一种很有希望的治疗小儿难治性LGS的方法,当各自的方法不合适时。福利持续多年。导联的定位和刺激方向对优化临床疗效至关重要。这些发现支持了小儿CM-DBS的可行性和安全性,并强调了前瞻性试验的必要性,该试验结合了基于脑电图的结果和以患者为中心的措施,包括合并症和生活质量。早期,以网络为目标的神经调节可能改善长期结果。https://thejns.org/doi/10.3171/CASE25478。
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