Warren Aaron E L, Xu Audrey, Barros Guinle Maria Isabel, Johnstone Thomas, Teeyagura Prathyusha, Solidum Rayann, Hyslop Ann, Kim Hyunmi, Grant Gerald, Parker Jonathon J, Buch Vivek, Porter Brenda E, Knowles Juliet K
Department of Neurosurgery, Mass General Brigham, Harvard Medical School, Boston, Massachusetts.
Department of Biology, Stanford University, Stanford, California.
J Neurosurg Case Lessons. 2025 Sep 22;10(12). doi: 10.3171/CASE25478.
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.
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.
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.
Lennox-Gastaut综合征（LGS）是一种严重的发育性和癫痫性脑病，其特征为多种发作类型、智力残疾和独特的脑电图表现。丘脑中央中核深部脑刺激（CM-DBS）是一种新兴的LGS治疗方法，但儿科经验仍然有限。
作者报告了在6名患有LGS的儿童和青少年（年龄12 - 18岁）中进行CM-DBS的单中心经验。1名患者在2个月后因感染相关原因移除了设备。其余5名患者持续接受刺激2.5至5年。所有5名患者的照料者均报告癫痫发作负担和警觉性有所改善，临床总体印象改善评分也证实了这一点。3名患者在将刺激靶点重新编程为先前确定的丘脑中央前外侧最佳刺激靶点后，癫痫发作明显减少。1名患者有术前和术后头皮脑电图记录，显示发作间期放电负担减轻。
当切除性治疗方法不适用时，CM-DBS是治疗儿科难治性LGS的一种有前景的治疗方法。益处持续了数年。电极定位和刺激方向对于优化临床益处似乎很重要。这些发现支持了儿科CM-DBS的可行性和安全性，并强调了开展纳入基于脑电图的结果以及以患者为中心的指标（包括合并症和生活质量）的前瞻性试验的必要性。早期、针对网络的神经调节可能会改善长期结果。https://thejns.org/doi/10.3171/CASE25478
