University of Missouri Kansas City, United States.
Division of Neurology, Department of Pediatrics, Children's Mercy Kansas City, United States.
Seizure. 2021 Mar;86:168-174. doi: 10.1016/j.seizure.2021.02.015. Epub 2021 Feb 17.
Lennox-Gastaut syndrome (LGS) is a severe drug-resistant epilepsy (DRE) of childhood. The Vagus Nerve Stimulator (VNS) is established as a safe and effective treatment for DRE. This study assesses efficacy and tolerability of the auto-stimulation VNS models in pediatric patients with LGS.
This is a retrospective chart review of a cohort of pediatric patients (Age 1-18 years old) with LGS implanted with an auto-stimulation VNS model at a single level four pediatric epilepsy center. Patient responder's rate was measured as seizure reduction over baseline and improvements in five quality-of-life measures as reported by the patients and families. Efficacy and tolerability were assessed at 1, 3, 6, 12, 18 and 24 months compared to baseline.
This cohort includes 71 consecutive children with Lennox-Gastaut syndrome who underwent implantation with one of two models of the auto-stimulation VNS. The average age of the children at implantation was 20.82 months. Of those patients, 55 % of patients achieved greater than 50 % seizure reduction at six months, 67.7 % at 12 months, and 65 % at 24 months. At 12 months 11 % of the patients were completely seizure free and at 24 months 17 % were seizure free. By 24 months post implantation most of the patient families reported at least a 50 % improvement rate in one or more of the quality-of-life measures. The most commonly reported adverse events were dysphonia, paresthesia, and shortness of breath, all of which were tolerated and subsided by 24 months.
This study provides evidence that VNS models with the auto-stimulation paradigm based on detection of tachycardia are well tolerated and effective in a pediatric population with LGS. Furthermore, this study shows that for this population, the auto-stimulation models of the VNS may provide additional benefits over the earlier VNS versions.
Lennox-Gastaut 综合征（LGS）是一种严重的儿童耐药性癫痫（DRE）。迷走神经刺激器（VNS）已被确立为治疗 DRE 的安全有效方法。本研究评估了自主刺激 VNS 模型在 LGS 儿科患者中的疗效和耐受性。
这是一项回顾性图表研究，纳入了在一家四级儿科癫痫中心植入自主刺激 VNS 模型的儿科患者（年龄 1-18 岁）队列。通过比较基线，评估患者的反应率（根据基线减少的癫痫发作次数）和患者及其家属报告的五项生活质量指标的改善情况。在 1、3、6、12、18 和 24 个月时评估疗效和耐受性。
本队列包括 71 例连续接受 Lennox-Gastaut 综合征植入自主刺激 VNS 模型的儿童。患儿植入时的平均年龄为 20.82 个月。其中 55%的患者在 6 个月时癫痫发作减少 50%以上，67.7%在 12 个月时，65%在 24 个月时。在 12 个月时，11%的患者完全无癫痫发作，在 24 个月时，17%的患者无癫痫发作。植入后 24 个月，大多数患者家属报告至少有一种生活质量指标改善了 50%以上。最常见的不良事件是发音困难、感觉异常和呼吸急促，所有这些不良事件在 24 个月时均耐受并缓解。
本研究表明，基于心动过速检测的自主刺激模式的 VNS 模型在 LGS 儿科患者中具有良好的耐受性和有效性。此外，本研究表明，对于这一人群，VNS 的自主刺激模型可能比早期版本的 VNS 提供额外的益处。
