Speakes Mikaela E, Reznik-Schaefer Kiersten, Al-Ramadhani Ruba, Fernandez Luis D, Hect Jasmine L, Abel Taylor J, Welch William P
Division of Pediatric Neurology, Department of Pediatrics, University of Pittsburgh School of Medicine, Children's Hospital of Pittsburgh, 8th Floor Faculty Pavilion, 4401 Penn Ave., Pittsburgh, PA 15224, United States.
Department of Neurological Surgery, University of Pittsburgh School of Medicine, UPMC Presbyterian Hospital, Suite B-400, 200 Lothrop Street, Pittsburgh, PA 15213, United States.
Epilepsy Res. 2025 Feb;210:107516. doi: 10.1016/j.eplepsyres.2025.107516. Epub 2025 Jan 22.
Responsive neurostimulation of the centromedian nucleus of the thalamus (CM RNS) is being investigated for treatment of drug-resistant generalized epilepsy with promising results. The aim of this study is to report outcomes of seven patients with pediatric-onset drug-resistant generalized epilepsy, including both genetic generalized epilepsy (GGE) and Lennox-Gastaut syndrome (LGS), who underwent treatment with bilateral CM RNS.
A retrospective chart review was performed for patients with drug-resistant generalized epilepsy who underwent treatment with bilateral CM RNS at Children's Hospital of Pittsburgh from 2020 to 2022. Improvement in seizure frequency was obtained through patient and/or caregiver reports on standardized patient questionnaires. The primary outcome measure was percent improvement in seizure frequency at time of last follow-up appointment compared to baseline seizure frequency.
Five of the seven patients (71 %) had an average 50 % or greater improvement in seizure frequency among seizure types including four of the five patients (80 %) with GGE and one of the two patients (50 %) with LGS. There were no serious adverse events including post-operative infection, stroke, or device malfunction/migration.
This data, along with other recent studies, suggests that CM RNS can improve seizure frequency in pediatric-onset drug-resistant generalized epilepsy, but larger systematic studies with longer follow-up times and standardized outcome measures are needed to determine long-term effectiveness and optimal patient selection for thalamic RNS.
丘脑中央中核响应性神经刺激(CM RNS)正在被研究用于治疗耐药性全身性癫痫,且已取得了有前景的结果。本研究的目的是报告7例小儿起病的耐药性全身性癫痫患者的治疗结果,这些患者包括遗传性全身性癫痫(GGE)和Lennox-Gastaut综合征(LGS),均接受了双侧CM RNS治疗。
对2020年至2022年在匹兹堡儿童医院接受双侧CM RNS治疗的耐药性全身性癫痫患者进行回顾性病历审查。通过患者和/或护理人员在标准化患者问卷上的报告来获取癫痫发作频率的改善情况。主要结局指标是与基线癫痫发作频率相比,最后一次随访预约时癫痫发作频率的改善百分比。
7例患者中有5例(71%)在包括5例GGE患者中的4例(80%)和2例LGS患者中的1例(50%)在内的癫痫发作类型中,癫痫发作频率平均改善了50%或更多。没有严重不良事件,包括术后感染、中风或设备故障/移位。
这些数据以及其他近期研究表明,CM RNS可以改善小儿起病的耐药性全身性癫痫的癫痫发作频率,但需要更大规模的系统研究,采用更长的随访时间和标准化的结局指标,以确定丘脑RNS的长期有效性和最佳患者选择。