Department of Neurology, Mayo Clinic Florida, FL, USA.
Department of Neurology, Mayo Clinic Rochester, MN, USA.
Epilepsy Res. 2022 Aug;184:106974. doi: 10.1016/j.eplepsyres.2022.106974. Epub 2022 Jul 1.
This study examines the safety and effectiveness of brain-responsive neurostimulation (RNS System) therapy in patients with refractory autoimmune-associated epilepsy (AAE).
We retrospectively reviewed 85 medical records of patients who were treated with the RNS System at the three Mayo Clinic sites to identify patients with AAE. We collected clinical data including demographics, epilepsy history, prior evaluations and treatment, RNS implantation and lead information, long term ambulatory electrocorticography (ECoG) data, and patient-reported seizure details. Inclusion criteria included: (1) confirmed neural antibodies, or absent/negative autoimmune panel with Antibody Prevalence in Epilepsy (APE2) score ≥ 4 and (2) at least 6 months of follow up after RNS implantation. The primary outcomes measured were patient-reported seizure frequencies at last follow-up as compared to baseline, ECoG long-episode frequencies, and adverse events following RNS implantation.
Of the 85 patients reviewed, nine (11 %) met the inclusion criteria. Three patients had GAD65 antibodies (mean serum titer = 816 ug/dl), one had Rasmussen's encephalitis (biopsy proven), and five had absent/negative neuronal antibody panel but APE scores were ≥ 4. Six out of nine patients (67 %) reported improvement in clinical seizure frequency, all reported improvements in seizure duration and intensity. Four of 9 patients (44 %) showed trends of decreasing frequency of prolonged periods of epileptiform activity over time. One patient (11 %) developed a superficial wound infection at the implant site.
The findings from our study suggest that adjunctive treatment with the RNS System may be a safe option for patients with refractory AAE.
本研究旨在评估脑反应性神经刺激(RNS 系统)疗法治疗难治性自身免疫相关性癫痫(AAE)患者的安全性和有效性。
我们回顾性分析了在梅奥诊所三个院区接受 RNS 系统治疗的 85 例患者的病历,以确定 AAE 患者。我们收集了临床数据,包括人口统计学资料、癫痫病史、既往评估和治疗、RNS 植入和导联信息、长期动态脑电图(ECoG)数据以及患者报告的发作细节。纳入标准包括:(1)明确的神经抗体,或抗体阳性预测值(APE2)评分≥4 且自身免疫panel 阴性/正常,以及(2)RNS 植入后至少 6 个月的随访。主要结局指标为末次随访与基线相比患者报告的发作频率、ECoG 长发作频率以及 RNS 植入后的不良事件。
在 85 例患者中,有 9 例(11%)符合纳入标准。其中 3 例患者存在 GAD65 抗体(平均血清滴度=816ug/dl),1 例存在 Rasmussen 脑炎(活检证实),5 例存在阴性/正常神经元抗体谱,但 APE 评分≥4。9 例患者中有 6 例(67%)报告临床发作频率改善,所有患者均报告发作持续时间和强度改善。9 例患者中有 4 例(44%)显示随着时间的推移,长时间癫痫样活动频率呈下降趋势。1 例(11%)患者出现植入部位浅表性伤口感染。
本研究结果表明,RNS 系统的辅助治疗可能是治疗难治性 AAE 患者的一种安全选择。