Department of Neurology, Comprehensive Epilepsy Center, Yale University School of Medicine, New Haven, Connecticut, USA.
Sorbonne Université, Institut du Cerveau, Paris Brain Institute, ICM, INSERM, CNRS, AP-HP, Hôpital de la Pitié-Salpêtrière, Paris, France.
Epilepsia. 2024 Aug;65(8):e148-e155. doi: 10.1111/epi.18014. Epub 2024 Jun 5.
In response to the evolving treatment landscape for new-onset refractory status epilepticus (NORSE) and the publication of consensus recommendations in 2022, we conducted a comparative analysis of NORSE management over time. Seventy-seven patients were enrolled by 32 centers, from July 2016 to August 2023, in the NORSE/FIRES biorepository at Yale. Immunotherapy was administered to 88% of patients after a median of 3 days, with 52% receiving second-line immunotherapy after a median of 12 days (anakinra 29%, rituximab 25%, and tocilizumab 19%). There was an increase in the use of second-line immunotherapies (odds ratio [OR] = 1.4, 95% CI = 1.1-1.8) and ketogenic diet (OR = 1.8, 95% CI = 1.3-2.6) over time. Specifically, patients from 2022 to 2023 more frequently received second-line immunotherapy (69% vs 40%; OR = 3.3; 95% CI = 1.3-8.9)-particularly anakinra (50% vs 13%; OR = 6.5; 95% CI = 2.3-21.0), and the ketogenic diet (OR = 6.8; 95% CI = 2.5-20.1)-than those before 2022. Among the 27 patients who received anakinra and/or tocilizumab, earlier administration after status epilepticus onset correlated with a shorter duration of status epilepticus (ρ = .519, p = .005). Our findings indicate an evolution in NORSE management, emphasizing the increasing use of second-line immunotherapies and the ketogenic diet. Future research will clarify the impact of these treatments and their timing on patient outcomes.
针对新发性难治性癫痫持续状态 (NORSE) 的治疗进展以及 2022 年发布的共识建议，我们对 NORSE 的治疗进行了时间上的比较分析。该研究在耶鲁大学 NORSE/FIRES 生物标本库纳入了 32 个中心于 2016 年 7 月至 2023 年 8 月期间收治的 77 名患者。中位时间为 3 天后，88%的患者接受了免疫治疗，52%的患者在中位时间 12 天后接受了二线免疫治疗（阿那白滞素 29%、利妥昔单抗 25%和托珠单抗 19%）。二线免疫治疗（比值比 [OR] = 1.4，95%置信区间 [CI] = 1.1-1.8）和生酮饮食（OR = 1.8，95%CI = 1.3-2.6）的使用率随时间推移而增加。具体来说，2022 年至 2023 年的患者更频繁地接受二线免疫治疗（69% vs 40%；OR = 3.3；95%CI = 1.3-8.9），尤其是阿那白滞素（50% vs 13%；OR = 6.5；95%CI = 2.3-21.0）和生酮饮食（OR = 6.8；95%CI = 2.5-20.1），而在 2022 年之前接受二线免疫治疗的患者比例较低。在接受阿那白滞素和/或托珠单抗治疗的 27 名患者中，癫痫持续状态发作后更早的给药与癫痫持续状态持续时间更短相关（ρ =.519，p =.005）。我们的研究结果表明，NORSE 的治疗方法在不断演变，强调了二线免疫治疗和生酮饮食的应用越来越多。未来的研究将阐明这些治疗方法及其时机对患者结局的影响。
