Seery Nabil, Wesselingh Robb, Beech Paul, McLaughlin Laurie M, Rushen Tiffany, Halliday Amy J, Horst Liora Ter, Griffith Sarah P, Forcadela Mirasol, Tan Tracie H, Kazzi Christina, Nesbitt Cassie, Broadley James, Buzzard Katherine, Duncan Andrew J, D'Souza Wendyl Jude, Tran Yang David, Van Der Walt Anneke, Skinner Genevieve, Taylor Bruce V, Swayne Andrew, Brodtmann Amy, Gillis David, Butler Ernest Gerard, Kalincik Tomas, Seneviratne Udaya K, Macdonell Richard A, Blum Stefan, Ramanathan Sudarshini, Malpas Charles B, Reddel Stephen William, Hardy Todd A, O'Brien Terence J, Sanfilippo Paul G, Butzkueven Helmut, Monif Mastura
Department of Neuroscience, Monash University, Melbourne, Australia.
Department of Neurology, Alfred Health, Melbourne, Australia.
Neurol Neuroimmunol Neuroinflamm. 2025 Jul;12(4):e200412. doi: 10.1212/NXI.0000000000200412. Epub 2025 Jun 19.
Few studies have evaluated acute immunotherapy and relapse prevention strategies in patients with anti-leucine-rich glioma-inactivated 1 (LGI1) antibody (Ab)-mediated encephalitis. The objective of this study was to analyze the outcomes of acute and long-term immunotherapy strategies in this population.
We undertook a multicenter cohort study of 55 patients with anti-LGI1 Ab-mediated encephalitis, either recruited prospectively or identified retrospectively from 10 Australian hospitals as part of the Australian Autoimmune Encephalitis Consortium. Clinical data were collected, including treatment durations of all relevant immunotherapies. Clinical outcomes that we examined included (1) time to first clinical relapse, (2) improvement on modified Rankin Scale (mRS), and (3) favorable binary composite clinical-functional outcome at 12 months. A favorable outcome was defined as fulfilling all three of mRS less than 3, a score of 1 or less in the memory dysfunction component of the Clinical Assessment Scale in Autoimmune Encephalitis, and absence of drug-resistant epilepsy.
Rituximab, adjusted for concomitant use of other immunotherapies, was associated with increased time to first relapse (hazard ratio 0.10; 95% CI 0.001-0.85; = 0.03). Intravenous pulsed methylprednisolone was associated with an improvement in mRS (OR 4.48; 95% CI 1.03-21.3; = 0.048) and a favorable composite clinical-functional outcome (OR 4.96; 95% CI 1.07-27.2; = 0.049) at 12 months.
Rituximab may be effective at preventing relapses in patients with anti-LGI1 Ab-mediated encephalitis. Acute methylprednisolone treatment may be associated with favorable outcomes at 12 months.
This study provides Class IV evidence that for patients with anti-LGI1 Ab-mediated encephalitis, rituximab prevents relapses and acute methylprednisolone is associated with favorable outcomes at 12 months.
很少有研究评估抗富含亮氨酸胶质瘤失活1（LGI1）抗体（Ab）介导的脑炎患者的急性免疫治疗及复发预防策略。本研究的目的是分析该人群急性和长期免疫治疗策略的结果。
我们开展了一项多中心队列研究，纳入55例抗LGI1 Ab介导的脑炎患者，这些患者要么是前瞻性招募的，要么是作为澳大利亚自身免疫性脑炎联盟的一部分，从澳大利亚10家医院回顾性识别出来的。收集临床数据，包括所有相关免疫治疗的疗程。我们检查的临床结局包括：（1）首次临床复发时间；（2）改良Rankin量表（mRS）评分改善情况；（3）12个月时良好的二元复合临床功能结局。良好结局定义为满足以下所有三项：mRS小于3、自身免疫性脑炎临床评估量表记忆功能障碍部分评分小于或等于1、无耐药性癫痫。
在调整其他免疫治疗的联合使用情况后，利妥昔单抗与首次复发时间延长相关（风险比0.10；95%置信区间0.001 - 0.85；P = 0.03）。静脉注射脉冲式甲泼尼龙与12个月时mRS评分改善（比值比4.48；95%置信区间1.03 - 21.3；P = 0.048）以及良好的复合临床功能结局（比值比4.96；95%置信区间1.07 - 27.2；P = 0.049）相关。
利妥昔单抗可能对预防抗LGI1 Ab介导的脑炎患者复发有效。急性甲泼尼龙治疗可能与12个月时的良好结局相关。
本研究提供了IV级证据，即对于抗LGI1 Ab介导的脑炎患者，利妥昔单抗可预防复发，急性甲泼尼龙与12个月时的良好结局相关。
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