Long-Term Clinical and Biological Prognostic Factors of Anti-NMDA Receptor Encephalitis in Children.

IF 7.8 1区医学 Q1 CLINICAL NEUROLOGY Neurology® Neuroimmunology & Neuroinflammation Pub Date : 2025-03-01 Epub Date: 2024-12-23 DOI:10.1212/NXI.0000000000200346

Maxime Mazowiecki, Lorraine Flet-Berliac, Julia Roux, Anne Lépine, Pascale Chretien, Salima Hacein-Bey-Abina, Laetitia Giorgi, Frederic Villega, Emmanuel Cheuret, Marie Benaiteau, Veronique Rogemond, Geraldine Picard, Sarah Baer, Pierre Cleuziou, Elodie Lametery, Isabelle Desguerre, Mélodie Aubart, Mathilde Chevignard, Roger Le Grand, Philippe Horellou, Carole Leroy, Bastien Joubert, Jerome Honnorat, Kumaran Deiva

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Abstract

Background and objectives: Anti-NMDAR encephalitis (NMDARE) is a severe neurologic condition, and recently, the NMDAR Encephalitis One-Year Functional Status (NEOS) score has emerged as a 1-year prognostic tool. This study aimed to evaluate NEOS score and biomarker (neurofilament light chains [NfL], total-Tau protein, glial fibrillary acidic protein, and serum cytokines) correlation with modified Rankin Scale (mRS), cognitive impairment, and clinical recovery in pediatric NMDARE over 2 years.

Methods: In this French multicenter observational study, 104 pediatric patients with NMDARE were followed for a minimum of 2 years. Clinical data and serum/plasma samples were collected. Biomarker levels, measured using electroluminescence mesoscale discovery (MSD) S-PLEX, were compared between patients and controls and assessed for correlations with disease activity, mRS, cognitive/language impairment, and recovery status at 2 years.

Results: At a median follow-up of 39.5 months, 68 percent of patients had unfavorable recovery and 54% had significant cognitive impairment. Both outcomes were strongly associated with younger age at diagnosis (OR 6.10 [1.91-27.3] p < 0.01 and 5.69 [1.46-27.7] p = 0.02, respectively). A higher NEOS score was significantly correlated with increased cognitive impairment (OR 2.53 [1.52-4.21], p < 0.001), higher mRS scores (OR 2.12 [1.34-3.57], p < 0.01), and unfavorable recovery at 2 years (OR 2.00 [1.30-3.06], p = 0.015). Elevated NfL levels were significantly associated with unfavorable recovery (OR 3.62 [1.29-10.9] p = 0.012) and severe cognitive impairment (OR 3.77 [1.38-10.9] p = 0.012) at 2 years. The combined area under the curve (AUC) for NfL and NEOS was significantly higher than the AUCs of NEOS and NfL alone (p = 0.01).

Discussion: The NEOS score strongly predicts long-term outcomes in NMDARE, with its predictive value extending beyond the first-year mR prediction. NfL levels at disease onset seem to improve accuracy in predicting poor outcomes, providing valuable information for treatment decisions and future clinical trials.

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儿童抗nmda受体脑炎的长期临床和生物学预后因素。

背景和目的：抗NMDAR脑炎（NMDARE）是一种严重的神经系统疾病，最近，NMDAR脑炎1年功能状态（NEOS）评分已成为1年预后工具。本研究旨在评估NEOS评分和生物标志物（神经丝轻链[NfL]、总tau蛋白、胶质纤维酸性蛋白和血清细胞因子）与改良Rankin量表（mRS）、认知障碍和儿童NMDARE 2年临床恢复的相关性。方法：在这项法国多中心观察性研究中，对104例NMDARE患儿进行了至少2年的随访。收集临床资料及血清/血浆样本。使用电致发光中尺度发现（MSD） S-PLEX测量的生物标志物水平在患者和对照组之间进行比较，并评估与疾病活动性、mRS、认知/语言障碍和2年后恢复状态的相关性。结果：在中位39.5个月的随访中，68%的患者恢复不良，54%的患者有明显的认知障碍。两种结果均与诊断年龄较低密切相关（OR分别为6.10 [1.91-27.3]p < 0.01和5.69 [1.46-27.7]p = 0.02）。较高的NEOS评分与认知功能障碍加重（OR 2.53 [1.52-4.21], p < 0.001）、较高的mRS评分（OR 2.12 [1.34-3.57], p < 0.01）、2年后恢复不良（OR 2.00 [1.30-3.06], p = 0.015）显著相关。2年后，NfL水平升高与不良恢复（OR 3.62 [1.29-10.9] p = 0.012）和严重认知障碍（OR 3.77 [1.38-10.9] p = 0.012）显著相关。NfL和NEOS的联合曲线下面积（AUC）显著高于NEOS和NfL单独的AUC （p = 0.01）。讨论：NEOS评分强有力地预测了NMDARE的长期预后，其预测价值超出了第一年mR预测。疾病发病时的NfL水平似乎提高了预测不良结果的准确性，为治疗决策和未来的临床试验提供了有价值的信息。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Neurology® Neuroimmunology & Neuroinflammation Neuroscience-Neurology

CiteScore

15.60

自引率

2.30%

发文量

219

审稿时长

8 weeks

期刊介绍： Neurology Neuroimmunology & Neuroinflammation is an official journal of the American Academy of Neurology. Neurology: Neuroimmunology & Neuroinflammation will be the premier peer-reviewed journal in neuroimmunology and neuroinflammation. This journal publishes rigorously peer-reviewed open-access reports of original research and in-depth reviews of topics in neuroimmunology & neuroinflammation, affecting the full range of neurologic diseases including (but not limited to) Alzheimer's disease, Parkinson's disease, ALS, tauopathy, and stroke; multiple sclerosis and NMO; inflammatory peripheral nerve and muscle disease, Guillain-Barré and myasthenia gravis; nervous system infection; paraneoplastic syndromes, noninfectious encephalitides and other antibody-mediated disorders; and psychiatric and neurodevelopmental disorders. Clinical trials, instructive case reports, and small case series will also be featured.