回顾性儿科队列研究验证了 NEOS 评分，并证明其在抗 NMDAR 脑炎儿童中的适用性。
Retrospective Pediatric Cohort Study Validates NEOS Score and Demonstrates Applicability in Children With Anti-NMDAR Encephalitis.
机构信息
From the Department of Pediatric Neurology (M.N., A.M.K., M.S., E.K.) and Center for Chronically Sick Children, Charité-Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin Institute of Health (BIH); Institute of Clinical Molecular Biology, Christian-Albrechts-University Kiel, University Hospital Schleswig Holstein, Campus Kiel; Department of Genetics and Bioinformatics (P.R.), Kiel; Department of Pediatric Neurology (K.R., A.B.), Children's Hospital Datteln, University Witten/Herdecke, Datteln, Germany; Neuropediatric Unit (R.W.), Karolinska University Hospital, Astrid Lindgren Children's Hospital, Stockholm, Sweden; Department of Pediatrics (J.J., J.D.), University Medical Center Hamburg-Eppendorf, Hamburg, Germany; Division of Pediatric Pulmonology, Allergology and Endocrinology, Department of Pediatrics and Adolescent Medicine (M.B.), Medical University of Vienna, Austria; Department of Pediatric Neurology (M.S.), University Children's Hospital Augsburg; Division of Pediatric Neurology, Department of Pediatrics (K.D.), Hospital Kassel; Department of Pediatrics (M.H., A.Q.), Division of Neuropediatrics and Social Pediatrics, Medical University Rheinisch-Westfälische Technische Hochschule (RWTH) Aachen; Division of Pediatric Neurology, Department of Pediatrics (A.B.), München Klinik Harlaching, Munich; Department of Pediatrics and Pediatric Neurology (H.R.), Georg August University, Göttingen; Department of Paediatric and Adolescent Medicine (C.S.), St Joseph Hospital, Berlin; Department of Pediatrics (K.v.), Vivantes Hospital Friedrichshain, Berlin; Department of Pediatrics (M.D.), Ernst von Bergmann Hospital, Potsdam; Department of Neurology (C.F., F.B.), Charité-Universitätsmedizin Berlin and Berlin School of Mind and Brain, Humboldt-Universität zu Berlin; Charité-Universitätsmedizin Berlin (A.M.K.), Corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health (BIH), Institute of Cell Biology and Neurobiology; Charité-Universitätsmedizin Berlin (M.S., E.K.), Corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin Institute of Health (BIH), NeuroCure Clinical Research Center Berlin, Germany.
From the Department of Pediatric Neurology (M.N., A.M.K., M.S., E.K.) and Center for Chronically Sick Children, Charité-Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin Institute of Health (BIH); Institute of Clinical Molecular Biology, Christian-Albrechts-University Kiel, University Hospital Schleswig Holstein, Campus Kiel; Department of Genetics and Bioinformatics (P.R.), Kiel; Department of Pediatric Neurology (K.R., A.B.), Children's Hospital Datteln, University Witten/Herdecke, Datteln, Germany; Neuropediatric Unit (R.W.), Karolinska University Hospital, Astrid Lindgren Children's Hospital, Stockholm, Sweden; Department of Pediatrics (J.J., J.D.), University Medical Center Hamburg-Eppendorf, Hamburg, Germany; Division of Pediatric Pulmonology, Allergology and Endocrinology, Department of Pediatrics and Adolescent Medicine (M.B.), Medical University of Vienna, Austria; Department of Pediatric Neurology (M.S.), University Children's Hospital Augsburg; Division of Pediatric Neurology, Department of Pediatrics (K.D.), Hospital Kassel; Department of Pediatrics (M.H., A.Q.), Division of Neuropediatrics and Social Pediatrics, Medical University Rheinisch-Westfälische Technische Hochschule (RWTH) Aachen; Division of Pediatric Neurology, Department of Pediatrics (A.B.), München Klinik Harlaching, Munich; Department of Pediatrics and Pediatric Neurology (H.R.), Georg August University, Göttingen; Department of Paediatric and Adolescent Medicine (C.S.), St Joseph Hospital, Berlin; Department of Pediatrics (K.v.), Vivantes Hospital Friedrichshain, Berlin; Department of Pediatrics (M.D.), Ernst von Bergmann Hospital, Potsdam; Department of Neurology (C.F., F.B.), Charité-Universitätsmedizin Berlin and Berlin School of Mind and Brain, Humboldt-Universität zu Berlin; Charité-Universitätsmedizin Berlin (A.M.K.), Corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health (BIH), Institute of Cell Biology and Neurobiology; Charité-Universitätsmedizin Berlin (M.S., E.K.), Corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin Institute of Health (BIH), NeuroCure Clinical Research Center Berlin, Germany
出版信息
Neurol Neuroimmunol Neuroinflamm. 2023 Mar 22;10(3). doi: 10.1212/NXI.0000000000200102. Print 2023 May.
BACKGROUND AND OBJECTIVES
Anti-N-methyl-D-aspartate receptor encephalitis (NMDARE) is the most common form of autoimmune encephalitis in children and adults. Although our understanding of the disease mechanisms has progressed, little is known about estimating patient outcomes. Therefore, the NEOS (anti-MDAR ncephalitis ne-Year Functional tatus) score was introduced as a tool to predict disease progression in NMDARE. Developed in a mixed-age cohort, it currently remains unclear whether NEOS can be optimized for pediatric NMDARE.
METHODS
This retrospective observational study aimed to validate NEOS in a large pediatric-only cohort of 59 patients (median age of 8 years). We reconstructed the original score, adapted it, evaluated additional variables, and assessed its predictive power (median follow-up of 20 months). Generalized linear regression models were used to examine predictability of binary outcomes based on the modified Rankin Scale (mRS). In addition, neuropsychological test results were investigated as alternative cognitive outcome.
RESULTS
The NEOS score reliably predicted poor clinical outcome (mRS ≥3) in children in the first year after diagnosis ( = 0.0014) and beyond ( = 0.036, 16 months after diagnosis). A score adapted to the pediatric cohort by adjusting the cutoffs of the 5 NEOS components did not improve predictive power. In addition to these 5 variables, further patient characteristics such as the " virus encephalitis (HSE) status" and "age at disease onset" influenced predictability and could potentially be useful to define risk groups. NEOS also predicted cognitive outcome with higher scores associated with deficits of executive function ( = 0.048) and memory ( = 0.043).
DISCUSSION
Our data support the applicability of the NEOS score in children with NMDARE. Although not yet validated in prospective studies, NEOS also predicted cognitive impairment in our cohort. Consequently, the score could help identify patients at risk of poor overall clinical outcome and poor cognitive outcome and thus aid in selecting not only optimized initial therapies for these patients but also cognitive rehabilitation to improve long-term outcomes.
背景与目的
抗 N-甲基-D-天冬氨酸受体脑炎（NMDARE）是儿童和成人中最常见的自身免疫性脑炎形式。尽管我们对疾病机制的理解有所进展，但对于评估患者预后的了解甚少。因此，引入了 NEOS（抗-MDAR 脑炎 1 年功能状态）评分作为预测 NMDARE 疾病进展的工具。该评分最初是在混合年龄队列中开发的，目前尚不清楚 NEOS 是否可以针对儿科 NMDARE 进行优化。
方法
本回顾性观察性研究旨在通过 59 名（中位年龄 8 岁）单纯儿科患者的大样本队列验证 NEOS。我们重建了原始评分，对其进行了调整，评估了其他变量，并评估了其预测能力（中位随访时间为 20 个月）。使用广义线性回归模型根据改良 Rankin 量表（mRS）检查二分类结局的可预测性。此外，还研究了神经心理学测试结果作为替代认知结局。
结果
NEOS 评分可靠地预测了儿童在诊断后 1 年内（ = 0.0014）和诊断后 16 个月（ = 0.036）时的不良临床结局（mRS≥3）。通过调整 5 个 NEOS 成分的截止值来调整儿科队列的评分并没有提高预测能力。除了这 5 个变量外，患者的其他特征，如“病毒脑炎（HSE）状态”和“发病年龄”，也影响了可预测性，并且可能有助于定义风险组。NEOS 还预测了认知结局，得分较高与执行功能（ = 0.048）和记忆（ = 0.043）缺陷相关。
讨论
我们的数据支持 NMDARE 患儿应用 NEOS 评分。尽管尚未在前瞻性研究中得到验证，但 NEOS 也预测了我们队列中的认知障碍。因此，该评分可以帮助识别预后不良和认知功能障碍风险较高的患者，从而不仅有助于为这些患者选择优化的初始治疗方案，还可以选择认知康复以改善长期结局。
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