Clinical and Magnetic Resonance Imaging Outcome Predictors in Pediatric Anti-N-Methyl-D-Aspartate Receptor Encephalitis
Clinical and Magnetic Resonance Imaging Outcome Predictors in Pediatric Anti-N-Methyl-D-Aspartate Receptor Encephalitis
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DOI：
10.1002/ana.25754
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发表时间：
2020-05-29
影响因子：
11.2
通讯作者：
Finke, Carsten
中科院分区：
文献类型：
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作者：
Bartels, Frederik;Krohn, Stephan;Finke, Carsten
目的评估欧洲多中心儿童抗N-甲基-D-天冬氨酸受体脑炎(NMDARE)患者的症状、临床和磁共振成像(MRI)表现，并进行纵向体积MRI分析。方法对38例NMDARE儿童(中位年龄12.9岁，范围1~18岁)和82例MRI扫描进行体积MRI分析。应用混合效应模型和脑体积z评分估计纵向脑体积发育。结果38例患者中15例(39.5%)MRI表现异常，主要表现为脑白质T2/液体衰减反转恢复高信号。体积MRI分析显示，NMDARE儿童的全脑和灰质以及海马区和基底节体积减少。纵向混合效应模型和z分数转换显示患者未能实现预期年龄的脑发育。重要的是，与MRI正常的患者相比，发病时MRI异常的患者预后更差(1，发病率比=3.5，95%可信区间[CI]=1.3-9.31，p=0.012)。经发病时间校正后的有序Logistic回归模型证实，发病时MRI异常(OR=9.90，95%CI=2.51~17.28，P=0.009)、感觉运动障碍(OR=13.71，95%CI=2.68~24.73，P=0.015)、治疗延迟和GT；4周(OR=5.15，95%CI=0.47~9.82，P=0.031)是预后不良的独立预测因素。异常的MRI表现，临床表现有感觉运动障碍，以及治疗延迟4周与较差的临床结果相关。这些特征代表了儿科NMDARE中有前景的预后生物标志物。Ann Neurol 2020
Objective To evaluate disease symptoms, and clinical and magnetic resonance imaging (MRI) findings and to perform longitudinal volumetric MRI analyses in a European multicenter cohort of pediatric anti-N-methyl-D-aspartate receptor encephalitis (NMDARE) patients.Methods We studied 38 children with NMDARE (median age = 12.9 years, range =1-18) and a total of 82 MRI scans for volumetric MRI analyses compared to matched healthy controls. Mixed-effect models and brain volume z scores were applied to estimate longitudinal brain volume development. Ordinal logistic regression and ordinal mixed models were used to predict disease outcome and severity.Results Initial MRI scans showed abnormal findings in 15 of 38 (39.5%) patients, mostly white matter T2/fluid-attenuated inversion recovery hyperintensities. Volumetric MRI analyses revealed reductions of whole brain and gray matter as well as hippocampal and basal ganglia volumes in NMDARE children. Longitudinal mixed-effect models and z score transformation showed failure of age-expected brain growth in patients. Importantly, patients with abnormal MRI findings at onset were more likely to have poor outcome (Pediatric Cerebral Performance Category score > 1, incidence rate ratio = 3.50, 95% confidence interval [CI] = 1.31-9.31, p = 0.012) compared to patients with normal MRI. Ordinal logistic regression models corrected for time from onset confirmed abnormal MRI at onset (odds ratio [OR] = 9.90, 95% CI = 2.51-17.28, p = 0.009), a presentation with sensorimotor deficits (OR = 13.71, 95% CI = 2.68-24.73, p = 0.015), and a treatment delay > 4 weeks (OR = 5.15, 95% CI = 0.47-9.82, p = 0.031) as independent predictors of poor clinical outcome.Interpretation Children with NMDARE exhibit significant brain volume loss and failure of age-expected brain growth. Abnormal MRI findings, a clinical presentation with sensorimotor deficits, and a treatment delay > 4 weeks are associated with worse clinical outcome. These characteristics represent promising prognostic biomarkers in pediatric NMDARE. ANN NEUROL 2020