Pediatric N-Methyl-d-Aspartate Receptor Encephalitis is Differentiated From Encephalitis Mimickers by Early Elevated Blood Pressure

IF 2.1 3区医学 Q2 CLINICAL NEUROLOGY

Pediatric neurology Pub Date : 2025-08-14 DOI:10.1016/j.pediatrneurol.2025.08.006

Naomi R. Kass BA , Elizabeth Ledbetter BS , Timothy A. Erickson PhD , Jonathan M. Yarimi MD , Anthony Zoghbi MD , Eyal Muscal MD , Kristy O. Murray DVM, PhD , Kristen S. Fisher DO , Alexander J. Sandweiss MD, PhD

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引用次数: 0

Abstract

Background

Pediatric N-methyl-d-aspartate receptor (NMDAR) encephalitis (pNMDARE) is characterized by severe neuropsychiatric symptoms and prolonged hospitalization and recovery. Early pNMDARE diagnosis is complicated by neuropsychiatric mimickers requiring strong clinical suspicion to escalate to the required lumbar puncture (LP), delaying diagnosis and treatment. Since autonomic dysfunction is a cardinal feature of pNMDARE, we hypothesized that early vital signs serve as a potential noninvasive biomarker to screen for appropriate escalation of pNMDARE evaluation.

Methods

This is a retrospective, case-control analysis of patients with pNMDARE between 2021 and 2023. Patients diagnosed with pNMDARE as determined by clinical presentation and positive cerebrospinal fluid (CSF) antibodies (Abs) were compared with control subjects who were evaluated for pNMDARE, including an LP, but were negative.

Results

Fifty-seven patients were included for analysis: 23 diagnosed with pNMDARE and 34 without. When standardized for age, sex, and height, the pNMDARE group had higher systolic and diastolic BP percentiles (BP%ile) compared with the anti-NMDAR Ab–negative group (systolic BP%: 95.0% ± 2.3% vs 68.8% ± 4.4%, respectively, P < 0.001; diastolic BP%: 88.3% ± 2.8% vs 61.3% ± 4.1%, respectively; P < 0.001). A receiver operator curve indicated that the shortest Euclidian distance for systolic BP%ile was 99^th%ile (specificity, 85.3%; sensitivity, 65.2% P < 0.0001) and for diastolic BP%ile was 88.5^th%ile (specificity, 73.5%; sensitivity, 65.2%; P < 0.005).

Conclusions

Although CSF Ab is required for the diagnosis of pNMDARE, screening tools may help hasten clinical suspicion for the need for an LP. We identified elevated BP as a potential differentiating early clinical marker specific to pNMDARE. This fact corroborates our current understanding of dysautonomia in pNMDARE and provides a potential clinical marker suitable for future research and validation.

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儿童n -甲基-d-天冬氨酸受体脑炎与模拟脑炎早期血压升高的区别

儿童n -甲基-d-天冬氨酸受体（NMDAR）脑炎（pNMDARE）的特点是严重的神经精神症状和长期住院和恢复。pNMDARE的早期诊断因神经精神模仿而复杂化，需要强烈的临床怀疑才能升级到需要腰椎穿刺（LP），从而延误诊断和治疗。由于自主神经功能障碍是pNMDARE的主要特征，我们假设早期生命体征可以作为一种潜在的无创生物标志物，用于筛选pNMDARE评估的适当升级。方法对2021 - 2023年pNMDARE患者进行回顾性病例对照分析。通过临床表现和脑脊液（CSF）抗体（Abs）阳性诊断为pNMDARE的患者与进行pNMDARE评估（包括LP）但呈阴性的对照组进行比较。结果纳入57例患者，其中23例诊断为pNMDARE， 34例未诊断为pNMDARE。当年龄、性别和身高标准化时，pNMDARE组的收缩压和舒张压百分位数（BP%ile）高于抗nmdar抗体阴性组（收缩压%:95.0%±2.3% vs 68.8%±4.4%,P < 0.001；舒张压%:88.3%±2.8% vs 61.3%±4.1%,P < 0.001）。受体操作者曲线显示，收缩压%ile的最短欧氏距离为99 % %（特异性85.3%，敏感性65.2% P < 0.0001），舒张压%ile的最短欧氏距离为88.5% %（特异性73.5%，敏感性65.2% P < 0.005）。结论：虽然诊断pNMDARE需要CSF Ab，但筛查工具可能有助于加快临床怀疑是否需要LP。我们将血压升高确定为pNMDARE特异性的潜在早期临床标志物。这一事实证实了我们目前对pNMDARE中自主神经异常的理解，并为未来的研究和验证提供了一个潜在的临床标志。

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来源期刊

Pediatric neurology 医学-临床神经学

CiteScore

4.80

自引率

2.60%

发文量

176

审稿时长

78 days

期刊介绍： Pediatric Neurology publishes timely peer-reviewed clinical and research articles covering all aspects of the developing nervous system. Pediatric Neurology features up-to-the-minute publication of the latest advances in the diagnosis, management, and treatment of pediatric neurologic disorders. The journal''s editor, E. Steve Roach, in conjunction with the team of Associate Editors, heads an internationally recognized editorial board, ensuring the most authoritative and extensive coverage of the field. Among the topics covered are: epilepsy, mitochondrial diseases, congenital malformations, chromosomopathies, peripheral neuropathies, perinatal and childhood stroke, cerebral palsy, as well as other diseases affecting the developing nervous system.