Clinical, Radiologic, and Optical Coherence Tomographic Features of Pediatric Acute Disseminated Encephalomyelitis: Comparison of Myelin Oligodendrocyte Glycoprotein Antibody–Positive and –Negative Cases

IF 2.1 3区医学 Q2 CLINICAL NEUROLOGY

Pediatric neurology Pub Date : 2025-07-28 DOI:10.1016/j.pediatrneurol.2025.07.013

Linda Nguyen MD, PhD , Sumit Singh MD , Cynthia X. Wang MD , Benjamin M. Greenberg MD, MHS

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引用次数: 0

Abstract

Background

Acute disseminated encephalomyelitis (ADEM) typically presents as a monophasic demyelinating disorder associated with multifocal neurological symptoms and encephalopathy in children. Myelin oligodendrocyte glycoprotein antibody (MOG-IgG) is detected in a subset of patients and may be linked to recurrence risk. This study evaluated the clinical, radiologic, and optic coherence tomographic (OCT) features of children with ADEM based on MOG-IgG serostatus.

Methods

We retrospectively reviewed pediatric ADEM cases with MOG-IgG testing at initial presentation between 2017 and 2024.

Results

Of 38 children with ADEM, 27 (71.1%) were MOG-IgG positive at initial presentation. Reported decreased vision and optic nerve involvement on imaging were more frequent in MOG-IgG-positive children (18.5% vs 0.0% and 36.4% vs 0.0%, respectively), although these differences did not reach statistical significance. Hospitalization duration and need for intensive care unit stay were similar between groups. On follow-up imaging, complete lesion resolution was significantly more common in MOG-IgG-negative children (75.5% vs 22.2%, P = 0.014). Among 30 children with geater than or equal to six months of follow-up, good recovery (modified Rankin scale score <2) occurred in 65.5% and relapse in 23.1%, with no significant differences between groups. OCT revealed a trend toward increased frequency of retinal nerve fiber layer thinning in MOG-IgG-positive children (50.0% vs 0.0%).

Conclusions

In this single-center study, MOG-IgG-positive ADEM is associated with greater optic nerve involvement, whereas MOG-IgG-negative ADEM showed more frequent complete lesion resolution. These findings support incorporating MOG-IgG testing, dedicated orbital imaging, and OCT into the diagnostic and follow-up evaluation of pediatric ADEM.

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小儿急性播散性脑脊髓炎的临床、放射学和光学相干断层扫描特征：髓鞘少突胶质细胞糖蛋白抗体阳性和阴性病例的比较

背景：急性播散性脑脊髓炎（ADEM）通常表现为儿童多灶性神经系统症状和脑病相关的单相脱髓鞘疾病。髓鞘少突胶质细胞糖蛋白抗体（MOG-IgG）在一部分患者中检测到，可能与复发风险有关。本研究评估了基于MOG-IgG血清状态的儿童ADEM的临床、放射学和光学相干断层扫描（OCT）特征。方法回顾性分析2017年至2024年首次就诊时进行MOG-IgG检测的儿童ADEM病例。结果38例ADEM患儿中，27例（71.1%）初次就诊时MOG-IgG阳性。mog - igg阳性的儿童在影像学上报告的视力下降和视神经受累更频繁（分别为18.5%对0.0%和36.4%对0.0%），尽管这些差异没有达到统计学意义。两组间住院时间和重症监护病房住院需求相似。在随访影像中，mog - igg阴性儿童的病变完全消退更为常见（75.5% vs 22.2%, P = 0.014）。30例随访大于等于6个月的患儿中，恢复良好（改良Rankin量表评分<；2）者占65.5%，复发者占23.1%，组间差异无统计学意义。OCT显示mog - igg阳性儿童视网膜神经纤维层变薄的频率增加（50.0% vs 0.0%）。结论在这项单中心研究中，mog - igg阳性的ADEM与更大的视神经受累有关，而mog - igg阴性的ADEM则表现出更频繁的病变完全消退。这些发现支持将MOG-IgG检测、专用眼眶成像和OCT纳入儿童ADEM的诊断和随访评估。

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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.