Clinical characteristics analysis of 24 cases of pediatric MOG antibody-associated diseases

IF 2.9 3区 医学 Q2 CLINICAL NEUROLOGY
Yanping Wang , Xiangyun Guo , Lin Zhang , Ying Hua , Miao Jing , Xiaoyue Hu , Xiaochun Fan , Mingxia Sun , Yanshan Liu , Jianbiao Wang
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Abstract

Objective

To investigate the clinical characteristics of children with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD).

Methods

A retrospective analysis was conducted on the clinical data, antibody tests, imaging, and factors associated with recurrence in 24 children diagnosed with MOGAD at Wuxi Children's Hospital from December 2017 to December 2023.

Results

Among the 24 included children, the clinical characteristics at the onset of the first episode included fever (12 cases), headache (8), decreased vision (7), drowsiness (6), convulsions (5), ataxia (3), paralysis of both lower limbs (2), urinary and fecal incontinence (2), and central facial palsy (1). Among them, one case started with paralysis of both lower limbs and urinary retention, and electromyography suggested the involvement of peripheral nerves, leading to the diagnosis of MOG antibody-associated central and peripheral demyelinating syndrome (MOGAD-CCPD). Cranial MRI abnormalities were observed in 20 children, and spinal MRI abnormalities were noted in 6 children. All children responded well to corticosteroids and intravenous immunoglobulin, but 7 children experienced a relapse. Among them, 3 children achieved disease control after the addition of mycophenolate mofetil (CellCept), with no further relapses observed during follow-up.

Conclusion

The disease course of MOGAD can be monophasic or relapsing. Most children have a good response to acute phase treatments. For those who relapse, immunosuppressants can be added as maintenance therapy, and the clinical prognosis is generally good. This article reports the first highly rare case in China of MOGAD-CCPD in childhood, suggesting that MOG IgG may serve as a potential biomarker associated with CCPD.
24 例小儿 MOG 抗体相关疾病的临床特征分析
摘要] 目的 探讨髓鞘少突胶质细胞糖蛋白抗体相关性疾病(MOGAD)患儿的临床特征。方法 对2017年12月至2023年12月无锡市儿童医院确诊的24例MOGAD患儿的临床资料、抗体检测、影像学检查及复发相关因素进行回顾性分析。结果纳入的24例患儿中,首次发病时的临床特征包括发热(12例)、头痛(8例)、视力下降(7例)、嗜睡(6例)、抽搐(5例)、共济失调(3例)、双下肢瘫痪(2例)、大小便失禁(2例)、中枢性面瘫(1例)。其中一例患者开始时出现双下肢瘫痪和尿潴留,肌电图检查提示周围神经受累,因此被诊断为 MOG 抗体相关中枢和周围脱髓鞘综合征(MOGAD-CCPD)。20名患儿的头颅磁共振成像出现异常,6名患儿的脊柱磁共振成像出现异常。所有患儿对皮质类固醇和静脉注射免疫球蛋白均反应良好,但有7名患儿病情复发。结论 MOGAD 的病程可以是单相的,也可以是复发的。大多数患儿对急性期治疗反应良好。对于复发的患儿,可加用免疫抑制剂作为维持治疗,临床预后一般良好。本文报道了中国首例高度罕见的儿童MOGAD-CCPD病例,提示MOG IgG可能是与CCPD相关的潜在生物标志物。
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
CiteScore
5.80
自引率
20.00%
发文量
814
审稿时长
66 days
期刊介绍: Multiple Sclerosis is an area of ever expanding research and escalating publications. Multiple Sclerosis and Related Disorders is a wide ranging international journal supported by key researchers from all neuroscience domains that focus on MS and associated disease of the central nervous system. The primary aim of this new journal is the rapid publication of high quality original research in the field. Important secondary aims will be timely updates and editorials on important scientific and clinical care advances, controversies in the field, and invited opinion articles from current thought leaders on topical issues. One section of the journal will focus on teaching, written to enhance the practice of community and academic neurologists involved in the care of MS patients. Summaries of key articles written for a lay audience will be provided as an on-line resource. A team of four chief editors is supported by leading section editors who will commission and appraise original and review articles concerning: clinical neurology, neuroimaging, neuropathology, neuroepidemiology, therapeutics, genetics / transcriptomics, experimental models, neuroimmunology, biomarkers, neuropsychology, neurorehabilitation, measurement scales, teaching, neuroethics and lay communication.
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