Abstract

Purpose: To evaluate the clinical differences between pediatric and adult patients with myelin oligodendrocyte glycoprotein antibody-associated encephalomyelitis (MOG-EM).Methods: We retrospectively reviewed the clinical features of pediatric and adult patients with MOG-EM in our center between November 2015 and October 2020.Results: Twenty-eight pediatric patients and 25 adults were admitted to our study. Bilateral optic neuritis (BON) was the most common initial phenotype in the pediatric group but less common in the adult group (28.57 vs. 0%, p = 0.0119). Almost half of the adult patients presented with neuromyelitis optica spectrum disease (NMOSD), which was less prevalent among the pediatrics (48 vs. 21.43%, p = 0.0414). Visual impairment was the most common symptom in both groups during the initial attack (pediatric group, 39.29%; adult group, 64%) and throughout the full course (pediatric group, 57.14%; adult group, 72%). More pediatric patients suffered from fever than adult patients at onset (pediatric group, 28.57%; adult group, 4%; p = 0.0442) and throughout the full course (pediatric group, 39.29%; adult group, 12%; p = 0.0245). Multiple patchy lesions in subcortical white matter (pediatric group, 40.74%; adult group, 45%), periventricular (pediatric group, 25.93%; adult group, 35%), infratentorial (pediatric group, 18.52%; adult group, 30%) and deep gray matter (pediatric group, 25.93%; adult group, 20%) were frequent in all cases, no significant difference was found between the two groups, while bilateral optic nerve involvement was more frequent in pediatric group (61.54 vs. 14.29%, p = 0.0042) and unilateral optic nerve involvement was higher in adult group (64.29 vs. 15.38%, p = 0.0052). At the last follow-up, adult patients had a higher average EDSS score (median 1.0, range 0–3) than pediatrics (median 0.0, range 0–3), though not significant (p = 0.0752). Patients aged 0–9 years (61.54%) and 10–18 years (70%), and patients presenting with encephalitis/meningoencephalitis (100%) and ADEM (75%) were more likely to recover fully.Conclusions: Visual impairment was the dominant symptom in both pediatric and adult patients, while fever was more frequent in pediatric patients. Data suggested that BON and bilateral optic nerve involvement were more common in pediatric cases whereas NMOSD and unilateral optic nerve involvement were more prevalent in adults. The younger patients and patients presenting with encephalitis/meningoencephalitis and ADEM tended to recover better.

Highlights

  • Myelin oligodendrocyte glycoprotein (MOG) is a glycoprotein expressed on the outer surface of myelin sheaths in the central nervous system (CNS) [1]

  • The most common initial phenotype was bilateral optic neuritis (ON) (BON) (28.57%, 8/28); it was more prevalent in the pediatric than in the adult group (0%, 0/25; p = 0.0119)

  • Almost half of the adult patients presented with neuromyelitis optica spectrum disease (NMOSD) (48%, 12/25), which was more prevalent than in the pediatric patients (21.43%, 6/28; p = 0.0414)

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Summary

Introduction

Myelin oligodendrocyte glycoprotein (MOG) is a glycoprotein expressed on the outer surface of myelin sheaths in the central nervous system (CNS) [1]. Immunoglobulin G (IgG) against MOG is considered to be a potential autoantibody that can induce demyelination in the CNS, and this was supported by the discovery of MOG-IgG in patients with multiple sclerosis (MS) and reports on the clinical relevance of antibodies against myelin oligodendrocyte glycoprotein in different types of MS [3]. Besides MS and NMOSD, MOG-IgG is considered to be related to other idiopathic inflammatory demyelinating diseases (IIDDs), including acute disseminated encephalomyelitis (ADEM), optic neuritis (ON), transverse myelitis (TM), and clinically isolated syndrome (CIS) [5,6,7]. It is currently defined as MOG antibody-associated encephalomyelitis (MOG-EM) [8, 9]

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