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Clinical analysis of myelin oligodendrocyte glycoprotein antibody-associated disease in a diverse cohort of children: A single-center observational study

医学 急性播散性脑脊髓炎 儿科 队列 单中心 疾病 内科学 免疫学
作者
Linda Nguyen,Cynthia X. Wang,R. Hernandez,Benjamin Greenberg
出处
期刊:Multiple sclerosis and related disorders [Elsevier BV]
卷期号:84: 105497-105497 被引量:3
标识
DOI:10.1016/j.msard.2024.105497
摘要

Background Prognostic markers for relapse and neurological disability following the first clinical event in children with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) remain lacking. We investigated the clinical profiles and early prognostic factors associated with relapsing disease or impaired functional outcome in a large single-center cohort of pediatric MOGAD. Methods We retrospectively analyzed the clinical and paraclinical data and treatment outcomes of children with MOGAD seen at Children's Health in Dallas, Texas from 2009 to 2022. Univariate analyses were used to evaluate factors from initial event associated with relapsing disease course and impaired functional outcome (modified Rankin scale [mRS] >1) at final follow-up. Results Our cohort comprised of 87 children of diverse race/ethnicity. Presentation with acute disseminated encephalomyelitis (ADEM) was more frequent in children aged ≤8 years and Caucasian background, whereas presentation with optic neuritis was more common in children aged >8 years and other race/ethnicity. 44.3% (27/61) had relapsing disease course, of whom 48.0% had multiple relapses. 30.3% (23/76) had final mRS >1. Children with abnormal electroencephalogram had reduced relapse risk. Children with ADEM presentation, severe disease, low MOG-IgG titer, and central and systemic inflammation (represented by cerebrospinal fluid pleocytosis and serum leukocytosis, respectively) at onset had higher likelihood of final mRS >1. Conclusion Abnormal electroencephalogram at the first event was associated with reduced relapse risk while disease severity and peripheral inflammation significantly contributed to final neurological disability. Further studies are needed to validate these findings as early risk factors for disability and relapse and to identify optimal treatment strategies.
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