髓鞘少突胶质细胞糖蛋白抗体相关性急性播散性脑脊髓炎患者的脑生长受损。

Impaired Brain Growth in Myelin Oligodendrocyte Glycoprotein Antibody-Associated Acute Disseminated Encephalomyelitis.

机构信息

From the Department of Neurology (F.B., G.C., C.F.), Charité-Universitätsmedizin Berlin; Berlin Institute of Health at Charité-Universitätsmedizin Berlin (F.B.); Berlin School of Mind and Brain (F.B., C.F.), Humboldt-Universität zu Berlin; Witten/Herdecke University (B.B., Annikki Bertolini, K.R.), Department of Pediatric Neurology, Children's Hospital Datteln; Charité-Universitätsmedizin Berlin (A.A.), Institute of Biometry and Clinical Epidemiology; Department of Pediatric Neurology and Developmental Medicine (Astrid Blaschek), LMU, Dr. von Hauner Children's Hospital, Munich; Department of Pediatric Neurology (E.M.W.), Olgahospital/Klinikum Stuttgart; Department of General Pediatrics, Neonatology and Pediatric Cardiology, Medical Faculty (M.K.), Heinrich-Heine-University Düsseldorf, Germany; Department of Pediatric I, Pediatric Neurology (M.B.), Medical University of Innsbruck, Austria; Department of Radiology (R.C., A.W.-P.), Children's Hospital Datteln, Witten/Herdecke University, Germany; Department of Pediatrics and Adolescent Medicine (S.L.), Hospital Dritter Orden, Munich, Germany; Department Neuropediatrics (M.S.), Regional Hospital of Bolzano, Italy; Department of Pediatrics (P.K.), Städtisches Klinikum Karlsruhe, Germany; Department of Pediatrics, Brandenburg (T.R.), Helios Klinik Hohenstücken, Germany; and Clinical Department of Neurology (M.R.), Medical University of Innsbruck, Innsbruck, Austria.

出版信息

Neurol Neuroimmunol Neuroinflamm. 2023 Feb 8;10(2). doi: 10.1212/NXI.0000000000200066. Print 2023 Mar.

DOI:10.1212/NXI.0000000000200066

PMID:36754833

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9909582/

Abstract

BACKGROUND AND OBJECTIVES

Acute disseminated encephalomyelitis (ADEM) is the most common phenotype in pediatric myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease. A previous study demonstrated impaired brain growth in ADEM. However, the effect of MOG antibodies on brain growth remains unknown. Here, we performed brain volume analyses in MOG-positive and MOG-negative ADEM at onset and over time.

METHODS

In this observational cohort study, we included a total of 62 MRI scans from 24 patients with ADEM (54.2% female; median age 5 years), of which 16 (66.7%) were MOG positive. Patients were compared with healthy controls from the NIH pediatric MRI data repository and a matched local cohort. Mixed-effect models were applied to assess group differences and other relevant factors, including relapses.

RESULTS

At baseline and before any steroid treatment, patients with ADEM, irrespective of MOG antibody status, showed reduced brain volume compared with matched controls (median [interquartile range] 1,741.9 cm3 [1,645.1-1,805.2] vs 1,810.4 cm [1,786.5-1,836.2]). Longitudinal analysis revealed reduced brain growth for both MOG-positive and MOG-negative patients with ADEM. However, MOG-negative patients showed a stronger reduction (-138.3 cm [95% CI -193.6 to -82.9]) than MOG-positive patients (-50.0 cm [-126.5 to -5.2]), independent of age, sex, and treatment. Relapsing patients (all MOG positive) showed additional brain volume loss (-15.8 cm [-68.9 to 37.3]).

DISCUSSION

Patients with ADEM exhibit brain volume loss and failure of age-expected brain growth. Importantly, MOG-negative status was associated with a more pronounced brain volume loss compared with MOG-positive patients.

摘要

背景与目的

急性播散性脑脊髓炎（ADEM）是儿童髓鞘少突胶质细胞糖蛋白（MOG）抗体相关疾病中最常见的表型。先前的研究表明 ADEM 存在脑生长受损。然而，MOG 抗体对脑生长的影响尚不清楚。在此，我们对发病时及发病后不同时间点的 MOG 阳性和 MOG 阴性 ADEM 患者进行脑容量分析。

方法

在这项观察性队列研究中，我们纳入了 24 例 ADEM 患者的总共 62 次 MRI 扫描（54.2%为女性；中位年龄 5 岁），其中 16 例（66.7%）为 MOG 阳性。患者与 NIH 儿科 MRI 数据存储库中的健康对照者和当地匹配队列进行比较。应用混合效应模型评估组间差异及其他相关因素，包括复发。

结果

在基线及任何类固醇治疗前，无论 MOG 抗体状态如何，ADEM 患者的脑容量均较匹配对照组减少（中位数[四分位数间距] 1741.9cm3[1645.1-1805.2]比 1810.4cm3[1786.5-1836.2]）。纵向分析显示 MOG 阳性和 MOG 阴性 ADEM 患者的脑生长均减少。然而，MOG 阴性患者的脑容量减少更明显（-138.3cm[95%CI-193.6 至-82.9]），而 MOG 阳性患者为-50.0cm[-126.5 至-5.2]），与年龄、性别和治疗无关。复发性患者（均为 MOG 阳性）则表现出额外的脑容量损失（-15.8cm[-68.9 至 37.3]）。