疑似自身免疫性脑炎患者血清和脑脊液中抗髓鞘少突胶质细胞糖蛋白（MOG）抗体的频率：一项巴西多中心研究的结果

Frequency of anti-MOG antibodies in serum and CSF of patients with possible autoimmune encephalitis: Results from a Brazilian multicentric study.

作者信息

de Freitas Dias Bruna, Toso Fabio Fieni, Barreto Maria Eduarda Slhessarenko Fraife, Dellavance Alessandra, Thomaz Rodrigo Barbosa, Kowacs Pedro André, Teive Hélio, Spitz Mariana, Juliano Aline Freire Borges, Rocha Letícia Januzi de Almeida, Granja Valéria Nogueira Tobias, Braga-Neto Pedro, Nóbrega Paulo Ribeiro, Oliveira-Filho Jamary, Dias Ronaldo Maciel, Amoras Jaene Andrade Pacheco, Pereira Renata Brasileiro Reis, Júnior Clécio de Oliveira Godeiro, Maia Fernanda Martins, Santos Mara Lúcia, de Melo Eduardo Sousa, Júnior Adaucto Wanderley da Nóbrega, Lin Katia, Paolilo Renata Barbosa, Krueger Mariana Braatz, Barsottini Orlando Graziani Povoas, Endmayr Verena, Andrade Luís Eduardo Coelho, Hoftberger Romana, Dutra Lívia Almeida

机构信息

Instituto do Cérebro, Hospital Israelita Albert Einstein, São Paulo, Brazil.

Research and Development Division, Fleury Group, São Paulo, Brazil.

出版信息

Mult Scler Relat Disord. 2024 Dec;92:106171. doi: 10.1016/j.msard.2024.106171. Epub 2024 Nov 14.

DOI:10.1016/j.msard.2024.106171

PMID:39579646

Abstract

INTRODUCTION

MOGAD encephalitis and ADEM share several clinical features with autoimmune encephalitis (AE) associated with antineuronal antibodies (ANeA); nonetheless, treatment and prognosis differ. Anti-MOG antibodies (abs) are not routinely tested in possible AE, and epidemiological studies on MOGAD encephalitis are scarce.

OBJECTIVES

To determine the frequency of anti-MOG abs in the serum and CSF in a cohort of possible AE and to compare the clinical characteristics of MOGAD patients and those with seropositive AE.

METHODS

481 patients with possible AE from the Brazilian Autoimmune Encephalitis Network underwent tissue-based assay and cell-based assay (CBA) for ANeA. Anti-MOG abs were assessed in serum and CSF with in-house CBA. Clinical and laboratory characteristics of MOGAD and seropositive AE patients were compared.

RESULTS

Of the 481 patients, 87 (18 %) had ANeA, and 17 (3.5 %) had anti-MOG abs. Three AE patients with anti-MOG abs and ANeA were excluded from further analysis. Anti-MOG abs were detected in 4 (1.2 %) of the 328 adults and 10 (6.5 %) of the 153 children. Of the 14 patients with MOGAD, nine had ADEM (mostly children), and five had encephalitis (including three adults). Only one patient with ADEM had anti-MOG abs exclusively in CSF. All patients with MOGAD encephalitis were seropositive for anti-MOG abs, and three had normal brain MRI. Patients with MOGAD had fewer behavioral changes (MOGAD 21 % x AE 96 %, p ≤ 0.0001) and movement disorders (MOGAD 42 % x AE 81 %, p = 0.0017) and more demyelinating symptoms, such as myelitis and optic neuritis (MOGAD 14 % x AE 0 %, p = 0.013).

CONCLUSION

Approximately 3.5 % of patients with possible AE harbor anti-MOG abs, and 0.9 % of the adults had MOGAD encephalitis. Anti-MOG abs were more frequent than other ANeAs regularly tested in AE. We provide evidence that MOGAD is a differential diagnosis in possible AE, even in adult patients with normal brain MRI, and that serum anti-MOG should be considered as an add-on diagnostic tool in AE among adults and pediatric patients.

摘要

引言

MOGAD 脑炎和急性播散性脑脊髓炎（ADEM）与抗神经元抗体（ANeA）相关的自身免疫性脑炎（AE）有若干共同临床特征；尽管如此，治疗方法和预后有所不同。在疑似 AE 患者中，抗髓鞘少突胶质细胞糖蛋白（MOG）抗体（抗体）并非常规检测项目，且关于 MOGAD 脑炎的流行病学研究较少。

目的

确定一组疑似 AE 患者血清和脑脊液中抗 MOG 抗体的频率，并比较 MOGAD 患者与血清学阳性 AE 患者的临床特征。

方法

来自巴西自身免疫性脑炎网络的 481 例疑似 AE 患者接受了基于组织的检测和基于细胞的检测（CBA）以检测 ANeA。采用内部 CBA 法评估血清和脑脊液中的抗 MOG 抗体。比较了 MOGAD 和血清学阳性 AE 患者的临床和实验室特征。

结果

481 例患者中，87 例（18%）有 ANeA，17 例（3.5%）有抗 MOG 抗体。3 例同时有抗 MOG 抗体和 ANeA 的 AE 患者被排除在进一步分析之外。在 328 例成人患者中有 4 例（1.2%）检测到抗 MOG 抗体，在 153 例儿童患者中有 10 例（6.5%）检测到。在 14 例 MOGAD 患者中，9 例有 ADEM（大多为儿童），5 例有脑炎（包括 3 例成人）。仅 1 例 ADEM 患者脑脊液中仅存在抗 MOG 抗体。所有 MOGAD 脑炎患者抗 MOG 抗体血清学均为阳性，3 例脑部 MRI 正常。MOGAD 患者行为改变较少（MOGAD 21% vs AE 96%，p≤0.0001），运动障碍较少（MOGAD 42% vs AE 81%，p = 0.0017），脱髓鞘症状较多，如脊髓炎和视神经炎（MOGAD 14% vs AE 0%，p = 0.013）。

结论

约 3.5%的疑似 AE 患者携带抗 MOG 抗体，0.9%的成人患有 MOGAD 脑炎。抗 MOG 抗体比 AE 中常规检测的其他 ANeA 更常见。我们提供的证据表明，即使在脑部 MRI 正常的成年患者中，MOGAD 也是疑似 AE 的鉴别诊断，血清抗 MOG 应被视为成人和儿童 AE 的补充诊断工具。