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巴西髓鞘少突胶质细胞糖蛋白抗体相关性疾病的临床谱:单中心经验。

Clinical spectrum of myelin oligodendrocyte glycoprotein antibody-associated disease in Brazil: a single-center experience.

机构信息

Universidade de São Paulo, Faculdade de Medicina de Ribeirão Preto, Departamento de Neurociências e Ciências do Comportamento, Ribeirão Preto SP, Brazil.

Universidade de São Paulo, Faculdade de Medicina de Ribeirão Preto, Departamento de Oftalmologia, Otorrinolaringologia e Cirurgia de Cabeça e Pescoço, São Paulo SP, Brazil.

出版信息

Arq Neuropsiquiatr. 2023 Nov;81(11):980-988. doi: 10.1055/s-0043-1777002. Epub 2023 Nov 30.

DOI:10.1055/s-0043-1777002
PMID:38035583
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10689103/
Abstract

BACKGROUND

Anti-myelin oligodendrocyte glycoprotein (anti-MOG) antibody-associated disease (MOGAD) is an immune-mediated neurological disorder with a broad spectrum of clinical presentation that is often difficult to distinguish from other demyelinating diseases, such as multiple sclerosis and neuromyelitis optica spectrum disorder.

OBJECTIVE

To describe the clinical and paraclinical characteristics of MOGAD in a Brazilian tertiary center.

METHODS

We retrospectively reviewed the records of adult and pediatric patients who tested positive for anti-MOG antibodies and presented with clinical and radiological diseases compatible with MOGAD.

RESULTS

Forty-one patients (10 children) were included: 56% female, 58% Caucasian, mean age at onset 31 years (range 6-64), with a mean disease duration of 59.6 months (range 1-264 months). The most frequent onset presentation was optic neuritis (68%), acute disseminated encephalomyelitis (ADEM, 12%), and myelitis (10%). A monophasic disease course was observed in 49%. EDSS median was 2.1 at the last visit. Most patients (83%) were under continuous immunosuppressive treatment. Azathioprine was the first-line treatment in 59%. In all ADEM cases, conus, and root involvement was radiologically observed on MRI.

CONCLUSION

Brazilian MOGAD patients presented with a similar spectrum of previously reported MOGAD phenotypes. Conus and spinal root involvement seems to be frequently present in MOGAD-ADEM and could serve as radiologic characteristics of this clinical entity.

摘要

背景

抗髓鞘少突胶质细胞糖蛋白(anti-MOG)抗体相关性疾病(MOGAD)是一种免疫介导的神经系统疾病,其临床表现广泛,常难以与多发性硬化症和视神经脊髓炎谱系障碍等其他脱髓鞘疾病相区别。

目的

描述巴西一家三级中心 MOGAD 的临床和辅助检查特征。

方法

我们回顾性分析了抗 MOG 抗体阳性且具有 MOGAD 临床和影像学特征的成人和儿童患者的病历记录。

结果

共纳入 41 例患者(10 例为儿童):女性占 56%,白种人占 58%,发病年龄平均为 31 岁(6-64 岁),平均病程为 59.6 个月(1-264 个月)。最常见的首发表现为视神经炎(68%)、急性播散性脑脊髓炎(ADEM,12%)和脊髓炎(10%)。49%的患者表现为单相病程。末次随访时 EDSS 中位数为 2.1。大多数患者(83%)持续接受免疫抑制治疗。59%的患者一线治疗药物为硫唑嘌呤。所有 ADEM 病例的 MRI 均显示圆锥和神经根受累。

结论

巴西 MOGAD 患者的临床表现与先前报道的 MOGAD 表型相似。圆锥和脊髓根受累似乎在 MOGAD-ADEM 中较为常见,可能成为该临床实体的影像学特征。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e5d7/10689103/973ce6e5cb74/10-1055-s-0043-1777002-i230121-2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e5d7/10689103/973ce6e5cb74/10-1055-s-0043-1777002-i230121-2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e5d7/10689103/973ce6e5cb74/10-1055-s-0043-1777002-i230121-2.jpg

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