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视神经脊髓炎谱系疾病(MOGAD)和视神经脊髓炎(NMOSD):来自阿联酋单一中心的患者影像学和实验室检查结果洞察

MOGAD and NMOSD: insights on patients' radiological and laboratory findings from a single UAE center.

作者信息

Alzarooni Hamdan, Inshasi Jihad, Alawadhi Ahmad, Giacomini Paul

机构信息

Neurology Department, Rashid Hospital, Dubai, United Arab Emirates.

Department of Neurology and Neurosurgery, Mcgill University, Montreal, QC, Canada.

出版信息

Front Neurol. 2024 Dec 9;15:1480723. doi: 10.3389/fneur.2024.1480723. eCollection 2024.

DOI:10.3389/fneur.2024.1480723
PMID:39717684
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11665212/
Abstract

INTRODUCTION

Although neuromyelitis optica spectrum disorders (NMOSD) and myelin oligodendrocyte glycoprotein antibody disease (MOGAD) are rare diseases, they pose a significant burden on both society and the healthcare system. This study aims to discuss the demographics and patient characteristics of these diseases in a single center in the United Arab Emirates (UAE).

METHODS

This is a retrospective, descriptive study that included patients with either NMOSD or MOGAD treated at Rashid Hospital, UAE during the period between January 2019 and January 2024. Patients were selected and categorized according to NMOSD criteria, aquaporin-4 antibodies, and MOG antibodies. Patient demographics, clinical characteristics, and medical history were retrieved from their medical records and descriptively analyzed in the light of patients' serological data.

RESULTS

We identified 34 patients with non-multiple sclerosis atypical CNS inflammatory/demyelinating syndromes. Twenty-seven patients (79.4%) fulfilled the criteria for NMOSD, while seven (20.6%) tested positive for MOG antibodies, fulfilling the criteria for MOGAD. In the NMOSD cohort, 19% ( = 5) were AQP4-antibody negative. Seventy-four percent of the NMOSD cohort and 43% of the MOGAD cohort were female. For MOGAD patients, disease onset was at a younger age (median onset age of 25 years) compared to the overall study population (mean onset age of 28.94 years). Long segment transverse myelitis was only detected in NMOSD patients (33.3%), and brainstem syndrome with area postrema syndrome was more common in the MOGAD cohort (29% vs. 4%). The rate of positive response to intravenous methylprednisolone as initial therapy was comparable across both cohorts (74% in case of NMOSD and 71% in case of MOGAD).

CONCLUSION

This study provides valuable insights into the status of NMOSD and MOGAD in the UAE, highlighting the need for larger, prospective studies to further characterize these diseases in the local population, as well as the need for improved understanding of the epidemiology and management of these rare but debilitating conditions.

摘要

引言

尽管视神经脊髓炎谱系障碍(NMOSD)和髓鞘少突胶质细胞糖蛋白抗体病(MOGAD)是罕见病,但它们给社会和医疗系统都带来了沉重负担。本研究旨在探讨阿拉伯联合酋长国(阿联酋)一个中心这些疾病的人口统计学特征和患者特点。

方法

这是一项回顾性描述性研究,纳入了2019年1月至2024年1月期间在阿联酋拉希德医院接受治疗的NMOSD或MOGAD患者。根据NMOSD标准、水通道蛋白4抗体和MOG抗体对患者进行选择和分类。从患者病历中获取患者人口统计学特征、临床特点和病史,并根据患者血清学数据进行描述性分析。

结果

我们确定了34例非多发性硬化的非典型中枢神经系统炎性/脱髓鞘综合征患者。27例(79.4%)符合NMOSD标准,7例(20.6%)MOG抗体检测呈阳性,符合MOGAD标准。在NMOSD队列中,19%(n = 5)为水通道蛋白4抗体阴性。NMOSD队列中7

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1cd9/11665212/a53c8394a0c9/fneur-15-1480723-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1cd9/11665212/e940b1fc7a7c/fneur-15-1480723-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1cd9/11665212/ab6fe6a65f75/fneur-15-1480723-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1cd9/11665212/a53c8394a0c9/fneur-15-1480723-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1cd9/11665212/e940b1fc7a7c/fneur-15-1480723-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1cd9/11665212/ab6fe6a65f75/fneur-15-1480723-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1cd9/11665212/a53c8394a0c9/fneur-15-1480723-g003.jpg

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本文引用的文献

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Epidemiology of myelin oligodendrocyte glycoprotein antibody-associated disease: a review of prevalence and incidence worldwide.髓鞘少突胶质细胞糖蛋白抗体相关疾病的流行病学:全球患病率和发病率综述
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皮下注射奥法木单抗治疗水通道蛋白4和髓鞘少突胶质细胞糖蛋白免疫球蛋白G双阳性视神经脊髓炎谱系疾病的病例报告
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