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视神经脊髓炎谱系疾病患者的临床和人口统计学特征:斯洛伐克队列 63 例患者的纵向回顾性分析。

Clinical and demographic characteristics of patients with NMOSD: a longitudinal retrospective analysis of a Slovak cohort of 63 patients.

机构信息

Department of Neurology, Pavol Jozef Šafárik University, Košice, Slovak Republic.

2nd Department of Internal Medicine, Pavol Jozef Šafárik University, L. Pasteur University Hospital, Trieda SNP 1, Košice, 04011, Slovak Republic.

出版信息

Neurol Sci. 2024 Feb;45(2):693-698. doi: 10.1007/s10072-023-07050-x. Epub 2023 Sep 12.

Abstract

BACKGROUND

Neuromyelitis optica spectrum disorders (NMOSD) are autoantibody-mediated inflammatory diseases of the central nervous system predominantly targeting optic nerves and the spinal cord. Two distinct phenotypes are recognized based on the presence of serum aquaporin-4 (AQP4-IgG) antibodies. However, contrasting clinical course patterns have been identified between AQP4-IgG-positive and AQP4-IgG-negative patients.

AIMS

This study aimed to present demographic and clinical characteristics of patients with NMOSD in Slovakia and to evaluate the significance of differences between AQP4-IgG-seropositive and AQP4-IgG-seronegative patients.

METHODS

We performed a longitudinal multi-centric retrospective study and analysed the clinical and demographic characteristics of a cohort of 63 Slovak NMOSD patients.

RESULTS

Eighty-six percent of patients were women, and ninety-four patients were Caucasian. The median age at diagnosis was 37 years. The most frequent initial manifestations were optic neuritis (47.6% of patients) and transverse myelitis (39.7% of patients). The median EDSS score deteriorated from the initial 3.0 to 4.0 at the last follow-up. Sixty-eight percent of patients were AQP4-IgG positive; 10% of patients were MOG-IgG positive; 27% of patients had no NMOSD-specific antibodies detected. There was a higher prevalence of autoimmune thyroiditis among AQP4-IgG-positive patients (25.6%) compared to AQP4-IgG-negative patients (0%) (p = 0.01).

CONCLUSION

This study provides a detailed overview of the clinical and demographic characteristics of NMOSD based on a retrospective analysis of a Slovak cohort of 63 NMOSD patients and extends information provided by similar recently published studies. The most important finding is that there is a high prevalence of autoimmune thyroiditis among AQP4-IgG-negative patients (25%).

摘要

背景

视神经脊髓炎谱系疾病(NMOSD)是一种以视神经和脊髓为主要靶器官的中枢神经系统自身免疫性炎症性疾病。根据血清水通道蛋白-4(AQP4-IgG)抗体的存在,识别出两种不同的表型。然而,AQP4-IgG 阳性和 AQP4-IgG 阴性患者的临床病程模式存在差异。

目的

本研究旨在介绍斯洛伐克 NMOSD 患者的人口统计学和临床特征,并评估 AQP4-IgG 阳性和 AQP4-IgG 阴性患者之间差异的意义。

方法

我们进行了一项纵向多中心回顾性研究,分析了 63 例斯洛伐克 NMOSD 患者的临床和人口统计学特征。

结果

86%的患者为女性,94 例为白种人。诊断时的中位年龄为 37 岁。最常见的首发表现为视神经炎(47.6%的患者)和横贯性脊髓炎(39.7%的患者)。中位 EDSS 评分从初始的 3.0 恶化到最后一次随访时的 4.0。68%的患者为 AQP4-IgG 阳性;10%的患者为 MOG-IgG 阳性;27%的患者未检测到 NMOSD 特异性抗体。AQP4-IgG 阳性患者自身免疫性甲状腺炎的患病率(25.6%)高于 AQP4-IgG 阴性患者(0%)(p=0.01)。

结论

本研究通过对 63 例斯洛伐克 NMOSD 患者的回顾性分析,提供了 NMOSD 临床和人口统计学特征的详细概述,并扩展了最近发表的类似研究提供的信息。最重要的发现是,AQP4-IgG 阴性患者自身免疫性甲状腺炎的患病率较高(25%)。

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