来自葡萄牙全国登记处的一组神经脊髓炎视网膜频谱障碍和 MOG 相关疾病患者的残疾预后因素。

IF 3.6 3区医学 Q1 CLINICAL NEUROLOGY Journal of the Neurological Sciences Pub Date : 2024-08-12 DOI:10.1016/j.jns.2024.123176

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引用次数: 0

摘要

导言：神经性脊髓炎视网膜频谱疾病（NMOSD）和MOG相关疾病（MOGAD）是一组日益被认可的中枢神经系统脱髓鞘疾病。以往的研究表明，发病年龄较大、复发次数、首次发作的严重程度和自身抗体状态可预测预后：目的：在对全国 NMOSD/MOGAD 队列进行的 3 年随访中，研究与残疾进展和额外复发相关的预后因素：在葡萄牙初始队列的 180 名患者中，有 82 名患者的数据在随访期（2019-2022 年）结束时可用。两名患者死亡。20名患者（24.4%）在此期间发作过一次或多次疾病（共25次），大部分为横贯性脊髓炎（TM）（56.0%）或视神经炎（32.0%）。MOGAD 与单相病程（p = 0.03）和较轻的发作（p = 0.01）明显相关，而 AQP4 + NMOSD 则与复发（p = 0.03）相关。最常见的治疗方法是硫唑嘌呤（38.8%）和利妥昔单抗（18.8%）。AQP4 + NMOSD 更经常需要慢性免疫抑制治疗，尤其是利妥昔单抗（p = 0.01）。18例患者（22.5%）在随访结束时EDSS≥6。AQP4+NMOSD（p 6）患者的年龄明显偏大（64.0 ± 2.8 对 31.0 ± 17.1，p = 0.017）。一个包括血清状态和病史中 TM 发作的二元逻辑回归模型成功预测了 72.2% 的患者 EDSS ≥6 的进展：本研究强调，脊髓炎可预测 NMOSD/MOGAG 患者的残疾程度（EDSS≥6），而 AQP4 阳性与残疾程度增加有关。

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Prognostic factors associated with disability in a cohort of neuromyelitis optica spectrum disorder and MOG-associated disease from a nationwide Portuguese registry

Introduction

Neuromyelitis optica spectrum disorders (NMOSD) and MOG-associated disease (MOGAD) are an increasingly recognized group of demyelinating disorders of the central nervous system. Previous studies suggest that prognosis is predicted by older age at onset, number of relapses, the severity of the first attack and autoantibody status.

Objective

To study prognostic factors associated with disability progression and additional relapses in the 3-year follow-up of a national NMOSD/MOGAD cohort.

Results

Out of 180 of the initial Portuguese cohort, data on 82 patients was available at the end of the follow-up period (2019–2022). Two patients died. Twenty (24.4%) patients had one or more attack in this period (25 attacks in total), mostly transverse myelitis (TM) (56.0%) or optic neuritis (32.0%). MOGAD was significantly associated with a monophasic disease course (p = 0.03), with milder attacks (p = 0.01), while AQP4 + NMOSD was associated with relapses (p = 0.03). The most common treatment modalities were azathioprine (38.8%) and rituximab (18.8%). AQP4 + NMOSD more frequently required chronic immunosuppressive treatment, particularly rituximab (p = 0.01). Eighteen (22.5%) had an EDSS ≥6 at the end of the follow-up. AQP4 + NMOSD (p < 0.01) and the occurrence of transverse myelitis (TM) during disease (p = 0.04) correlated with an EDSS≥6 at the end of the follow-up period. MOGAD was significantly associated with an EDSS<6 (p < 0.01), and MOG+ cases that reached an EDSS>6 were significantly older (64.0 ± 2.8 versus 31.0 ± 17.1, p = 0.017). A bivariate logistic regression model including the serostatus and TM attacks during disease history successfully predicted 72.2% of patients that progressed to an EDSS≥6.

Conclusion

This study highlights that myelitis predict increased disability (EDSS≥6) in NMOSD/MOGAG and AQP4 positivity is associated with increased disability.

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来源期刊

Journal of the Neurological Sciences 医学-临床神经学

CiteScore

7.60

自引率

2.30%

发文量

313

审稿时长

22 days

期刊介绍： The Journal of the Neurological Sciences provides a medium for the prompt publication of original articles in neurology and neuroscience from around the world. JNS places special emphasis on articles that: 1) provide guidance to clinicians around the world (Best Practices, Global Neurology); 2) report cutting-edge science related to neurology (Basic and Translational Sciences); 3) educate readers about relevant and practical clinical outcomes in neurology (Outcomes Research); and 4) summarize or editorialize the current state of the literature (Reviews, Commentaries, and Editorials). JNS accepts most types of manuscripts for consideration including original research papers, short communications, reviews, book reviews, letters to the Editor, opinions and editorials. Topics considered will be from neurology-related fields that are of interest to practicing physicians around the world. Examples include neuromuscular diseases, demyelination, atrophies, dementia, neoplasms, infections, epilepsies, disturbances of consciousness, stroke and cerebral circulation, growth and development, plasticity and intermediary metabolism.