Characterizing double seronegative neuromyelitis optica spectrum disorder: A distinct subgroup or part of the continuum?

IF 3.1 4区医学 Q3 IMMUNOLOGY

Journal of neuroimmunology Pub Date : 2026-02-01 Epub Date: 2025-12-01 DOI:10.1016/j.jneuroim.2025.578822

María Agustina Piedrabuena , Mariano Marrodan , María Agustina Zárate , Marcela Fiol , María Celica Ysrraelit , Jorge Correale

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引用次数: 0

Abstract

Background

Neuromyelitis optica spectrum disorder (NMOSD) encompasses inflammatory demyelinating CNS conditions. Patients negative for aquaporin-4 (AQP4-IgG) and myelin oligodendrocyte glycoprotein (MOG-IgG) antibodies are classified as double seronegative (DN).

Objective

To characterize and compare the clinical, radiological, and therapeutic profiles of DN, AQP4-IgG+, and MOG-IgG+ patients.

Methods

Retrospective analysis of patients meeting 2015 NMOSD criteria, grouped by serostatus (AQP4-IgG+, MOG-IgG+, and DN). Demographic, clinical, and imaging data were compared.

Results

We analyzed 111 patients: 64 AQP4-IgG+, 31 DN, and 16 MOG-IgG+. At onset, AQP4-IgG+ patients were older than DN and MOG-IgG+ (49 ± 14 vs 41 ± 12 vs 35 ± 12 years, p < 0.0001). DN had more relapses in the first two years (p = 0.02) and higher EDSS (p < 0.001). Myelitis was common in DN and AQP4-IgG+, while optic neuritis in MOG-IgG+. Relapse rates were highest in AQP4-IgG+ (p < 0.001). Oligoclonal bands were more common in DN (39 %) than in AQP4-IgG+ (27.7 %) and MOG-IgG+ (25 %)(p < 0.0001). Area postrema lesions were more frequent in DN (p = 0.02). Rituximab was mainly used in AQP4-IgG+ and DN (p < 0.001), with lower failure in DN (4.8 % vs 18 %).

Conclusion

DN NMOSD shows distinct features but comparable disability to AQP4-IgG+ individuals. Conventional immunosuppressants seem effective, though further research is needed to clarify mechanisms and optimize treatment strategies.

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表征双血清阴性神经脊髓炎视谱障碍：是一个独特的亚群还是连续体的一部分？

视神经脊髓炎谱系障碍（NMOSD）包括中枢神经系统炎症性脱髓鞘疾病。水通道蛋白-4 （AQP4-IgG）和髓鞘少突胶质细胞糖蛋白（MOG-IgG）抗体阴性的患者被归类为双血清阴性（DN）。目的探讨并比较DN、AQP4-IgG+和MOG-IgG+患者的临床、放射学和治疗特点。方法回顾性分析符合2015年NMOSD标准的患者，按血清状态（AQP4-IgG+、MOG-IgG+、DN）分组。比较人口学、临床和影像学资料。结果111例患者：AQP4-IgG+ 64例，DN 31例，MOG-IgG+ 16例。发病时，AQP4-IgG+患者比DN和MOG-IgG+患者年龄大（49±14岁vs 41±12岁vs 35±12岁，p < 0.0001）。DN患者前2年复发较多（p = 0.02）， EDSS较高（p < 0.001）。DN组和AQP4-IgG+组多见脊髓炎，MOG-IgG+组多见视神经炎。AQP4-IgG+复发率最高（p < 0.001）。寡克隆带在DN（39%）中比AQP4-IgG+(27.7%)和MOG-IgG+(25%)中更常见（p < 0.0001）。DN患者多见术后区域病变（p = 0.02）。利妥昔单抗主要用于AQP4-IgG+和DN (p < 0.001)， DN的失败率较低（4.8% vs 18%）。结论dn NMOSD与AQP4-IgG阳性个体表现出明显的特征，但致残性与AQP4-IgG阳性个体相似。传统的免疫抑制剂似乎是有效的，但需要进一步的研究来阐明机制和优化治疗策略。

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来源期刊

Journal of neuroimmunology 医学-免疫学

CiteScore

6.10

自引率

3.00%

发文量

154

审稿时长

37 days

期刊介绍： The Journal of Neuroimmunology affords a forum for the publication of works applying immunologic methodology to the furtherance of the neurological sciences. Studies on all branches of the neurosciences, particularly fundamental and applied neurobiology, neurology, neuropathology, neurochemistry, neurovirology, neuroendocrinology, neuromuscular research, neuropharmacology and psychology, which involve either immunologic methodology (e.g. immunocytochemistry) or fundamental immunology (e.g. antibody and lymphocyte assays), are considered for publication.