{
  "abstract": "Background\n\nRecent 2025 neuromyelitis optica spectrum disorder (NMOSD) criteria distinguish AQP4-IgG-positive disease from seronegative-syndromic groups, into which double-seronegative-NMOSD (DN-NMOSD) may be reclassified, suggesting the need to better characterize DN-NMOSD within prior diagnostic frameworks.Objective\n\nTo determine the proportion and clinical characteristics of DN-NMOSD using rigorously applied 2015 NMOSD criteria with cell-based assays (CBAs).Methods\n\nIn this multicenter retrospective cohort study across four Korean referral centers, 634 individuals fulfilling the 2015 criteria were identified. AQP4-IgG and MOG-IgG were assessed using CBAs, with repeated testing in initially seronegative individuals.Results\n\nDN-NMOSD accounted for 8.4% (53/634). Median onset age was 36 years, with mild female predominance (59%). At disease onset, classical NMO phenotype (simultaneous optic neuritis and myelitis) was rare (2%), and isolated brain involvement without myelitis, optic neuritis, or area postrema syndrome was uncommon (13%). Over a median 9-year follow-up, 93% showed a relapsing course, and maintenance immunosuppressive therapy was used in 91% with reduction in relapse rate after treatment (p < 0.001).Conclusions\n\nIn a large multicenter cohort, DN-NMOSD represents a small proportion of NMOSD when stringent diagnostic criteria and repeated CBA testing are applied. These findings may provide data relevant to ongoing discussions on classification of the seronegative-syndromic group within evolving diagnostic frameworks.",
  "abstract_shown": true,
  "affiliations": [
    "Department of Neurology, Research Institute and Hospital of National Cancer Center, Goyang, Republic of Korea.",
    "Department of Neurology, Severance Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea.",
    "Department of Neurology, Chonnam National University Hospital and Medical School, Gwangju, Republic of Korea.",
    "Department of Neurology, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea."
  ],
  "article_type": "brief report",
  "arxiv": null,
  "author_total": 9,
  "authors": [
    {
      "affs": [
        0
      ],
      "name": "Jae-Won Hyun",
      "orcid": "0000-0003-0288-771X"
    },
    {
      "affs": [
        1
      ],
      "name": "Ki Hoon Kim",
      "orcid": "0000-0001-6428-061X"
    },
    {
      "affs": [
        1
      ],
      "name": "Seung Woo Kim",
      "orcid": "0000-0002-5621-0811"
    },
    {
      "affs": [
        1
      ],
      "name": "Ha Young Shin",
      "orcid": "0000-0002-4408-8265"
    },
    {
      "affs": [
        2
      ],
      "name": "You-Ri Kang",
      "orcid": "0000-0001-5189-1323"
    },
    {
      "affs": [
        2
      ],
      "name": "Tai-Seung Nam",
      "orcid": "0000-0003-2771-8728"
    },
    {
      "affs": [
        3
      ],
      "name": "Woojun Kim",
      "orcid": "0000-0001-8204-8881"
    },
    {
      "affs": [
        0
      ],
      "name": "Su-Hyun Kim",
      "orcid": "0000-0002-0679-0918"
    },
    {
      "affs": [
        0
      ],
      "name": "Ho Jin Kim",
      "orcid": "0000-0002-8672-8419"
    }
  ],
  "bibtex": "https://research.draconitic.com/paper/5wgt5920cncc1.bib",
  "cited_by": 0,
  "doi": "10.1111/ene.70808",
  "fulltext_url": "https://europepmc.org/articles/PMC13728997",
  "id": "5wgt5920cncc1",
  "issns": [
    "1351-5101",
    "1468-1331"
  ],
  "landing_url": "https://europepmc.org/article/MED/42974736",
  "language": "eng",
  "licence": "CC-BY-NC-ND",
  "licence_label": "CC BY-NC-ND",
  "licence_source": "article",
  "licence_url": "https://creativecommons.org/licenses/by-nc-nd/4.0/",
  "metadata_licence": null,
  "native_subjects": [
    [
      "heading",
      "Short Communication"
    ],
    [
      "openalex",
      "2734|Multiple Sclerosis Research Studies"
    ]
  ],
  "openalex": "W7225164773",
  "pdf_url": "https://europepmc.org/articles/PMC13728997?pdf=render",
  "pmcid": "PMC13728997",
  "pmid": "42974736",
  "published": "2026-11-01",
  "publisher": "Wiley",
  "ris": "https://research.draconitic.com/paper/5wgt5920cncc1.ris",
  "sources": [
    {
      "first_seen": "2026-11-04T15:20:47Z",
      "native_id": "MED:42974736",
      "source": "europepmc",
      "url": "https://europepmc.org/article/MED/42974736"
    },
    {
      "first_seen": "2026-11-07T12:21:50Z",
      "native_id": "PMC13728997",
      "source": "pmc",
      "url": "https://doi.org/10.1111/ene.70808"
    }
  ],
  "subjects": [
    {
      "field": "medicine",
      "origin": "openalex:2734|Multiple Sclerosis Research Studies",
      "subfield": "general"
    }
  ],
  "title": "Proportion and Clinical Characteristics of Double-Seronegative NMOSD in a Multicenter Real-World Cohort.",
  "title_text": "Proportion and Clinical Characteristics of Double-Seronegative NMOSD in a Multicenter Real-World Cohort.",
  "type": "peer-reviewed",
  "updated_at": "2026-11-07T12:21:50Z",
  "url": "https://research.draconitic.com/paper/5wgt5920cncc1",
  "venue": "European journal of neurology",
  "versions": []
}