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Related Experiment Video

Updated: Jul 16, 2025

Induction of Paralysis and Visual System Injury in Mice by T Cells Specific for Neuromyelitis Optica Autoantigen Aquaporin-4
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Double-negative neuromyelitis optica spectrum disorder.

Yan Wu1, Ruth Geraldes2, Maciej Juryńczyk3

  • 1Neurology Department of First Affiliated Hospital of Kunming Medical University, Kunming, China/Nuffield Department of Clinical Neurosciences, Oxford University Hospitals, Oxford, UK.

Multiple Sclerosis (Houndmills, Basingstoke, England)
|September 23, 2023
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Summary

Double-negative Neuromyelitis Optica Spectrum Disorders (DN NMOSD) present a diagnostic challenge, as this syndrome has heterogeneous causes and clinical presentations, differing from antibody-positive NMOSD.

Keywords:
AQP4-IgGMOG-IgGdouble seronegativemultiple sclerosisneuromyelitis optica spectrum disorder

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Area of Science:

  • Neuroimmunology
  • Neurology

Background:

  • Neuromyelitis Optica Spectrum Disorders (NMOSD) are typically associated with antibodies against aquaporin-4 (AQP4-IgG) or myelin oligodendrocyte glycoprotein (MOG-IgG).
  • A subset of patients, termed double-negative (DN) NMOSD, lack these specific antibodies, posing diagnostic and therapeutic challenges.
  • DN NMOSD represents a heterogeneous syndrome, not a single disease, with variable clinical courses and potential overlap with other neurological conditions.

Purpose of the Study:

  • To reappraise the definition of antibody-negative NMOSD in light of evolving diagnostic assays.
  • To delineate the clinical characteristics and pathophysiology of DN NMOSD, comparing it with antibody-positive NMOSD.
  • To summarize laboratory and MRI findings and discuss current treatment strategies for DN NMOSD.

Main Methods:

  • Topical review of existing literature.
  • Analysis of clinical, laboratory, and imaging data.
  • Comparison of DN NMOSD with antibody-positive NMOSD and other mimics.

Main Results:

  • The definition of DN NMOSD has evolved with advancements in AQP4-IgG and MOG-IgG testing.
  • DN NMOSD exhibits diverse clinical presentations, ranging from monophasic to chronic forms.
  • Pathophysiological mechanisms underlying DN NMOSD are likely heterogeneous.

Conclusions:

  • DN NMOSD requires careful diagnostic consideration due to its heterogeneity.
  • Further research is needed to elucidate the specific mechanisms and optimize treatments for DN NMOSD.
  • Distinguishing DN NMOSD from antibody-positive NMOSD and mimics is crucial for appropriate management.