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    This study presents the clinical and epidemiologic characteristics of optic neuritis in Japan.

    Study design

    The authors evaluated the epidemiology of optic neuritis in Japan in the era of aquaporin-4 (AQP4) and myelin oligodendrocyte glycoprotein (MOG) antibody (Ab) biomarkers. The retrospective study included 531 cases of noninfectious optic neuritis from 33 institutions in Japan between April 2015 and January 2018.


    Sixty-six cases were AQP4-Ab positive, 54 were MOG-Ab positive, 1 was positive for both AQP4-Ab and MOG-Ab, and 410 were double negative. Fifteen patients in the double-negative group were diagnosed with multiple sclerosis. The AQP4-Ab positive group had a high proportion of females (84%) and 22% of the patients had visual acuity of counting fingers or worse. The MOG-Ab positive group experienced optic disc edema (76%) and pain with eye movements (77%) but had better recovery than the AQP4-Ab positive group; only 5% had count fingers or worse.

    A multivariate analysis revealed that older age and AQP4-Ab were associated with worse visual outcomes, while MOG-Ab was associated with higher chance of recovery. Concurrent abnormalities on MRI, especially spinal cord lesions, were highest in the AQP4-Ab positive group and lowest in the MOG-Ab positive group.


    Although the sample size was large and the cohort was drawn from multiple centers, this study was not a true population-based study. There may have been a bias toward more severe optic neuritis, thus increasing the percentage of AQP4-Ab and MOG-Ab cases. The retrospective study design also resulted in variable follow-up duration and treatments.

    Clinical significance

    This study highlights the racial differences in optic neuritis. In this Japanese cohort, AQP4-Ab positive and MOG-Ab positive optic neuritis were more common than multiple sclerosis-associated optic neuritis. This is in stark contrast with Caucasian populations, where multiple sclerosis is the most common etiology. This study confirms that AQP4-Ab positive optic neuritis has the worst visual outcome, while MOG-Ab positive optic neuritis has better recovery and a favorable visual outcome.