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Treatment outcomes of first-ever episode of severe optic neuritis

  • Author Footnotes
    ⁎ Co-first authors: these authors contributed equally
    Kristin Galetta
    Correspondence
    Corresponding author at: Brigham Multiple Sclerosis Center, 60 Fenwood Road, Boston, MA 02115 USA
    Footnotes
    ⁎ Co-first authors: these authors contributed equally
    Affiliations
    Department of Neurology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA

    Icahn School of Medicine at Mount Sinai, New York, NY

    Department of Neurology, Mayo Clinic Alix School of Medicine, Rochester MN
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  • Author Footnotes
    ⁎ Co-first authors: these authors contributed equally
    Sophia Ryan
    Footnotes
    ⁎ Co-first authors: these authors contributed equally
    Affiliations
    Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA

    Department of Neurology, Mount Sinai Health System, New York, NY

    Icahn School of Medicine at Mount Sinai, New York, NY

    Department of Neurology, Mayo Clinic Alix School of Medicine, Rochester MN
    Search for articles by this author
  • Giovanna Manzano
    Affiliations
    Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA

    Icahn School of Medicine at Mount Sinai, New York, NY

    Department of Neurology, Mayo Clinic Alix School of Medicine, Rochester MN
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  • Lori B. Chibnik
    Affiliations
    Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA

    Department of Epidemiology, Harvard T.H. Chan School of Public Health, Boston MA

    Icahn School of Medicine at Mount Sinai, New York, NY

    Department of Neurology, Mayo Clinic Alix School of Medicine, Rochester MN
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  • Denis Balaban
    Affiliations
    Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA

    Icahn School of Medicine at Mount Sinai, New York, NY

    Department of Neurology, Mayo Clinic Alix School of Medicine, Rochester MN
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  • Sashank Prasad
    Affiliations
    Department of Neurology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA

    Icahn School of Medicine at Mount Sinai, New York, NY

    Department of Neurology, Mayo Clinic Alix School of Medicine, Rochester MN
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  • Bart K. Chwalisz
    Affiliations
    Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA

    Department of Ophthalmology, Massachusetts Eye and Ear, Harvard Medical School, Boston, MA

    Icahn School of Medicine at Mount Sinai, New York, NY

    Department of Neurology, Mayo Clinic Alix School of Medicine, Rochester MN
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  • Andrea Salazar-Camelo
    Affiliations
    Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD

    Icahn School of Medicine at Mount Sinai, New York, NY

    Department of Neurology, Mayo Clinic Alix School of Medicine, Rochester MN
    Search for articles by this author
  • Sarah Conway
    Affiliations
    Department of Neurology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA

    Icahn School of Medicine at Mount Sinai, New York, NY

    Department of Neurology, Mayo Clinic Alix School of Medicine, Rochester MN
    Search for articles by this author
  • Michael Levy
    Affiliations
    Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA

    Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD

    Icahn School of Medicine at Mount Sinai, New York, NY

    Department of Neurology, Mayo Clinic Alix School of Medicine, Rochester MN
    Search for articles by this author
  • Marcelo Matiello
    Affiliations
    Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA

    Icahn School of Medicine at Mount Sinai, New York, NY

    Department of Neurology, Mayo Clinic Alix School of Medicine, Rochester MN
    Search for articles by this author
  • Author Footnotes
    ⁎ Co-first authors: these authors contributed equally

      Highlights

      • Severe optic neuritis is seen in disorders including neuromyelitis optica spectrum disorder
      • Acute treatment for patients with severe optic neuritis of unclear etiology is not clear
      • Patients with severe optic neuritis of unknown cause may benefit from treatment escalation to PLEX

      Abstract

      Background

      Severe optic neuritis (ON) is an acute inflammatory attack of the optic nerve(s) leading to severe visual loss that may occur in isolation or as part of a relapsing neuroinflammatory disease, such neuromyelitis optica spectrum disorder (NMOSD), myelin oligodendrocyte glycoprotein antibody associated disease (MOGAD), or more rarely multiple sclerosis (MS). In cases of first-ever severe ON of uncertain etiology best treatment strategies remain unclear.

      Methods

      We reviewed records of all patients with a documented diagnosis of ON between 2004 and 2019 at Mass General Brigham (MGB) and Johns Hopkins University (JHU) hospitals. Out of 381 patients identified, 90 (23.6%) satisfied the study criteria for severe ON with visual acuity (VA) equal to or worse than 20/200 (logMAR=1) at nadir in the affected eye and had sufficient follow-up data. Treatment strategies with corticosteroids only or treatment escalation with therapeutic plasma exchange (PLEX) after steroids were compared and evaluated for differences in visual outcomes at follow-up.

      Results

      Of the 90 patients with severe optic neuritis, 71(78.9%) received corticosteroids only, and 19 (17.0%) underwent PLEX following corticosteroids. Of the 71 patients who received steroids without escalation to PLEX, 30 patients (42.2%) achieved complete recovery (VA 20/20 on the affected eye), whereas 35 (49.3%) had a partial recovery and 6 (8.4%) had no recovery. Among the 19 corticosteroid non-responders patients who underwent escalation treatment, 13 (68.4%) made complete recovery, 6 (31.6%) had partial visual recoveries (p=0.0434). The median delta logMAR of patients who underwent escalation of care was -1.2 compared with 2.0 for the ones who did not (p=0.0208). A change of delta logmar 2.0 is equivalent of going from hand motion to light perception and the positive delta value refers to intra-attack worsening. Other than not responding to steroids, patients who underwent PLEX tended to have more severe ON with significantly worse nadir visual acuity compared with those who received corticosteroids alone (logMAR 3.12 (min 2.0 – max 5.0) vs. 2.17 (min 1.3 – max 3.0); p=0.004).

      Conclusion

      In our cohort of first-ever severe optic neuritis of unknown etiology, patients that did not respond adequately to corticosteroids benefited from treatment escalation to PLEX, followed in most cases by Rituximab, regardless of final etiology. Randomized controlled trials are needed to confirm the best treatment strategies.

      Keywords

      Abbreviations:

      IQR (Interquartile range), logMAR (Logarithm of the minimum angle of resolution), max (maximum), min (minimum), MOGAD (Myelin oligodendrocyte antibody associated disease), NMOSD (Neuromyelitis optics spectrum disorder), ON (Optic Neuritis), PLEX (Plasma exchange)
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