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Neuromyelitis Optica: Clinical Course and Potential Prognostic Indicators

Published:November 18, 2022DOI:https://doi.org/10.1016/j.msard.2022.104414

      Highlights

      • Patients with NMOSD may regain functional ability over time
      • No association between neurologic outcomes in NMOSD and initial AQP4 serostatus
      • No association between neurologic outcomes in NMOSD and initial AQP4 titer levels
      • Need for more research on effects of timely PLEX administration on NMOSD outcomes

      Abstract

      Background

      Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune neurological disorder associated with antibodies to aquaporin-4 (AQP4). NMOSD has been thought to follow a progressive disease course, with step-wise accumulation of disability over time, even in patients undergoing immunosuppressive/immunomodulatory therapy. The influence of factors such as AQP4 seropositivity, AQP4 serum titer levels, and administration of plasmapheresis on NMOSD prognosis is, as yet, unclear.

      Methods

      We performed a retrospective chart review of 53 persons with NMOSD at Duke University Hospital—collecting data on longitudinal disease course, imaging, demographics, and serum AQP4 titers (measured using the ELISA or FACS method). Most patients in our cohort were treated with high-dose corticosteroids and, following diagnosis, received maintenance immunosuppressive/immunomodulatory therapies. Longitudinal data on EDSS scores were used to calculate the slope of disability over time for each participant. We additionally investigated the correlation between initial AQP4 seropositivity, initial AQP4 serum titer levels, and treatment with plasmapheresis on disability progression for each participant.

      Results

      Contrary to current views on NMOSD disease course, the majority of our participants showed either no change (31.9%) or improvement (27.1%) in disability over time. Our results additionally revealed no significant association between clinical prognosis and initial AQP4 seropositivity (p=0.830), initial AQP4 serum titer levels (p=0.338), or administration of plasmapheresis (p=0.1149).

      Conclusions

      Our study presents a contemporary view of the clinical course of NMOSD and shows a more favorable view of its disease course than prior studies (performed before high-efficacy disease modifying therapies became widely-used for this patient population). Most patients in this study received treatment with high-dose corticosteroids following NMOSD flares, as well as a variety of maintenance immunosuppressive therapies. The results of this study cannot shed light on the disease course of untreated NMOSD. Our findings additionally challenge the theory that AQP4 seropositivity or serum titer levels at time of diagnosis may be used to effectively predict NMOSD prognosis. While we were unable to find evidence supporting a favorable effect of plasmapheresis administration on disease outcomes, further research is needed to determine the role plasmapheresis ought to play in the treatment of NMOSD.

      Graphical abstract

      Keywords

      Abbreviations:

      Neuromyelitis optica spectrum disorder ((NMOSD)), optic neuritis ((ON)), transverse myelitis ((TM)), Extended Disability Status Scale ((EDSS)), aquaporin-4 ((AQP4))
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