Neuromyelitis optica: Clinical course and potential prognostic indicators.

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.

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Citation

Published Version (Please cite this version)

10.1016/j.msard.2022.104414

Publication Info

Masha, Nidhila, Dorlan J Kimbrough, Christopher P Eckstein, Nicholas M Hudak, Mark B Skeen, F Lee Hartsell, Michael W Lutz, Suma Shah, et al. (2023). Neuromyelitis optica: Clinical course and potential prognostic indicators. Multiple sclerosis and related disorders, 69. p. 104414. 10.1016/j.msard.2022.104414 Retrieved from https://hdl.handle.net/10161/28898.

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Scholars@Duke

Eckstein

Christopher Paul Eckstein

Associate Professor of Neurology
Hudak

Nicholas Mark Hudak

Associate Professor in Family Medicine and Community Health

Nicholas Hudak is an Associate Professor in the Department of Family Medicine and Community Health in the Duke University School of Medicine. He is faculty clinical coordinator with the Duke Physician Assistant (PA) Program, practicing PA in the Department of Neurology, and an Assistant Director in the Duke Center for Interprofessional Education and Care. 

Skeen

Mark Brian Skeen

Professor of Neurology
Hartsell

Fletcher Lee Hartsell

Assistant Professor of Neurology

Emerging MS Therapeutics (Immunomodulators as well as symptomatic therapies), Role of Vitamin D in MS Immunopathogenesis, Underlying mechanisms of MS symptoms.

Lutz

Michael William Lutz

Professor in Neurology

Developing and using computational biology methods to understand the genetic basis of disease with a focus on Alzheimer’s Disease.   Recent work has focused on identification and validation of clinically-relevant biomarkers for Alzheimer’s disease and Alzheimer’s disease with Lewy bodies.

Shah

Suma Shah

Associate Professor of Neurology

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