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Observational Study
. 2025 Jan;12(1):169-179.
doi: 10.1002/acn3.52268. Epub 2024 Dec 21.

The epidemiology and clinical presentation of seropositive neuromyelitis optica spectrum disorder in a US population

Aaron M Carlson  1 , Carlos E V Sollero  2 , Andrew B Wolf  1 , Stefan Sillau  1 , Barrie L Schmitt  1 , Kelli M Money  3 , Kavita V Nair  1   4 , Amanda L Piquet  1 , Jeffrey L Bennett  5
Affiliations
Observational Study

The epidemiology and clinical presentation of seropositive neuromyelitis optica spectrum disorder in a US population

Aaron M Carlson et al. Ann Clin Transl Neurol. 2025 Jan.

Abstract

Objective: To define the epidemiology and clinical presentation of seropositive neuromyelitis optica spectrum disorder (NMOSD) in a large US health system.

Methods: We completed a retrospective observational study of adult patients in the University of Colorado Health System from 1 January 2011 to 31 December 2020, using Health Data Compass (HDC), a data warehouse that combines electronic health information with claims and public health data in Colorado. We screened HDC for patients with either (1) an abnormal aquaporin-4 IgG test or (2) any G36 ICD-10 code. We extracted key clinical elements by chart review and confirmed diagnosis by the 2015 International Panel for NMO Diagnosis criteria. Annual incidence and prevalence rates were calculated.

Results: Our population consisted of 2,475,591 individuals contributing 11,103,522.72 person-years of observation. In total, 115 seropositive NMOSD patients were identified. The average yearly incidence was 0.22 per 100,000 person-years. Age and sex-adjusted prevalence (per 100,000) was 4.33, and highest among those identifying as Asian or Pacific Islander (17.72), and Black (14.74), as separately by Hispanic ethnicity (8.02). Prevalence was higher in women (6.20:1 female:male ratio). Transverse myelitis (45%) and optic neuritis (43%) were the most common presenting clinical syndromes. In total, 6% of initial presentations were characterized by short-segment transverse myelitis without other features.

Interpretation: Seropositive NMOSD incidence is higher in our cohort than many contemporary studies. Women and those identifying as Asian or Pacific Islander, Black, and Hispanic shoulder the highest burden of disease. Clinical onset with short-segment myelitis underscores the need for serum aquaporin-4 IgG testing in acute myelitis presentations.

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Conflict of interest statement

Aaron M. Carlson reports salary support and grant funding related to this project from Horizon Therapeutics, as well as recent unrelated grant funding from the National Multiple Sclerosis Society and recent service on the Health Services Subcommittee for the American Academy of Neurology. Carlos E.V. Sollero reports no relevant conflicts of interest. Andrew B. Wolf reports unrelated research funding from the Rocky Mountain MS Center, Brotherton Foundation, Genentech, and the Mowry family, unrelated consulting for Genentech and TG Therapeutics, and unrelated honoraria from MedLink Neurology and Horizon Therapeutics. Stefan Sillau reports grant funding from Amgen for this study. Barrie Schmitt reports no relevant conflicts of interest. Kelli M. Money reports no relevant conflicts of interest. Kavita V. Nair reports grant funding from Amgen for this study, grant funding from Genentech, PhRMA Foundation, Bristol Myers Squibb, Novartis, and the National Institute of Neurological Disorders and Stroke unrelated to the grant; she reports consulting unrelated to this project for Bristol Myers Squibb, Novartis, Biogen, TG Therapeutics, and EMD Serono; and she reports honoraria unrelated to this project for Sanofi‐Genzyme and Horizon Therapeutics. Amanda L. Piquet grant funding from Amgen for this study, research grants from the University of Colorado, Rocky Mountain MS Center, and the Foundation for Sarcoidosis; consulting fees from Genentech/Roche, UCB, EMD Serono, and Alexion; and honorarium from MedLink and publication royalties from Springer as co‐editor of a medical textbook. Jeffrey L. Bennett reports grant funding from Amgen for this study, personal fees from Alexion AstraZeneca Rare Disease, Antigenomycs, BeiGene, Chugai, Clene Nanomedicine, Genentech, Genzyme, Reistone Bio, Roche, Imcyse, Mitsubishi‐Tanabe, and TG; speaker fees from Alexion; grants from Alexion and the National Institutes of Health. In addition, Dr Bennett has a patent Compositions and methods for the treatment of neuromyelitis optica.

Figures

Figure 1
Figure 1
Schematic of study design. Both AQP4‐IgG testing and G36 code analysis were undertaken to ensure NMOSD patient capture. All patients with AQP4‐IgG testing were also represented in the G36 cohort.
Figure 2
Figure 2
Yearly incidence rate of NMOSD by clinical onset (A) and by diagnosis (B).
Figure 3
Figure 3
Yearly prevalence of NMOSD diagnosis.
See this image and copyright information in PMC

References

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