Factors Associated With Disability Improvement and Worsening Independent of Attacks in Patients With AQP4-IgG+ NMOSD and MOGAD: A Multicenter Cohort Study.

Authors

Daniel Engels, Patrick Schindler, Joachim Havla, Katinka Fischer, Marius Ringelstein, Martin W Hümmert, Franziska Bütow, Insa Schiffmann, Charlotte Schubert, Judith Bellmann-Strobl, Katrin Giglhuber, Clarissa Zappe, Sven Jarius, Lisa Revie, Carolin Schwake, Ioannis Vardakas, Florian Then Bergh, Yavor Yalachkov, Annette Walter, Leila Husseini, Markus C Kowarik, Matthias Grothe, Antonios Bayas, Klemens Angstwurm, Steffen Pfeuffer, Uwe K Zettl, Ingo Kleiter, Clemens Warnke, Simone C Tauber, Jonathan Wickel, Makbule Senel, Ilya Ayzenberg, Luisa Klotz, Brigitte Wildemann, Achim Berthele, Vivien Häußler, Corinna Trebst, Orhan Aktas, Friedemann Paul, Tania Kümpfel, as the Neuromyelitis Optica Study Group (NEMOS)

Published in

Neurology. Volume 107. Issue 2. Pages e218199. Jul 28, 2026. Epub Jul 02, 2026.

Abstract

Disability trajectories in aquaporin-4 immunoglobulin G-seropositive neuromyelitis optica spectrum disorder (AQP4-IgG+ NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) are primarily driven by attack-related damage. Confirmed disability worsening (CDW) independent of attacks has been described but occurs infrequently in AQP4-IgG+ NMOSD and MOGAD. Confirmed disability improvement (CDI) has not been evaluated in large cohorts. We determined the frequency of CDI and CDW independent of attacks and identified clinical factors associated with these outcomes in AQP4-IgG+ NMOSD and MOGAD.
This retrospective, multicenter cohort study analyzed data from the German Neuromyelitis Optica Study Group (NEMOS) registry. Adult patients with AQP4-IgG+ NMOSD or MOGAD and longitudinal Expanded Disability Status Scale (EDSS) assessments were included. EDSS episodes were defined as periods with ≥3 EDSS assessments without attacks, obtained ≥90 days after attack. CDW and CDI were defined as sustained EDSS increase or decrease (≥1.5 for baseline EDSS 0; ≥1.0 for EDSS 1.0-5.5; ≥0.5 for EDSS ≥6.0) confirmed after at least 6 months. The primary outcomes were annualized CDI and CDW rates. Risk factors were assessed using multivariable Anderson-Gill regression models.
A total of 338 EDSS episodes of 307 patients (n: 202/105, median age at EDSS change: 56/41 years, 88/49% female, both p < 0.001; AQP4-IgG+ NMOSD/MOGAD) were included. Adjusted annualized CDI and CDW rates did not differ between AQP4-IgG+ NMOSD (CDI: 0.083, 95% CI 0.029-0.233; CDW: 0.025, 95% CI 0.007-0.092) and MOGAD (CDI: 0.057, 95% CI 0.012-0.277; CDW: 0.036, 95% CI 0.002-0.513). In AQP4-IgG+ NMOSD, a lower number of prior attacks was associated with higher CDI rates (hazard ratio [HR] 0.89, 95% CI 0.82-0.97). Younger age was associated with increased CDI rates in both AQP4-IgG+ NMOSD and MOGAD (HR 0.96, 95% CI 0.94-0.99, for both).
CDI and CDW independent of attacks, although rare, occur in AQP4-IgG+ NMOSD and MOGAD. The association between fewer prior attacks and higher CDI rates in AQP4-IgG+ NMOSD underscores the importance of early attack prevention. Limitations include the retrospective design, and the limited number of CDI and CDW events.

PMID:
42391599
Bibliographic data and abstract were imported from PubMed on 03 Jul 2026.

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