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Observational Study
. 2025 May 27;104(10):e213557.
doi: 10.1212/WNL.0000000000213557. Epub 2025 May 5.

Clinical Features and Factors Associated With Outcome in Late Adult-Onset Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease

Affiliations
Observational Study

Clinical Features and Factors Associated With Outcome in Late Adult-Onset Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease

Alessandro Dinoto et al. Neurology. .

Abstract

Background and objectives: Data regarding late adult-onset myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) are scant. This study sought to assess the frequency, characteristics, and outcome of late adult-onset MOGAD and identify differences from early adult-onset MOGAD.

Methods: This retrospective, observational study included Mayo Clinic patients with MOGAD per 2023 diagnostic criteria and with onset age 50 years or older. Clinical, laboratory, radiologic, treatment, and outcome data were collected and compared between patients aged 50-59 years and those aged 60 years or older. Finally, the characteristics and outcome of patients with late adult-onset MOGAD (aged 50 or older) were compared with a reference group of patients with early adult-onset (aged 18-49) MOGAD (n = 141).

Results: There were 107 patients with late adult-onset MOGAD included, representing 25% of the MOGAD cohort (n = 436). The median age at onset was 59 years (range: 50-88), and 71 (66%) were female. Medical comorbidities were noted in 86 of 105 (83%). Optic neuritis was the most frequent onset attack (77/107, 72%), and the median Expanded Disability Status Scale (EDSS) score at nadir was 3 (range: 1-9). In 32 of 107 (30%), a potential trigger was noted, mostly infections. In 32 patients (30%), an alternative diagnosis was considered before MOGAD identification, most commonly giant cell arteritis (n = 15 [11 undergoing temporal artery biopsy]). By a median follow-up duration of 22 months (range: 0-306), 50 patients (47%) had a relapsing course, and preventive treatment was administered in 53 (50%). Medication side effects were common (41/107, 38%). No differences were observed in patients with MOGAD aged 50-59 years vs 60 years or older. Lastly, late adult-onset patients had more optic nerve involvement (p = 0.015; OR: 1.9; 95% CI 1.1-3.3), brainstem/cerebellar involvement (p = 0.008; OR: 3.4; 95% CI 1.1-3.3), and cognitive decline (p = 0.01; OR: 5.3; 95% CI 1.4-19.4) and less frequent myelitis (p < 0.001; OR: 0.4; 95% CI 0.2-0.7) vs those with early adult-onset MOGAD, but EDSS scores and frequency of a relapsing course were similar.

Discussion: Late adult-onset MOGAD accounts for one-quarter of patients with MOGAD. Optic neuritis is the dominant phenotype in this age group with MOGAD and is under-recognized and frequently misdiagnosed. Outcomes in late adult-onset MOGAD are similar to those in early adult-onset disease.

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Conflict of interest statement

A. Dinoto received research grants from the Autoimmune Encephalitis Alliance and Encephalitis International, and travelling grants from Horizon. L. Cacciaguerra received speaker and consultant honoraria from ACCMED, Roche, BMS Celgene, and Sanofi; and travel support for conferences from Merck-Serono. N. Vorasoot and V. Redenbaugh report no disclosures. S.A. Lopez-Chiriboga has served on advisory boards for Genentech and Horizon Therapeutics. C. Valencia-Sanchez, K. Guo, S. Thakolwboon, S.E. Horsman, S.B. Syc-Mazurek, N. Tisavipat, J. Mandrekar, D. Tajfirouz, E.R. Eggenberger, M.L. Pless, and K. Chodnicki report no disclosures. J.-M. Tillema is an associate editor for the Journal of Child Neurology. S.J. Pittock has received personal compensation for serving on scientific advisory boards or data safety monitoring boards for F. Hoffmann-La Roche AG (who manufacture satralizumab, an approved targeted therapy for NMOSD); his institution has received grants, personal fees, nonfinancial support, research support, and compensation for serving as a consultant for Alexion, AstraZeneca Rare Disease (who hold the patent rights to ravulizumab and eculizumab); he holds Patent # 9,891,219B2, Application # 12-573,942, Methods for Treating Neuromyelitis Optica (NMO) by Administration of Eculizumab to an individual That Is Aquaporin-4 (AQP4)-IgG Autoantibody Positive, which has been issued and for which he has received royalties; and he also reports grants, personal fees, nonfinancial support, and other support from Horizon Therapeutics (previously MedImmune, who manufacture inebilizumab, an approved anti-CD19 antibody used in NMOSD). J.J.-W. Chen is a consultant to UCB and Horizon. E.P. Flanagan has served on advisory boards for Alexion, Genentech, Horizon Therapeutics, and UCB; has received research support from UCB; has received speaker honoraria from Pharmacy Times; and received royalties from UpToDate. E.P. Flanagan was a site primary investigator in a randomized clinical trial on inebilizumab in neuromyelitis optica spectrum disorder run by Medimmune/Viela-Bio/Horizon Therapeutics; has received funding from the NIH (R01NS113828); is a member of the medical advisory board of the MOG project; is an editorial board member of the Journal of the Neurological Sciences and Neuroimmunology Reports. A patent has been submitted on DACH1-IgG as a biomarker of paraneoplastic autoimmunity. Go to Neurology.org/N for full disclosures.

Comment in

  • Neurology. 104:e213681.

References

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