Clinical spectrum and prognostic value of CNS MOG autoimmunity in adults: The MOGADOR study.
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Cobo-Calvo Alvaro, Ruiz Anne, Maillart Elisabeth, Audoin Bertrand, Zephir Helene, Bourre Bertrand, Ciron Jonathan, Collongues Nicolas, Brassat David, Cotton Francois, Papeix Caroline, Durand-Dubief Francoise, Laplaud David, Deschamps Romain, Cohen Mikaël, Biotti Damien, Ayrignac Xavier, Tilikete Caroline, Thouvenot Eric, Brochet Bruno, Dulau Cecile, Moreau Thibault, Tourbah Ayman, Lebranchu Pierre, Michel Laure, Lebrun-Frenay Christine, Montcuquet Alexis, Mathey Guillaume, Debouverie Marc, Pelletier Jean, Labauge Pierre, Derache Nathalie, Coustans Marc, Rollot Fabien, De Seze Jérôme, Vukusic Sandra, Marignier Romain, OFSEP and NOMADMUS Study Group
Аннотация
METHODS: Clinical data were obtained from 197 MOG-Ab-positive patients ≥18 years of age. Complete imaging data were available in 108, and 54 serum samples were eligible for longitudinal evaluation. For survival analysis comparison, 169 aquaporin-4 antibody (AQP4-Ab)-positive patients from the NOMADMUS database were included.
RESULTS: Median age at onset was 36.46 (range 18.0-76.8) years, and patients were predominantly white (92.9%) with male:female ratio, 1.1. Clinical phenotype at onset included optic neuritis or myelitis in 90.86%, isolated brainstem or encephalopathy syndromes in 6.6%, and a combination of syndromes in 2.5%. Distinctive brain MRI findings in MOG-Ab-positive patients were thalamic and pontine lesions. Cortical and leptomeningeal lesions were found in 16.3% and 6.1%, respectively. The probability of reaching a first relapse after 2 and 5 years was 44.8% and 61.8%, respectively. MOG-Ab-positive patients were at lower risk at presentation of further clinical relapse (hazard ratio [HR] 0.45, 95% confidence interval [CI] 0.26-0.79) compared to AQP4-Ab-positive individuals. MOG-Ab-positive individuals had a lower risk of reaching Disability Status Scale score of 3.0 (HR 0.46, 95% CI 0.22-0.94) and visual acuity of 20/100 (HR 0.23, 95% CI 0.07-0.72). Finally, MOG-Ab titers were higher at relapse than in remission (p = 0.009).
CONCLUSION: In adults, MOG-Ab-associated disease extends beyond clinical and radiologic abnormalities in the optic nerve and spinal cord. Despite the relapsing course, the overall visual and motor outcome is better compared with AQP4-Ab-positive patients.