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Late-onset neuromyelitis optica spectrum disorder: The importance of autoantibody serostatus.

Neurol Neuroimmunol Neuroinflamm. 2019 Aug 30;6(6). pii: e607. doi: 10.1212/NXI.0000000000000607. Print 2019 Nov.

Sepulveda M1, Delgado-García G1, Blanco Y1, Sola-Valls N1, Martinez-Lapiscina EH1, Armangué T1, Montejo C1, Pulido-Valdeolivas I1, Martinez-Hernandez E1, Ariño H1, Escudero D1, Ruiz-García R1, Llufriu S1, Dalmau J1, Graus F1, Saiz A2.

To describe the clinical features of late-onset (≥50 years) neuromyelitis optica spectrum disorder (LO-NMOSD), to compare the outcome with that of early-onset (EO-NMOSD), and to identify predictors of disability.

A retrospective, multicenter study of 238 patients with NMOSD identified by the 2015 criteria. Clinical and immunologic features of patients with LO-NMOSD were compared with those with EO-NMOSD. All patients were evaluated for aquaporin-4 (AQP4-IgG) and myelin oligodendrocyte glycoprotein (MOG-IgG) antibodies.

Sixty-nine (29%) patients had LO-NMOSD. Demographic features, initial disease presentation, annualized relapse rate, and frequency of AQP4-IgG and MOG-IgG did not differ between patients with LO-NMOSD and EO-NMOSD. Among patients with AQP4-IgG or double seronegativity, those with LO-NMOSD had a higher risk to require a cane to walk (hazard ratio [HR], 2.10, 95% CI 1.3-3.54, p = 0.003 for AQP4-IgG, and HR, 13.0, 95% CI 2.8-59.7, p = 0.001, for double seronegative). No differences in outcome were observed between patients with MOG-IgG and LO-NMOSD or EO-NMOSD. Older age at onset (for every 10-year increase, HR 1.63, 95% CI 1.35-1.92 p < 0.001) in NMOSD, and higher disability after the first attack (HR 1.68, 95% CI 1.32-2.14, p < 0.001), and double seronegativity (HR 3.74, 95% CI 1.03-13.6, p = 0.045) in LO-NMOSD were the main independent predictors of worse outcome.

Patients with LO-NMOSD have similar clinical presentation but worse outcome than EO-NMOSD when they are double seronegative or AQP4-IgG positive. Serostatus and residual disability after first attack are the main predictors of LO-NMOSD outcome.

Copyright © 2019 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.

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