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

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

Author information

1
From the Center of Neuroimmunology (M.S., G.D.-G., Y.B., N.S.-V., E.H.M.-L., T.A., C.M., I.P.-V., E.M.-H., H.A., D.E., S.L., J.D., F.G., A.S.), Service of Neurology, Laboratory of Advanced Imaging in Neuroimmunological Diseases, Hospital Clínic of Barcelona, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), and Universitat de Barcelona, Barcelona, Spain; Division of Neurology (G.D.-G.), National Institute of Neurology and Neurosurgery, Mexico City; Pediatric Neuroimmunology Unit (T.A.), Neurology Service, Sant Joan de Deu Children's Hospital, University of Barcelona, Spain; Servicio de Inmunología (R.R.-G.), Centro de Diagnóstico Biomédico, Hospital Clinic of Barcelona, Spain; Department of Neurology (J.D.), University of Pennsylvania, Philadelphia, PA; and Catalan Institution for research and Advanced Studies (ICREA) (J.D.), Barcelona, Spain.
2
From the Center of Neuroimmunology (M.S., G.D.-G., Y.B., N.S.-V., E.H.M.-L., T.A., C.M., I.P.-V., E.M.-H., H.A., D.E., S.L., J.D., F.G., A.S.), Service of Neurology, Laboratory of Advanced Imaging in Neuroimmunological Diseases, Hospital Clínic of Barcelona, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), and Universitat de Barcelona, Barcelona, Spain; Division of Neurology (G.D.-G.), National Institute of Neurology and Neurosurgery, Mexico City; Pediatric Neuroimmunology Unit (T.A.), Neurology Service, Sant Joan de Deu Children's Hospital, University of Barcelona, Spain; Servicio de Inmunología (R.R.-G.), Centro de Diagnóstico Biomédico, Hospital Clinic of Barcelona, Spain; Department of Neurology (J.D.), University of Pennsylvania, Philadelphia, PA; and Catalan Institution for research and Advanced Studies (ICREA) (J.D.), Barcelona, Spain. asaiz@clinic.cat.

Abstract

OBJECTIVE:

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.

METHODS:

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.

RESULTS:

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.

CONCLUSIONS:

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.

PMID:
31471461
DOI:
10.1212/NXI.0000000000000607
Free PMC Article

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