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, = 0.003 for AQP4-IgG, and HR, 13.0, 95% CI 2.8-59.7, = 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 0.001) in NMOSD, and higher disability after the first attack (HR 1.68, 95% CI 1.32-2.14, < 0.001), and double seronegativity (HR 3.74, 95% CI 1.03-13.6, = 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.
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http://dx.doi.org/10.1212/NXI.0000000000000607 | DOI Listing |
Mult Scler Relat Disord
February 2023
Department of Neurology and Neuroscience Center, The First Hospital of Jilin University, Changchun, China. Electronic address:
Background: Anti-aquaporin-4 (AQP-4) immunoglobulin G (IgG) is a major autoimmune antibody that contributes to the pathogenesis of neuromyelitis optica spectrum disorder (NMOSD). NMOSD often presents as disability, severe sensory impairment, and sleep disorders, which can cause anxiety and depression and further affect the quality of life. The age of onset is a key factor influencing the prognosis of NMOSD.
View Article and Find Full Text PDFFront Immunol
December 2022
Department of Geriatric Neurology, Xiangya Hospital, Central South University, Changsha, Hunan, China.
Objective: Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory disease preferentially affects the optic nerve and the spinal cord. The first attack usually occurs in the third or fourth decade, though patients with disease onset in the fifties or later are not uncommon. This study aimed to investigate the clinical characteristics and prognosis in patients with different age of onset and to explore the correlations between age of onset and clinical characteristics and prognostic outcomes.
View Article and Find Full Text PDFMult Scler Relat Disord
August 2022
CBI en Demencias y Enfermedades Desmielinizantes del Sistema Nervioso, Instituto Nacional de Ciencias Neurológicas, Lima, Peru.
Background: Neuromyelitis Optica Spectrum Disorders (NMOSD) is an autoimmune, inflammatory disorder of the Central Nervous System that typically involves the spinal cord and the optic nerves. Recently, the clinical and radiological spectrum of NMOSD has been increasing in Latin America. In Peru, there have only been a few clinical reports on NMOSD published.
View Article and Find Full Text PDFEur J Neurol
April 2022
Department of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, Shanghai, China.
Background And Purpose: This study aimed to evaluate the clinical characteristics and prognosis of late onset (≥50 years) neuromyelitis optica spectrum disorder (LO-NMOSD), and compare them with those of early onset (<50 years) NMOSD (EO-NMOSD) and NMOSD with various antibody serostatuses.
Methods: From January 2015 to December 2020, 360 anti-aquaporin 4 antibody (AQP4-ab)-positive and 130 anti-myelin oligodendrocyte glycoprotein antibody (MOG-ab)-positive patients presented to the Huashan Hospital, China. We retrospectively reviewed their medical records, including the Expanded Disability Status Scale (EDSS) score at each visit and the annualized relapse rate (ARR).
Rev Neurol (Paris)
March 2022
Multiple Sclerosis Research Center, Neuroscience Institute, Sina Hospital, Tehran University of Medical Sciences, Hasan Abad Sq, Tehran, Iran. Electronic address:
Introduction: Neuromyelitis optica spectrum disorder (NMOSD) is a disabling autoimmune disease of the central nervous system that can start at ages of 50 or more, when it is called late-onset NMOSD (LO-NMOSD). Data on this disorder are sparse. In this cross-sectional study, patient characteristics of the disease were studied.
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