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Title: | Neuromyelitis optica spectrum disorders. Comparison according to the phenotype and serostatus |
Author: | Sepúlveda, María Armangué, Thaís Sola Valls, Nuria Arrambide, Georgina Meca Lallana, José E. Oreja-Guevara, Celia Mendibe, Mar Alvarez de Arcaya, Amaya Aladro, Yolanda Casanova, Bonaventura Olascoaga, Javier Jiménez Huete, Adolfo Fernandez Fournier, Mireya Ramió Torrentà, Lluís Cobo Calvo, Álvaro Viñals, Montserrat Andrés, Clara de Meca Lallana, Virginia Cervelló, Angeles Calles, Carmen Barón Rubio, Manuel Ramo Tello, Cristina Caminero, Ana Munteis, Elvira Antigüedad, Alfredo R. Blanco, Yolanda Villoslada, Pablo Montalbán Gairín, Xavier Graus Ribas, Francesc Saiz Hinarejos, Albert Spanish NMO Study Group |
Keywords: | Malalties del nervi òptic Malalties del sistema nerviós central Optic nerve diseases Central nervous system diseases |
Issue Date: | Jun-2016 |
Publisher: | Lippincott Williams & Wilkins |
Abstract: | Objective: To (1) determine the value of the recently proposed criteria of neuromyelitis optica (NMO) spectrum disorder (NMOSD) that unify patients with NMO and those with limited forms (NMO/LF) with aquaporin-4 immunoglobulin G (AQP4-IgG) antibodies; and (2) investigate the clinical significance of the serologic status in patients with NMO. Methods: This was a retrospective, multicenter study of 181 patients fulfilling the 2006 NMO criteria (n = 127) or NMO/LF criteria with AQP4-IgG (n = 54). AQP4-IgG and myelin oligodendrocyte glycoprotein immunoglobulin G (MOG-IgG) antibodies were tested using cell-based assays. Results: Patients were mainly white (86%) and female (ratio 6.5:1) with median age at onset 39 years (range 10-77). Compared to patients with NMO and AQP4-IgG (n = 94), those with NMO/LF presentedmore often with longitudinally extensive transverse myelitis (LETM) (p<0.001), and had lower relapse rates (p = 0.015), but similar disability outcomes. Nonwhite ethnicity and optic neuritis presentation doubled the risk for developing NMO compared with white race (p = 0.008) or LETM presentation (p = 0.008). Nonwhite race (hazard ratio [HR] 4.3, 95% confidence interval [CI] 1.4-13.6) and older age at onset were associated with worse outcome (for every 10-year increase, HR 1.7, 95% CI 1.3-2.2). Patients with NMO and MOG-IgG (n = 9) had lower female: male ratio (0.8:1) and better disability outcome than AQP4-IgG-seropositive or double-seronegative patients (p<0.001). Conclusions: In patients with AQP4-IgG, the similar outcomes regardless of the clinical phenotype support the unified term NMOSD; nonwhite ethnicity and older age at onset are associated with worse outcome. Double-seronegative and AQP4-IgG-seropositive NMO have a similar clinical outcome. The better prognosis of patients with MOG-IgG and NMO suggests that phenotypic and serologic classification is useful. |
Note: | Reproducció del document publicat a: https://doi.org/10.1212/NXI.0000000000000225 |
It is part of: | Neurology-Neuroimmunology & Neuroinflammation, 2016, vol. 3, num. 3 |
URI: | http://hdl.handle.net/2445/126844 |
Related resource: | https://doi.org/10.1212/NXI.0000000000000225 |
Appears in Collections: | Articles publicats en revistes (IDIBAPS: Institut d'investigacions Biomèdiques August Pi i Sunyer) Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL)) |
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