Neuromyelitis optica spectrum disorders: Comparison according to the phenotype and serostatus

Maria Sepúlveda, Thaís Armangué, Nuria Sola-Valls, Georgina Arrambide, José E. Meca-Lallana, Celia Oreja-Guevara, Mar Mendibe, Amaya Alvarez De Arcaya, Yolanda Aladro, Bonaventura Casanova, Javier Olascoaga, Adolfo Jiménez-Huete, Mireya Fernández-Fournier, Lluis Ramió-Torrentà, Alvaro Cobo-Calvo, Montserrat Viñals, Clara De Andrés, Virginia Meca-Lallana, Angeles Cervelló, Carmen CallesManuel Barón Rubio, Cristina Ramo-Tello, Ana Caminero, Elvira Munteis, Alfredo R. Antigüedad, Yolanda Blanco, Pablo Villoslada, Xavier Montalban, Francesc Graus, Albert Saiz

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© 2016 American Academy of Neurology. 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 transversemyelitis (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 5 =.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 5 9) had lower female: male ratio (0.8:1) and better disability outcome than AQP4-IgG-seropositive or doubleseronegative 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.
Original languageEnglish
Article numbere225
JournalNeurology: Neuroimmunology and NeuroInflammation
Issue number3
Publication statusPublished - 1 Jan 2016


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