Neuromyelitis optica spectrum disorders: Comparison according to the phenotype and serostatus. Issue 3 (June 2016)

• Record Type: Journal Article
• Authors: Sepúlveda, Maria; 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; Fernández-Fournier, Mireya; Ramió-Torrentà, Lluis; Cobo-Calvo, Alvaro; Viñals, Montserrat; de Andrés, Clara; Meca-Lallana, Virginia; Cervelló, Angeles; Calles, Carmen; Rubio, Manuel Barón; Ramo-Tello, Cristina; Caminero, Ana; Munteis, Elvira; Antigüedad, Alfredo R.; Blanco, Yolanda; Villoslada, Pablo; Montalban, Xavier; Graus, Francesc; Saiz, Albert; Borrego, Laura; Villaverde, Francisco José J.; Sempere, Ángel P.; Brieva, Luis; Bonet, Macarena; González-Platas, Montserrat; Puma, Delon La; Llufriu, Sara; Martínez, Eugenia H.; Boyero, Sabas; Benito-León, Julián; López, Juan C.; Robles, René; Foronda, Jesús; Navarró, Laura; Millán, Tomás; Hervás, Miguel; Tallón, Antonio; Puertas, Inmaculada; Huertas, Nuria; Redondo, Laura; Santos, Sonia; Palmí, Itziar; Salgado, Mónica; Martínez, José E.; Rubio, Miguel A.; Oterino, Agustín; Ara, Ramón; Villaverde, Ramón; Ayuso, Teresa; Erro, Elena; Marco, Miquel; Villar, Luisa; Álvarez-Cermeño, José C.; Agüera, Eduardo; Bescansa, Enrique; Yugüeros, Isabel; Tola, Miguel A.; Orviz-García, Aida; del Haro, Tomás; Bestué, María; Gómez, Monserrat; Querol, Luis; Rodríguez, Elena; Ballabriga, Jordi; Tintoré, Mar; Castilló, Joaquin; Rio, Jordi; Muñoz, Delicias; Midaglia, Luciana; Carreón-Guarnizo, Ester; Casado, José L.; Uclés, Antonio; García-Montero, Rosa;
• Is Part Of: Neurology. Volume 3:Issue 3(2016)
• Publication Date: 2016-06
• Subjects: Neuroimmunology -- Periodicals; Neurology -- Periodicals; 616.8
• DOI: 10.1212/NXI.0000000000000225
• Languages: English
• ISSNs: 2332-7812

Abstract

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 presented more 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.