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Títol:	Neuromyelitis optica spectrum disorders. Comparison according to the phenotype and serostatus
Autor/a:	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 Hinajeros, Albert; Spanish NMO Study Group
Notes:	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.
Matèries:	-Malalties del nervi òptic -Malalties del sistema nerviós central -Optic nerve diseases -Central nervous system diseases
Drets:	cc by-nc-nd (c) American Academy of Neurology, 2016 http://creativecommons.org/licenses/by-nc-nd/3.0/es/
Tipus de document:	Article Article - Versió publicada
Publicat per:	Lippincott Williams & Wilkins
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