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

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, Lluís
Cobo Calvo, Alvaro
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
Montalban Gairín, Xavier
Graus, Francesc
Saiz, Albert
NMO Study Group
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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.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.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).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 ​
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