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

نویسندگان

  • 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 Calles
  • Manuel Barón Rubio
  • Cristina Ramo-Tello
  • Ana Caminero
  • Elvira Munteis
  • Alfredo R Antigüedad
  • Yolanda Blanco
  • Pablo Villoslada
  • Xavier Montalban
  • Francesc Graus
  • Albert Saiz
چکیده

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.

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عنوان ژورنال:
  • Neurology(R) neuroimmunology & neuroinflammation

دوره 3 3  شماره 

صفحات  -

تاریخ انتشار 2016