Neuromyelitis optica spectrum disorders: Comparison according to the phenotype and serostatus
dc.contributor.author
dc.date.accessioned
2017-06-22T07:22:50Z
dc.date.available
2017-06-22T07:22:50Z
dc.date.issued
2016-04-14
dc.identifier.uri
dc.description.abstract
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
dc.description.sponsorship
This study was supported in part by Red Española de Esclerosis Múltiple
(REEM) Instituto de Salud Carlos III, Spain (RD07/0060/01, P.V.;
RD12/0032/0002, A.S.; Marató de TV3 [20141830], F.G.) and Instituto
de Salud Carlos III, Spain (CM14/00081; T.A.)
dc.format.mimetype
application/pdf
dc.language.iso
eng
dc.publisher
Lippincott, Williams & Wilkins
dc.relation.isformatof
Reproducció digital del document publicat a: http://dx.doi.org/10.1212/NXI.0000000000000225
dc.relation.ispartof
Neurology, Neuroimmunology and Neuroinflammation, 2016, vol. 3, núm 3, p. e225
dc.relation.ispartofseries
Articles publicats (IdIBGi)
dc.rights
Attribution-NonCommercial-NoDerivs 3.0 Spain
dc.rights.uri
dc.subject
dc.title
Neuromyelitis optica spectrum disorders: Comparison according to the phenotype and serostatus
dc.type
info:eu-repo/semantics/article
dc.rights.accessRights
info:eu-repo/semantics/openAccess
dc.embargo.terms
Cap
dc.type.version
info:eu-repo/semantics/publishedVersion
dc.identifier.doi
dc.identifier.idgrec
027207
dc.identifier.eissn
2332-7812