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

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• dc.contributor.author Sepúlveda, Mariaca
• dc.contributor.author Munteis Olivas, Elviraca
• dc.contributor.author Saiz, Albertca
• dc.date.accessioned 2016-07-25T07:20:26Z
• dc.date.available 2016-07-25T07:20:26Z
• dc.date.issued 2016
• dc.description.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.ca
• 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/pdfca
• dc.identifier.citation Sepúlveda M, Armangué T, Sola-Valls N, Arrambide G, Meca-Lallana JE, Oreja-Guevara C. et al. Neuromyelitis optica spectrum disorders: comparison according to the phenotype and serostatus. Neurol Neuroimmunol Neuroinflamm. 2016 Apr 14;3(3):e225. doi: 10.1212/NXI.0000000000000225ca
• dc.identifier.doi http://dx.doi.org/10.1212/NXI.0000000000000225
• dc.identifier.issn 2332-7812
• dc.identifier.uri http://hdl.handle.net/10230/27111
• dc.language.iso engca
• dc.publisher Wolters Kluwer (LWW)ca
• dc.relation.ispartof Neurology Neuroimmunology & Neuroinflammation. 2016 Apr 14;3(3):e225
• dc.rights This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND), which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially.ca
• dc.rights.accessRights info:eu-repo/semantics/openAccessca
• dc.rights.uri https://creativecommons.org/licenses/by-nc-nd/4.0/ca
• dc.subject.other Ulls -- Malalties i defectesca
• dc.title Neuromyelitis optica spectrum disorders: comparison according to the phenotype and serostatusca
• dc.type info:eu-repo/semantics/articleca
• dc.type.version info:eu-repo/semantics/publishedVersionca