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

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...

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Autores: Sepúlveda, Maria, Munteis Olivas, Elvira, Saiz, Albert
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2016
País:España
Institución:Universitat Pompeu Fabra
Repositorio:Repositorio Digital de la UPF
OAI Identifier:oai:repositori.upf.edu:10230/27111
Acceso en línea:http://hdl.handle.net/10230/27111
http://dx.doi.org/10.1212/NXI.0000000000000225
Access Level:acceso abierto
Palabra clave:Ulls -- Malalties i defectes
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spelling Neuromyelitis optica spectrum disorders: comparison according to the phenotype and serostatusSepúlveda, MariaMunteis Olivas, ElviraSaiz, AlbertUlls -- Malalties i defectesOBJECTIVE: 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.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.).Wolters Kluwer (LWW)201620162016info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfapplication/pdfhttp://hdl.handle.net/10230/27111http://dx.doi.org/10.1212/NXI.0000000000000225reponame:Repositorio Digital de la UPFinstname:Universitat Pompeu FabraInglésNeurology Neuroimmunology & Neuroinflammation. 2016 Apr 14;3(3):e225This 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.https://creativecommons.org/licenses/by-nc-nd/4.0/info:eu-repo/semantics/openAccessoai:repositori.upf.edu:10230/271112026-06-12T07:21:37Z
dc.title.none.fl_str_mv Neuromyelitis optica spectrum disorders: comparison according to the phenotype and serostatus
title Neuromyelitis optica spectrum disorders: comparison according to the phenotype and serostatus
spellingShingle Neuromyelitis optica spectrum disorders: comparison according to the phenotype and serostatus
Sepúlveda, Maria
Ulls -- Malalties i defectes
title_short Neuromyelitis optica spectrum disorders: comparison according to the phenotype and serostatus
title_full Neuromyelitis optica spectrum disorders: comparison according to the phenotype and serostatus
title_fullStr Neuromyelitis optica spectrum disorders: comparison according to the phenotype and serostatus
title_full_unstemmed Neuromyelitis optica spectrum disorders: comparison according to the phenotype and serostatus
title_sort Neuromyelitis optica spectrum disorders: comparison according to the phenotype and serostatus
dc.creator.none.fl_str_mv Sepúlveda, Maria
Munteis Olivas, Elvira
Saiz, Albert
author Sepúlveda, Maria
author_facet Sepúlveda, Maria
Munteis Olivas, Elvira
Saiz, Albert
author_role author
author2 Munteis Olivas, Elvira
Saiz, Albert
author2_role author
author
dc.subject.none.fl_str_mv Ulls -- Malalties i defectes
topic Ulls -- Malalties i defectes
description 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.
publishDate 2016
dc.date.none.fl_str_mv 2016
2016
2016
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv http://hdl.handle.net/10230/27111
http://dx.doi.org/10.1212/NXI.0000000000000225
url http://hdl.handle.net/10230/27111
http://dx.doi.org/10.1212/NXI.0000000000000225
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.relation.none.fl_str_mv Neurology Neuroimmunology & Neuroinflammation. 2016 Apr 14;3(3):e225
dc.rights.none.fl_str_mv https://creativecommons.org/licenses/by-nc-nd/4.0/
info:eu-repo/semantics/openAccess
rights_invalid_str_mv https://creativecommons.org/licenses/by-nc-nd/4.0/
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
application/pdf
dc.publisher.none.fl_str_mv Wolters Kluwer (LWW)
publisher.none.fl_str_mv Wolters Kluwer (LWW)
dc.source.none.fl_str_mv reponame:Repositorio Digital de la UPF
instname:Universitat Pompeu Fabra
instname_str Universitat Pompeu Fabra
reponame_str Repositorio Digital de la UPF
collection Repositorio Digital de la UPF
repository.name.fl_str_mv
repository.mail.fl_str_mv
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