Late Onset Neuromyelitis Optica Spectrum Disorders (LONMOSD) from a Nationwide Portuguese Study: Anti-AQP4 Positive, Anti-MOG Positive and Seronegative Subgroups

Bibliographic Details
Main Author: Santos, E
Publication Date: 2022
Other Authors: Moura, J, Samões, R, Sousa, AP, Mendonça, T, Abreu, P, Guimarães, J, Correia, I, Durães, J, Sousa, L, Ferreira, J, de Sá, J, Sousa, F, Sequeira, M, Correia, AS, André, AL, Basílio, C, Arenga, M, Brás Marques, I, Perdigão, S, Alves, I, Santos, M, Salgado, V, Palos, A, Guerreiro, R, Isidoro, L, Boleixa, D, Carneiro, P, Neves, E, Martins Silva, A, Gonçalves, G, Sá, MJ
Format: Article
Language: eng
Source: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Download full: http://hdl.handle.net/10400.17/4413
Summary: Introduction: Several neuroimmunological disorders have distinct phenotypes according to the age of onset, as in multiple sclerosis or myasthenia gravis. It is also described that late onset NMOSD (LONMOSD) has a different phenotype. Objective: To describe the clinical/demographic characteristics of the LONMOSD and distinguish them from those with early onset (EONMOSD). Methods: From a nationwide Portuguese NMOSD study we analyzed the clinical/demographic characteristics of the LONMOSD. Results: From the 180 Portuguese patients 45 had disease onset after 50 years old, 80% were female. 23 had anti-AQP4 antibodies (51.1%), 13 anti-MOG antibodies (28.9%) and 9 were double seronegative (20.0%). The most common presenting phenotypes in LONMOSD were transverse myelitis (53.3%) and optic neuritis (26.7%), without difference from EONMOSD (p = 0.074). The mean EDSS for LONMOSD was 6.0 (SD=2.8), after a mean follow-up time of 4.58 (SD=4.47) years, which was significantly greater than the mean EDSS of EONMOSD (3.25, SD=1.80)(p = 0.022). Anti-AQP4 antibodies positive LONMOSD patients had increased disability compared to anti-MOG antibodies positive LONMOSD (p = 0.022). The survival analysis showed a reduced time to use a cane for LONMOSD, irrespective of serostatus (p<0.001). Conclusions: LONMOSD has increased disability and faster progression, despite no differences in the presenting clinical phenotype were seen in our cohort.
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spelling Late Onset Neuromyelitis Optica Spectrum Disorders (LONMOSD) from a Nationwide Portuguese Study: Anti-AQP4 Positive, Anti-MOG Positive and Seronegative SubgroupsCHLC NEUAquaporin 4HumansMaleFemaleAutoantibodiesMyelitis, Transverse*Neuromyelitis Optica* / epidemiologyPortugal / epidemiologyIntroduction: Several neuroimmunological disorders have distinct phenotypes according to the age of onset, as in multiple sclerosis or myasthenia gravis. It is also described that late onset NMOSD (LONMOSD) has a different phenotype. Objective: To describe the clinical/demographic characteristics of the LONMOSD and distinguish them from those with early onset (EONMOSD). Methods: From a nationwide Portuguese NMOSD study we analyzed the clinical/demographic characteristics of the LONMOSD. Results: From the 180 Portuguese patients 45 had disease onset after 50 years old, 80% were female. 23 had anti-AQP4 antibodies (51.1%), 13 anti-MOG antibodies (28.9%) and 9 were double seronegative (20.0%). The most common presenting phenotypes in LONMOSD were transverse myelitis (53.3%) and optic neuritis (26.7%), without difference from EONMOSD (p = 0.074). The mean EDSS for LONMOSD was 6.0 (SD=2.8), after a mean follow-up time of 4.58 (SD=4.47) years, which was significantly greater than the mean EDSS of EONMOSD (3.25, SD=1.80)(p = 0.022). Anti-AQP4 antibodies positive LONMOSD patients had increased disability compared to anti-MOG antibodies positive LONMOSD (p = 0.022). The survival analysis showed a reduced time to use a cane for LONMOSD, irrespective of serostatus (p<0.001). Conclusions: LONMOSD has increased disability and faster progression, despite no differences in the presenting clinical phenotype were seen in our cohort.ElsevierRepositório do Centro Hospitalar Universitário de Lisboa Central, EPESantos, EMoura, JSamões, RSousa, APMendonça, TAbreu, PGuimarães, JCorreia, IDurães, JSousa, LFerreira, Jde Sá, JSousa, FSequeira, MCorreia, ASAndré, ALBasílio, CArenga, MBrás Marques, IPerdigão, SAlves, ISantos, MSalgado, VPalos, AGuerreiro, RIsidoro, LBoleixa, DCarneiro, PNeves, EMartins Silva, AGonçalves, GSá, MJ2023-02-20T15:13:32Z20222022-01-01T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10400.17/4413engMult Scler Relat Disord . 2022 Jul;63:103845.10.1016/j.msard.2022.103845info:eu-repo/semantics/openAccessreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAP2023-03-10T09:46:23Zoai:repositorio.chlc.min-saude.pt:10400.17/4413Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T17:21:45.851963Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse
dc.title.none.fl_str_mv Late Onset Neuromyelitis Optica Spectrum Disorders (LONMOSD) from a Nationwide Portuguese Study: Anti-AQP4 Positive, Anti-MOG Positive and Seronegative Subgroups
title Late Onset Neuromyelitis Optica Spectrum Disorders (LONMOSD) from a Nationwide Portuguese Study: Anti-AQP4 Positive, Anti-MOG Positive and Seronegative Subgroups
spellingShingle Late Onset Neuromyelitis Optica Spectrum Disorders (LONMOSD) from a Nationwide Portuguese Study: Anti-AQP4 Positive, Anti-MOG Positive and Seronegative Subgroups
Santos, E
CHLC NEU
Aquaporin 4
Humans
Male
Female
Autoantibodies
Myelitis, Transverse*
Neuromyelitis Optica* / epidemiology
Portugal / epidemiology
title_short Late Onset Neuromyelitis Optica Spectrum Disorders (LONMOSD) from a Nationwide Portuguese Study: Anti-AQP4 Positive, Anti-MOG Positive and Seronegative Subgroups
title_full Late Onset Neuromyelitis Optica Spectrum Disorders (LONMOSD) from a Nationwide Portuguese Study: Anti-AQP4 Positive, Anti-MOG Positive and Seronegative Subgroups
title_fullStr Late Onset Neuromyelitis Optica Spectrum Disorders (LONMOSD) from a Nationwide Portuguese Study: Anti-AQP4 Positive, Anti-MOG Positive and Seronegative Subgroups
title_full_unstemmed Late Onset Neuromyelitis Optica Spectrum Disorders (LONMOSD) from a Nationwide Portuguese Study: Anti-AQP4 Positive, Anti-MOG Positive and Seronegative Subgroups
title_sort Late Onset Neuromyelitis Optica Spectrum Disorders (LONMOSD) from a Nationwide Portuguese Study: Anti-AQP4 Positive, Anti-MOG Positive and Seronegative Subgroups
author Santos, E
author_facet Santos, E
Moura, J
Samões, R
Sousa, AP
Mendonça, T
Abreu, P
Guimarães, J
Correia, I
Durães, J
Sousa, L
Ferreira, J
de Sá, J
Sousa, F
Sequeira, M
Correia, AS
André, AL
Basílio, C
Arenga, M
Brás Marques, I
Perdigão, S
Alves, I
Santos, M
Salgado, V
Palos, A
Guerreiro, R
Isidoro, L
Boleixa, D
Carneiro, P
Neves, E
Martins Silva, A
Gonçalves, G
Sá, MJ
author_role author
author2 Moura, J
Samões, R
Sousa, AP
Mendonça, T
Abreu, P
Guimarães, J
Correia, I
Durães, J
Sousa, L
Ferreira, J
de Sá, J
Sousa, F
Sequeira, M
Correia, AS
André, AL
Basílio, C
Arenga, M
Brás Marques, I
Perdigão, S
Alves, I
Santos, M
Salgado, V
Palos, A
Guerreiro, R
Isidoro, L
Boleixa, D
Carneiro, P
Neves, E
Martins Silva, A
Gonçalves, G
Sá, MJ
author2_role author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
dc.contributor.none.fl_str_mv Repositório do Centro Hospitalar Universitário de Lisboa Central, EPE
dc.contributor.author.fl_str_mv Santos, E
Moura, J
Samões, R
Sousa, AP
Mendonça, T
Abreu, P
Guimarães, J
Correia, I
Durães, J
Sousa, L
Ferreira, J
de Sá, J
Sousa, F
Sequeira, M
Correia, AS
André, AL
Basílio, C
Arenga, M
Brás Marques, I
Perdigão, S
Alves, I
Santos, M
Salgado, V
Palos, A
Guerreiro, R
Isidoro, L
Boleixa, D
Carneiro, P
Neves, E
Martins Silva, A
Gonçalves, G
Sá, MJ
dc.subject.por.fl_str_mv CHLC NEU
Aquaporin 4
Humans
Male
Female
Autoantibodies
Myelitis, Transverse*
Neuromyelitis Optica* / epidemiology
Portugal / epidemiology
topic CHLC NEU
Aquaporin 4
Humans
Male
Female
Autoantibodies
Myelitis, Transverse*
Neuromyelitis Optica* / epidemiology
Portugal / epidemiology
description Introduction: Several neuroimmunological disorders have distinct phenotypes according to the age of onset, as in multiple sclerosis or myasthenia gravis. It is also described that late onset NMOSD (LONMOSD) has a different phenotype. Objective: To describe the clinical/demographic characteristics of the LONMOSD and distinguish them from those with early onset (EONMOSD). Methods: From a nationwide Portuguese NMOSD study we analyzed the clinical/demographic characteristics of the LONMOSD. Results: From the 180 Portuguese patients 45 had disease onset after 50 years old, 80% were female. 23 had anti-AQP4 antibodies (51.1%), 13 anti-MOG antibodies (28.9%) and 9 were double seronegative (20.0%). The most common presenting phenotypes in LONMOSD were transverse myelitis (53.3%) and optic neuritis (26.7%), without difference from EONMOSD (p = 0.074). The mean EDSS for LONMOSD was 6.0 (SD=2.8), after a mean follow-up time of 4.58 (SD=4.47) years, which was significantly greater than the mean EDSS of EONMOSD (3.25, SD=1.80)(p = 0.022). Anti-AQP4 antibodies positive LONMOSD patients had increased disability compared to anti-MOG antibodies positive LONMOSD (p = 0.022). The survival analysis showed a reduced time to use a cane for LONMOSD, irrespective of serostatus (p<0.001). Conclusions: LONMOSD has increased disability and faster progression, despite no differences in the presenting clinical phenotype were seen in our cohort.
publishDate 2022
dc.date.none.fl_str_mv 2022
2022-01-01T00:00:00Z
2023-02-20T15:13:32Z
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
format article
status_str publishedVersion
dc.identifier.uri.fl_str_mv http://hdl.handle.net/10400.17/4413
url http://hdl.handle.net/10400.17/4413
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv Mult Scler Relat Disord . 2022 Jul;63:103845.
10.1016/j.msard.2022.103845
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.publisher.none.fl_str_mv Elsevier
publisher.none.fl_str_mv Elsevier
dc.source.none.fl_str_mv reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
instacron:RCAAP
instname_str Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
instacron_str RCAAP
institution RCAAP
reponame_str Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
collection Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
repository.name.fl_str_mv Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
repository.mail.fl_str_mv
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