Neuromyelitis optica spectrum disorders: A nationwide Portuguese clinical epidemiological study

Detalhes bibliográficos
Autor(a) principal: Santos, E
Data de Publicação: 2021
Outros Autores: Rocha, AL, Oliveira, V, Ferro, D, Samões, R, Sousa, AP, Figueiroa, S, Mendonça, T, Abreu, P, Guimarães, J, Sousa, R, Melo, C, Correia, I, Durães, J, Sousa, L, Ferreira, J, de Sá, J, Sousa, F, Sequeira, M, Correia, AS, André, AL, Basílio, C, Arenga, M, Mendes, I, Marques, IB, Perdigão, S, Felgueiras, H, Alves, I, Correia, F, Barroso, C, Morganho, A, Carmona, C, Palavra, F, Santos, M, Salgado, V, Palos, A, Nzwalo, H, Timóteo, A, Guerreiro, R, Isidoro, L, Boleixa, D, Carneiro, P, Neves, E, Silva, AM, Gonçalves, G, Leite, MI, Sá, MJ
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Texto Completo: http://hdl.handle.net/10400.26/37588
Resumo: Introduction: Neuromyelitis optica spectrum disorder (NMOSD) is a rare disorder in which astrocyte damage and/or demyelination often cause severe neurological deficits. Objective: To identify Portuguese patients with NMOSD and assess their epidemiological/clinical characteristics. Methods: This was a nationwide multicenter study. Twenty-four Portuguese adult and 3 neuropediatric centers following NMOSD patients were included. Results: A total of 180 patients met the 2015 Wingerchuk NMOSD criteria, 77 were AQP4-antibody positive (Abs+), 67 MOG-Abs+, and 36 seronegative. Point prevalence on December 31, 2018 was 1.71/100,000 for NMOSD, 0.71/100,000 for AQP4-Abs+, 0.65/100,000 for MOG-Abs+, and 0.35/100,000 for seronegative NMOSD. A total of 44 new NMOSD cases were identified during the two-year study period (11 AQP4-Abs+, 27 MOG-Abs+, and 6 seronegative). The annual incidence rate in that period was 0.21/100,000 person-years for NMOSD, 0.05/100,000 for AQP4-Abs+, 0.13/100,000 for MOG-Abs+, and 0.03/100,000 for seronegative NMOSD. AQP4-Abs+ predominated in females and was associated with autoimmune disorders. Frequently presented with myelitis. Area postrema syndrome was exclusive of this subtype, and associated with higher morbidity/mortality than other forms of NMOSD. MOG-Ab+ more often presented with optic neuritis, required less immunosuppression, and had better outcome. Conclusion: Epidemiological/clinical NMOSD profiles in the Portuguese population are similar to other European countries
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spelling Neuromyelitis optica spectrum disorders: A nationwide Portuguese clinical epidemiological studyNeuromielite Óptica/diagnósticoNeuromielite Óptica/epidemiologyNeuromyelitis Optica /diagnosisNeuromyelitis Optica/epidemiologyIntroduction: Neuromyelitis optica spectrum disorder (NMOSD) is a rare disorder in which astrocyte damage and/or demyelination often cause severe neurological deficits. Objective: To identify Portuguese patients with NMOSD and assess their epidemiological/clinical characteristics. Methods: This was a nationwide multicenter study. Twenty-four Portuguese adult and 3 neuropediatric centers following NMOSD patients were included. Results: A total of 180 patients met the 2015 Wingerchuk NMOSD criteria, 77 were AQP4-antibody positive (Abs+), 67 MOG-Abs+, and 36 seronegative. Point prevalence on December 31, 2018 was 1.71/100,000 for NMOSD, 0.71/100,000 for AQP4-Abs+, 0.65/100,000 for MOG-Abs+, and 0.35/100,000 for seronegative NMOSD. A total of 44 new NMOSD cases were identified during the two-year study period (11 AQP4-Abs+, 27 MOG-Abs+, and 6 seronegative). The annual incidence rate in that period was 0.21/100,000 person-years for NMOSD, 0.05/100,000 for AQP4-Abs+, 0.13/100,000 for MOG-Abs+, and 0.03/100,000 for seronegative NMOSD. AQP4-Abs+ predominated in females and was associated with autoimmune disorders. Frequently presented with myelitis. Area postrema syndrome was exclusive of this subtype, and associated with higher morbidity/mortality than other forms of NMOSD. MOG-Ab+ more often presented with optic neuritis, required less immunosuppression, and had better outcome. Conclusion: Epidemiological/clinical NMOSD profiles in the Portuguese population are similar to other European countriesRepositório ComumSantos, ERocha, ALOliveira, VFerro, DSamões, RSousa, APFigueiroa, SMendonça, TAbreu, PGuimarães, JSousa, RMelo, CCorreia, IDurães, JSousa, LFerreira, Jde Sá, JSousa, FSequeira, MCorreia, ASAndré, ALBasílio, CArenga, MMendes, IMarques, IBPerdigão, SFelgueiras, HAlves, ICorreia, FBarroso, CMorganho, ACarmona, CPalavra, FSantos, MSalgado, VPalos, ANzwalo, HTimóteo, AGuerreiro, RIsidoro, LBoleixa, DCarneiro, PNeves, ESilva, AMGonçalves, GLeite, MISá, MJ2021-09-27T20:54:31Z20212021-01-01T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10400.26/37588engMult Scler Relat Disord. 2021;56: 10325810.1016/j.msard.2021.103258info: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:RCAAP2023-07-25T04:46:29Zoai:comum.rcaap.pt:10400.26/37588Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T20:09:33.736689Repositório 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 Neuromyelitis optica spectrum disorders: A nationwide Portuguese clinical epidemiological study
title Neuromyelitis optica spectrum disorders: A nationwide Portuguese clinical epidemiological study
spellingShingle Neuromyelitis optica spectrum disorders: A nationwide Portuguese clinical epidemiological study
Santos, E
Neuromielite Óptica/diagnóstico
Neuromielite Óptica/epidemiology
Neuromyelitis Optica /diagnosis
Neuromyelitis Optica/epidemiology
title_short Neuromyelitis optica spectrum disorders: A nationwide Portuguese clinical epidemiological study
title_full Neuromyelitis optica spectrum disorders: A nationwide Portuguese clinical epidemiological study
title_fullStr Neuromyelitis optica spectrum disorders: A nationwide Portuguese clinical epidemiological study
title_full_unstemmed Neuromyelitis optica spectrum disorders: A nationwide Portuguese clinical epidemiological study
title_sort Neuromyelitis optica spectrum disorders: A nationwide Portuguese clinical epidemiological study
author Santos, E
author_facet Santos, E
Rocha, AL
Oliveira, V
Ferro, D
Samões, R
Sousa, AP
Figueiroa, S
Mendonça, T
Abreu, P
Guimarães, J
Sousa, R
Melo, C
Correia, I
Durães, J
Sousa, L
Ferreira, J
de Sá, J
Sousa, F
Sequeira, M
Correia, AS
André, AL
Basílio, C
Arenga, M
Mendes, I
Marques, IB
Perdigão, S
Felgueiras, H
Alves, I
Correia, F
Barroso, C
Morganho, A
Carmona, C
Palavra, F
Santos, M
Salgado, V
Palos, A
Nzwalo, H
Timóteo, A
Guerreiro, R
Isidoro, L
Boleixa, D
Carneiro, P
Neves, E
Silva, AM
Gonçalves, G
Leite, MI
Sá, MJ
author_role author
author2 Rocha, AL
Oliveira, V
Ferro, D
Samões, R
Sousa, AP
Figueiroa, S
Mendonça, T
Abreu, P
Guimarães, J
Sousa, R
Melo, C
Correia, I
Durães, J
Sousa, L
Ferreira, J
de Sá, J
Sousa, F
Sequeira, M
Correia, AS
André, AL
Basílio, C
Arenga, M
Mendes, I
Marques, IB
Perdigão, S
Felgueiras, H
Alves, I
Correia, F
Barroso, C
Morganho, A
Carmona, C
Palavra, F
Santos, M
Salgado, V
Palos, A
Nzwalo, H
Timóteo, A
Guerreiro, R
Isidoro, L
Boleixa, D
Carneiro, P
Neves, E
Silva, AM
Gonçalves, G
Leite, MI
Sá, MJ
author2_role author
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author
author
author
author
author
author
author
author
author
author
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dc.contributor.none.fl_str_mv Repositório Comum
dc.contributor.author.fl_str_mv Santos, E
Rocha, AL
Oliveira, V
Ferro, D
Samões, R
Sousa, AP
Figueiroa, S
Mendonça, T
Abreu, P
Guimarães, J
Sousa, R
Melo, C
Correia, I
Durães, J
Sousa, L
Ferreira, J
de Sá, J
Sousa, F
Sequeira, M
Correia, AS
André, AL
Basílio, C
Arenga, M
Mendes, I
Marques, IB
Perdigão, S
Felgueiras, H
Alves, I
Correia, F
Barroso, C
Morganho, A
Carmona, C
Palavra, F
Santos, M
Salgado, V
Palos, A
Nzwalo, H
Timóteo, A
Guerreiro, R
Isidoro, L
Boleixa, D
Carneiro, P
Neves, E
Silva, AM
Gonçalves, G
Leite, MI
Sá, MJ
dc.subject.por.fl_str_mv Neuromielite Óptica/diagnóstico
Neuromielite Óptica/epidemiology
Neuromyelitis Optica /diagnosis
Neuromyelitis Optica/epidemiology
topic Neuromielite Óptica/diagnóstico
Neuromielite Óptica/epidemiology
Neuromyelitis Optica /diagnosis
Neuromyelitis Optica/epidemiology
description Introduction: Neuromyelitis optica spectrum disorder (NMOSD) is a rare disorder in which astrocyte damage and/or demyelination often cause severe neurological deficits. Objective: To identify Portuguese patients with NMOSD and assess their epidemiological/clinical characteristics. Methods: This was a nationwide multicenter study. Twenty-four Portuguese adult and 3 neuropediatric centers following NMOSD patients were included. Results: A total of 180 patients met the 2015 Wingerchuk NMOSD criteria, 77 were AQP4-antibody positive (Abs+), 67 MOG-Abs+, and 36 seronegative. Point prevalence on December 31, 2018 was 1.71/100,000 for NMOSD, 0.71/100,000 for AQP4-Abs+, 0.65/100,000 for MOG-Abs+, and 0.35/100,000 for seronegative NMOSD. A total of 44 new NMOSD cases were identified during the two-year study period (11 AQP4-Abs+, 27 MOG-Abs+, and 6 seronegative). The annual incidence rate in that period was 0.21/100,000 person-years for NMOSD, 0.05/100,000 for AQP4-Abs+, 0.13/100,000 for MOG-Abs+, and 0.03/100,000 for seronegative NMOSD. AQP4-Abs+ predominated in females and was associated with autoimmune disorders. Frequently presented with myelitis. Area postrema syndrome was exclusive of this subtype, and associated with higher morbidity/mortality than other forms of NMOSD. MOG-Ab+ more often presented with optic neuritis, required less immunosuppression, and had better outcome. Conclusion: Epidemiological/clinical NMOSD profiles in the Portuguese population are similar to other European countries
publishDate 2021
dc.date.none.fl_str_mv 2021-09-27T20:54:31Z
2021
2021-01-01T00:00:00Z
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/10400.26/37588
url http://hdl.handle.net/10400.26/37588
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv Mult Scler Relat Disord. 2021;56: 103258
10.1016/j.msard.2021.103258
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.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
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