Spreading in ALS: The relative impact of upper and lower motor neuron involvement

Detalhes bibliográficos
Autor(a) principal: Gromicho, Marta
Data de Publicação: 2020
Outros Autores: Figueiral, Manuel, Uysal, Hilmi, Grosskreutz, Julian, Kuzma‐Kozakiewicz, Magdalena, Pinto, Susana, Petri, Susanne, Madeira, Sara C., Swash, Michael, Carvalho, Mamede
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Texto Completo: http://hdl.handle.net/10451/53289
Resumo: © 2020 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals LLC on behalf of American Neurological Association. This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.
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spelling Spreading in ALS: The relative impact of upper and lower motor neuron involvement© 2020 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals LLC on behalf of American Neurological Association. This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.Objective: To investigate disease spread in amyotrophic lateral sclerosis (ALS), and determine the influence of lower (LMN) and upper motor neuron (UMN) involvement. Methods: We assessed disease spread in ALS in 1376 consecutively studied patients, from five European centers, applying an agreed proforma to assess LMN and UMN signs. We defined the pattern of disease onset and progression from predominant UMN or lower motor neuron (LMN) dysfunction in bulbar, upper limbs, lower limbs, and thoracic regions Non-linear regression analysis was applied to fit the data to a model that described the relation between two random variables, graphically represented by an inverse exponential curve. We analyzed the probability, rate of spread, and both combined (area under the curve). Results: We found that progression was more likely and quicker to or from the region of onset to close spinal regions. When the disease had a limb onset, bulbar motor neurons were more resistant. Furthermore, in the same time frame more patients progressed from bulbar to lower limbs than vice-versa, whether predominantly UMN or LMN involvement. Patients with initial thoracic involvement had a higher probability for rapid change. The presence of predominant UMN signs was associated with a faster caudal progression. Interpretation: Contiguous progression was leading pattern, and predominant UMN involvement is important in shortening the time for cranial-caudal spread. Our results can best be fitted to a model of independent LMN and UMN degeneration, with regional progression of LMN degeneration mostly by contiguity. UMN lesion causes an acceleration of rostral-caudal LMN loss.This is an EU Joint Programme - Neurodegenerative Disease Research (JPND) project. The project is supported through national funding organizations under the aegis of JPND - www.jpnd.eu. This project was also partially supported by FCT funding to Neuroclinomics2 (PTDC/EEI-SII/1937/2014).WileyRepositório da Universidade de LisboaGromicho, MartaFigueiral, ManuelUysal, HilmiGrosskreutz, JulianKuzma‐Kozakiewicz, MagdalenaPinto, SusanaPetri, SusanneMadeira, Sara C.Swash, MichaelCarvalho, Mamede2022-06-06T15:01:17Z20202020-01-01T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10451/53289engAnn Clin Transl Neurol. 2020 Jul;7(7):1181-119210.1002/acn3.510982328-9503info: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:RCAAP2023-11-08T16:58:59Zoai:repositorio.ul.pt:10451/53289Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T22:04:15.866564Repositório 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 Spreading in ALS: The relative impact of upper and lower motor neuron involvement
title Spreading in ALS: The relative impact of upper and lower motor neuron involvement
spellingShingle Spreading in ALS: The relative impact of upper and lower motor neuron involvement
Gromicho, Marta
title_short Spreading in ALS: The relative impact of upper and lower motor neuron involvement
title_full Spreading in ALS: The relative impact of upper and lower motor neuron involvement
title_fullStr Spreading in ALS: The relative impact of upper and lower motor neuron involvement
title_full_unstemmed Spreading in ALS: The relative impact of upper and lower motor neuron involvement
title_sort Spreading in ALS: The relative impact of upper and lower motor neuron involvement
author Gromicho, Marta
author_facet Gromicho, Marta
Figueiral, Manuel
Uysal, Hilmi
Grosskreutz, Julian
Kuzma‐Kozakiewicz, Magdalena
Pinto, Susana
Petri, Susanne
Madeira, Sara C.
Swash, Michael
Carvalho, Mamede
author_role author
author2 Figueiral, Manuel
Uysal, Hilmi
Grosskreutz, Julian
Kuzma‐Kozakiewicz, Magdalena
Pinto, Susana
Petri, Susanne
Madeira, Sara C.
Swash, Michael
Carvalho, Mamede
author2_role author
author
author
author
author
author
author
author
author
dc.contributor.none.fl_str_mv Repositório da Universidade de Lisboa
dc.contributor.author.fl_str_mv Gromicho, Marta
Figueiral, Manuel
Uysal, Hilmi
Grosskreutz, Julian
Kuzma‐Kozakiewicz, Magdalena
Pinto, Susana
Petri, Susanne
Madeira, Sara C.
Swash, Michael
Carvalho, Mamede
description © 2020 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals LLC on behalf of American Neurological Association. This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.
publishDate 2020
dc.date.none.fl_str_mv 2020
2020-01-01T00:00:00Z
2022-06-06T15:01:17Z
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/10451/53289
url http://hdl.handle.net/10451/53289
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv Ann Clin Transl Neurol. 2020 Jul;7(7):1181-1192
10.1002/acn3.51098
2328-9503
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 Wiley
publisher.none.fl_str_mv Wiley
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
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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
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