Emotional lability at disease onset is an independent prognostic factor of faster disease progression in Amyotrophic Lateral Sclerosis

Detalhes bibliográficos
Autor(a) principal: Kuźma-Kozakiewicz, Magdalena
Data de Publicação: 2020
Outros Autores: Uysal, Hilmi, Steinbach, Robert, Rödiger, Annekathrin, Petri, Susanne, Grosskreutz, Julian, Gromicho, Marta, Carvalho, Mamede, Nieporęcki, Krzysztof, Szacka, Katarzyna, Barć, Krzysztof
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Texto Completo: http://hdl.handle.net/10451/50319
Resumo: Copyright: © 2019 Barc K et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
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spelling Emotional lability at disease onset is an independent prognostic factor of faster disease progression in Amyotrophic Lateral SclerosisAmyotrophic lateral sclerosisDisease progressionEmotional labilityEpidemiologyPathological laughing and cryingPrognosisPseudobulbar affectCopyright: © 2019 Barc K et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.Amyotrophic lateral sclerosis (ALS) is a fast progressing neurodegenerative disease leading to quadriplegia, anarthria and respiratory insufficiency. A large variety of phenotypes and disability progression requires individually tailored management. Identification of predictors of poor prognosis may not only improve management, but also allow for more precise patients' stratification for clinical trials or research studies. The aim of the study was to investigate the influence of emotional lability present at disease onset on ALS progression by exploring its direct impact on the decay of the ALS Functional Rating Scale-Revised (ALSFRS-R). The study was performed in a group of 1145 patients from Germany, Poland, Portugal and Turkey between 2014 and 2018. The analysis showed that the presence of emotional lability at ALS onset was linked to a faster decline of ALSFRS-R (0.70 vs 0.50, p<0.0001), in case of either bulbar (0.80 vs 0.65, p<0.05) or limb disease onset (0.59 vs 0.46, p <0.01). It was most prominent in the bulbar subscore of ALSFRS-R. A multiple regression analysis showed a direct influence of emotional lability at ALS onset on disease progression, regardless of age, gender, site of onset, weight loss, cognitive impairment and diagnosis delay (β=0.071; p=0.019). It can therefore be concluded that the presence of emotional lability at the disease onset is an independent factor of faster disease progression in ALS.This study was supported by OnWebDuals project (JNPD 01ED1511B; DZP/2/JPND-III/2015). This is an EU Joint Programme - Neurodegenerative Disease Research (JPND) project. The project is supported through the following funding organization under the aegis of JPND - www.jpnd.eu: Germany, Bundes-ministerium für Bildung und Forschung (BMBF); Poland, Narodowe Centrum Badań i Rozwoju (NCBiR); Portugal, Fundação a Ciência e a Tecnologia (FCT); Sweden, Vetenskapsrådet (VR).JKL InternationalRepositório da Universidade de LisboaKuźma-Kozakiewicz, MagdalenaUysal, HilmiSteinbach, RobertRödiger, AnnekathrinPetri, SusanneGrosskreutz, JulianGromicho, MartaCarvalho, MamedeNieporęcki, KrzysztofSzacka, KatarzynaBarć, Krzysztof2021-12-09T16:25:26Z20202020-01-01T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10451/50319engAging Dis. 2020 Oct 1;11(5):1021-10282152-525010.14336/AD.2019.1120info: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:RCAAP2023-11-08T16:54:26Zoai:repositorio.ul.pt:10451/50319Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T22:01:48.491251Repositório 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 Emotional lability at disease onset is an independent prognostic factor of faster disease progression in Amyotrophic Lateral Sclerosis
title Emotional lability at disease onset is an independent prognostic factor of faster disease progression in Amyotrophic Lateral Sclerosis
spellingShingle Emotional lability at disease onset is an independent prognostic factor of faster disease progression in Amyotrophic Lateral Sclerosis
Kuźma-Kozakiewicz, Magdalena
Amyotrophic lateral sclerosis
Disease progression
Emotional lability
Epidemiology
Pathological laughing and crying
Prognosis
Pseudobulbar affect
title_short Emotional lability at disease onset is an independent prognostic factor of faster disease progression in Amyotrophic Lateral Sclerosis
title_full Emotional lability at disease onset is an independent prognostic factor of faster disease progression in Amyotrophic Lateral Sclerosis
title_fullStr Emotional lability at disease onset is an independent prognostic factor of faster disease progression in Amyotrophic Lateral Sclerosis
title_full_unstemmed Emotional lability at disease onset is an independent prognostic factor of faster disease progression in Amyotrophic Lateral Sclerosis
title_sort Emotional lability at disease onset is an independent prognostic factor of faster disease progression in Amyotrophic Lateral Sclerosis
author Kuźma-Kozakiewicz, Magdalena
author_facet Kuźma-Kozakiewicz, Magdalena
Uysal, Hilmi
Steinbach, Robert
Rödiger, Annekathrin
Petri, Susanne
Grosskreutz, Julian
Gromicho, Marta
Carvalho, Mamede
Nieporęcki, Krzysztof
Szacka, Katarzyna
Barć, Krzysztof
author_role author
author2 Uysal, Hilmi
Steinbach, Robert
Rödiger, Annekathrin
Petri, Susanne
Grosskreutz, Julian
Gromicho, Marta
Carvalho, Mamede
Nieporęcki, Krzysztof
Szacka, Katarzyna
Barć, Krzysztof
author2_role author
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 Kuźma-Kozakiewicz, Magdalena
Uysal, Hilmi
Steinbach, Robert
Rödiger, Annekathrin
Petri, Susanne
Grosskreutz, Julian
Gromicho, Marta
Carvalho, Mamede
Nieporęcki, Krzysztof
Szacka, Katarzyna
Barć, Krzysztof
dc.subject.por.fl_str_mv Amyotrophic lateral sclerosis
Disease progression
Emotional lability
Epidemiology
Pathological laughing and crying
Prognosis
Pseudobulbar affect
topic Amyotrophic lateral sclerosis
Disease progression
Emotional lability
Epidemiology
Pathological laughing and crying
Prognosis
Pseudobulbar affect
description Copyright: © 2019 Barc K et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
publishDate 2020
dc.date.none.fl_str_mv 2020
2020-01-01T00:00:00Z
2021-12-09T16:25:26Z
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/10451/50319
url http://hdl.handle.net/10451/50319
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv Aging Dis. 2020 Oct 1;11(5):1021-1028
2152-5250
10.14336/AD.2019.1120
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 JKL International
publisher.none.fl_str_mv JKL International
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
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