Non-ataxia signs in Brazilian individuals with spinocerebellar ataxia type 3

Detalhes bibliográficos
Autor(a) principal: Martins, Camilla Polonini
Data de Publicação: 2021
Outros Autores: Galvez, Mariana da Silva Salvino Klem, de Araujo, Yuri Rodrigues Luz, Keim, Leda Maria Neumann, Figueiredo, Fernanda Baseggio Lopes, de Oliveira, Laura Alice Santos
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Fisioterapia em Movimento
Texto Completo: https://periodicos.pucpr.br/fisio/article/view/28667
Resumo: Introduction: Spinocerebellar ataxia 3 (SCA3) is a hereditary disease associated with progressive cerebellar and extracerebellar degeneration. Although there is no effective therapy for SCA3, some of its symptoms can be relieved with symptomatic treatment. Identifying the presence of this signs in patients may contribute to their clinical management and thus improve their quality of life. Objective: To identify the presence and frequency of non-ataxia signs in a sample of Brazilian individuals with SCA3 and to investigate its association with severity and duration of the disease. Methods: This is a cross-sectional study. The inclusion criteria of this study were participants should be diagnosed with SCA3 and be between 18–70 years old. The exclusion criteria were participants with a score of less than 18 points on the Mini Mental State Examination (MMSE) and those with other neurological or orthopedic problems. Twenty-three participants were evaluated by the Inventory of Non-ataxia Signs (INAS) and the Scale for the Assessment and Rating of Ataxia (SARA). Results: The median score found for INAS was [median (min-max)] 3 (0-6) points and 10 (2-23) for SARA. Only one participant did not show non-ataxic signs. The most prevalent non-ataxic manifestations were areflexia, urinary dysfunction, hyperreflexia and spasticity. Spearman correlation test indicated a moderate and significant correlation between INAS and SARA scores (rho = 0.428, 95% CI = 0.39-0.704, p = 0.033). There was no association between INAS scores and the disease duration (rho = 0.003, 95% CI = -0.398-0.704, p = 0.393). Conclusion: Areflexia, urinary dysfunction, hyperreflexia and spasticity were the most prevalent non-ataxic signs. We identified a moderate correlation between the presence of non-ataxic signs and disease severity. This findings can help the professionals dealing with these patients.
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spelling Non-ataxia signs in Brazilian individuals with spinocerebellar ataxia type 3Ataxia. Spinocerebellar Ataxia Type 3. Machado-Joseph disease. Signs and symptoms.Introduction: Spinocerebellar ataxia 3 (SCA3) is a hereditary disease associated with progressive cerebellar and extracerebellar degeneration. Although there is no effective therapy for SCA3, some of its symptoms can be relieved with symptomatic treatment. Identifying the presence of this signs in patients may contribute to their clinical management and thus improve their quality of life. Objective: To identify the presence and frequency of non-ataxia signs in a sample of Brazilian individuals with SCA3 and to investigate its association with severity and duration of the disease. Methods: This is a cross-sectional study. The inclusion criteria of this study were participants should be diagnosed with SCA3 and be between 18–70 years old. The exclusion criteria were participants with a score of less than 18 points on the Mini Mental State Examination (MMSE) and those with other neurological or orthopedic problems. Twenty-three participants were evaluated by the Inventory of Non-ataxia Signs (INAS) and the Scale for the Assessment and Rating of Ataxia (SARA). Results: The median score found for INAS was [median (min-max)] 3 (0-6) points and 10 (2-23) for SARA. Only one participant did not show non-ataxic signs. The most prevalent non-ataxic manifestations were areflexia, urinary dysfunction, hyperreflexia and spasticity. Spearman correlation test indicated a moderate and significant correlation between INAS and SARA scores (rho = 0.428, 95% CI = 0.39-0.704, p = 0.033). There was no association between INAS scores and the disease duration (rho = 0.003, 95% CI = -0.398-0.704, p = 0.393). Conclusion: Areflexia, urinary dysfunction, hyperreflexia and spasticity were the most prevalent non-ataxic signs. We identified a moderate correlation between the presence of non-ataxic signs and disease severity. This findings can help the professionals dealing with these patients.Editora PUCPRESS2021-01-26info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfhttps://periodicos.pucpr.br/fisio/article/view/2866710.1590/fm.2021.34123Fisioterapia em Movimento (Physical Therapy in Movement); Vol. 34 (2021): n. cont.Fisioterapia em Movimento; v. 34 (2021): n. cont.1980-5918reponame:Fisioterapia em Movimentoinstname:Pontifícia Universidade Católica do Paraná (PUC-PR)instacron:PUC_PRenghttps://periodicos.pucpr.br/fisio/article/view/28667/pdfCopyright (c) 2022 PUCPRESSinfo:eu-repo/semantics/openAccessMartins, Camilla PoloniniGalvez, Mariana da Silva Salvino Klemde Araujo, Yuri Rodrigues LuzKeim, Leda Maria NeumannFigueiredo, Fernanda Baseggio Lopesde Oliveira, Laura Alice Santos2022-03-07T19:03:03Zoai:ojs.periodicos.pucpr.br:article/28667Revistahttps://periodicos.pucpr.br/fisioPRIhttps://periodicos.pucpr.br/fisio/oairubia.farias@pucpr.br||revista.fisioterapia@pucpr.br1980-59180103-5150opendoar:2022-03-07T19:03:03Fisioterapia em Movimento - Pontifícia Universidade Católica do Paraná (PUC-PR)false
dc.title.none.fl_str_mv Non-ataxia signs in Brazilian individuals with spinocerebellar ataxia type 3
title Non-ataxia signs in Brazilian individuals with spinocerebellar ataxia type 3
spellingShingle Non-ataxia signs in Brazilian individuals with spinocerebellar ataxia type 3
Martins, Camilla Polonini
Ataxia. Spinocerebellar Ataxia Type 3. Machado-Joseph disease. Signs and symptoms.
title_short Non-ataxia signs in Brazilian individuals with spinocerebellar ataxia type 3
title_full Non-ataxia signs in Brazilian individuals with spinocerebellar ataxia type 3
title_fullStr Non-ataxia signs in Brazilian individuals with spinocerebellar ataxia type 3
title_full_unstemmed Non-ataxia signs in Brazilian individuals with spinocerebellar ataxia type 3
title_sort Non-ataxia signs in Brazilian individuals with spinocerebellar ataxia type 3
author Martins, Camilla Polonini
author_facet Martins, Camilla Polonini
Galvez, Mariana da Silva Salvino Klem
de Araujo, Yuri Rodrigues Luz
Keim, Leda Maria Neumann
Figueiredo, Fernanda Baseggio Lopes
de Oliveira, Laura Alice Santos
author_role author
author2 Galvez, Mariana da Silva Salvino Klem
de Araujo, Yuri Rodrigues Luz
Keim, Leda Maria Neumann
Figueiredo, Fernanda Baseggio Lopes
de Oliveira, Laura Alice Santos
author2_role author
author
author
author
author
dc.contributor.author.fl_str_mv Martins, Camilla Polonini
Galvez, Mariana da Silva Salvino Klem
de Araujo, Yuri Rodrigues Luz
Keim, Leda Maria Neumann
Figueiredo, Fernanda Baseggio Lopes
de Oliveira, Laura Alice Santos
dc.subject.por.fl_str_mv Ataxia. Spinocerebellar Ataxia Type 3. Machado-Joseph disease. Signs and symptoms.
topic Ataxia. Spinocerebellar Ataxia Type 3. Machado-Joseph disease. Signs and symptoms.
description Introduction: Spinocerebellar ataxia 3 (SCA3) is a hereditary disease associated with progressive cerebellar and extracerebellar degeneration. Although there is no effective therapy for SCA3, some of its symptoms can be relieved with symptomatic treatment. Identifying the presence of this signs in patients may contribute to their clinical management and thus improve their quality of life. Objective: To identify the presence and frequency of non-ataxia signs in a sample of Brazilian individuals with SCA3 and to investigate its association with severity and duration of the disease. Methods: This is a cross-sectional study. The inclusion criteria of this study were participants should be diagnosed with SCA3 and be between 18–70 years old. The exclusion criteria were participants with a score of less than 18 points on the Mini Mental State Examination (MMSE) and those with other neurological or orthopedic problems. Twenty-three participants were evaluated by the Inventory of Non-ataxia Signs (INAS) and the Scale for the Assessment and Rating of Ataxia (SARA). Results: The median score found for INAS was [median (min-max)] 3 (0-6) points and 10 (2-23) for SARA. Only one participant did not show non-ataxic signs. The most prevalent non-ataxic manifestations were areflexia, urinary dysfunction, hyperreflexia and spasticity. Spearman correlation test indicated a moderate and significant correlation between INAS and SARA scores (rho = 0.428, 95% CI = 0.39-0.704, p = 0.033). There was no association between INAS scores and the disease duration (rho = 0.003, 95% CI = -0.398-0.704, p = 0.393). Conclusion: Areflexia, urinary dysfunction, hyperreflexia and spasticity were the most prevalent non-ataxic signs. We identified a moderate correlation between the presence of non-ataxic signs and disease severity. This findings can help the professionals dealing with these patients.
publishDate 2021
dc.date.none.fl_str_mv 2021-01-26
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.uri.fl_str_mv https://periodicos.pucpr.br/fisio/article/view/28667
10.1590/fm.2021.34123
url https://periodicos.pucpr.br/fisio/article/view/28667
identifier_str_mv 10.1590/fm.2021.34123
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv https://periodicos.pucpr.br/fisio/article/view/28667/pdf
dc.rights.driver.fl_str_mv Copyright (c) 2022 PUCPRESS
info:eu-repo/semantics/openAccess
rights_invalid_str_mv Copyright (c) 2022 PUCPRESS
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.publisher.none.fl_str_mv Editora PUCPRESS
publisher.none.fl_str_mv Editora PUCPRESS
dc.source.none.fl_str_mv Fisioterapia em Movimento (Physical Therapy in Movement); Vol. 34 (2021): n. cont.
Fisioterapia em Movimento; v. 34 (2021): n. cont.
1980-5918
reponame:Fisioterapia em Movimento
instname:Pontifícia Universidade Católica do Paraná (PUC-PR)
instacron:PUC_PR
instname_str Pontifícia Universidade Católica do Paraná (PUC-PR)
instacron_str PUC_PR
institution PUC_PR
reponame_str Fisioterapia em Movimento
collection Fisioterapia em Movimento
repository.name.fl_str_mv Fisioterapia em Movimento - Pontifícia Universidade Católica do Paraná (PUC-PR)
repository.mail.fl_str_mv rubia.farias@pucpr.br||revista.fisioterapia@pucpr.br
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