Non-ataxia signs in Brazilian individuals with spinocerebellar ataxia type 3
Autor(a) principal: | |
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Data de Publicação: | 2021 |
Outros Autores: | , , , , |
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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Fisioterapia em Movimento |
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|
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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 |
_version_ |
1799138749550952448 |