Functionality and disease severity in spinocerebellar ataxias
Autor(a) principal: | |
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Data de Publicação: | 2022 |
Outros Autores: | , , , , , , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Arquivos de neuro-psiquiatria (Online) |
Texto Completo: | http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2022000200137 |
Resumo: | ABSTRACT Background: Spinocerebellar ataxias (SCAs) are a group of neurodegenerative diseases characterized by deterioration of balance and functionality that tends to follow disease progression. There is no established link between formal clinical markers for severity and functional/balance scores that could guide rehabilitation teams. Objective: To evaluate the relationship between functional scales and ataxia severity in order to identify cutoff landmarks for functional loss and estimate the mean SARA (Scale for Assessment and Rating of Ataxia) score for the risk ratings for falls on the BBS (Berg Balance Scale). Methods: Consecutive patients with a molecular diagnosis of SCA (total 89: 31 with SCA2 and 58 with SCA3) were assessed for functionality FIM-ADL (Functional Independence Measure-activities of daily living and Lawton-IADL (instrumental activities of daily living), balance (BBS) and disease severity (SARA). Results: The main disability cutoff landmarks were that the need for supervision for FIM-ADL starts with 12 points on SARA and the need for supervision for Lawton-IADL starts with 14 points on SARA. The first items to require assistance were “expression” and “shopping”, respectively. At 20 points on SARA, patients were dependent on all FIM and Lawton items. The item with the greatest impact on distinguishing dependents from independents was “means of transport” in Lawton-IADL and the domain “locomotion” in FIM-ADL. The mean SARA score for patients classified as low risk in the BBS was 9.9 points, and it was 17.4 for medium risk and 25.2 for high risk. Conclusions: Analysis on the correlation between the severity of ataxia and functional scales can form an important guide for understanding the progression of functional dependence among individuals with SCAs. |
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Functionality and disease severity in spinocerebellar ataxiasSpinocerebellar AtaxiasSeverity of Illness IndexPostural BalanceFunctional Residual CapacityABSTRACT Background: Spinocerebellar ataxias (SCAs) are a group of neurodegenerative diseases characterized by deterioration of balance and functionality that tends to follow disease progression. There is no established link between formal clinical markers for severity and functional/balance scores that could guide rehabilitation teams. Objective: To evaluate the relationship between functional scales and ataxia severity in order to identify cutoff landmarks for functional loss and estimate the mean SARA (Scale for Assessment and Rating of Ataxia) score for the risk ratings for falls on the BBS (Berg Balance Scale). Methods: Consecutive patients with a molecular diagnosis of SCA (total 89: 31 with SCA2 and 58 with SCA3) were assessed for functionality FIM-ADL (Functional Independence Measure-activities of daily living and Lawton-IADL (instrumental activities of daily living), balance (BBS) and disease severity (SARA). Results: The main disability cutoff landmarks were that the need for supervision for FIM-ADL starts with 12 points on SARA and the need for supervision for Lawton-IADL starts with 14 points on SARA. The first items to require assistance were “expression” and “shopping”, respectively. At 20 points on SARA, patients were dependent on all FIM and Lawton items. The item with the greatest impact on distinguishing dependents from independents was “means of transport” in Lawton-IADL and the domain “locomotion” in FIM-ADL. The mean SARA score for patients classified as low risk in the BBS was 9.9 points, and it was 17.4 for medium risk and 25.2 for high risk. Conclusions: Analysis on the correlation between the severity of ataxia and functional scales can form an important guide for understanding the progression of functional dependence among individuals with SCAs.Academia Brasileira de Neurologia - ABNEURO2022-02-01info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2022000200137Arquivos de Neuro-Psiquiatria v.80 n.2 2022reponame:Arquivos de neuro-psiquiatria (Online)instname:Academia Brasileira de Neurologiainstacron:ABNEURO10.1590/0004-282x-anp-2020-0580info:eu-repo/semantics/openAccessCRUZ,Geanison Castro daZONTA,Marise BuenoMUNHOZ,Renato PuppiMELLO,Neliana Maria deMEIRA,Alex TiburtinoNUNES,Maria Cristina de AlencarARANHA,Naiara Talita GuimarãesCAMARGO,Carlos Henrique FerreiraLOPES NETO,Francisco Diego NegrãoTEIVE,Hélio Afonso Ghizonieng2022-03-22T00:00:00Zoai:scielo:S0004-282X2022000200137Revistahttp://www.scielo.br/anphttps://old.scielo.br/oai/scielo-oai.php||revista.arquivos@abneuro.org1678-42270004-282Xopendoar:2022-03-22T00:00Arquivos de neuro-psiquiatria (Online) - Academia Brasileira de Neurologiafalse |
dc.title.none.fl_str_mv |
Functionality and disease severity in spinocerebellar ataxias |
title |
Functionality and disease severity in spinocerebellar ataxias |
spellingShingle |
Functionality and disease severity in spinocerebellar ataxias CRUZ,Geanison Castro da Spinocerebellar Ataxias Severity of Illness Index Postural Balance Functional Residual Capacity |
title_short |
Functionality and disease severity in spinocerebellar ataxias |
title_full |
Functionality and disease severity in spinocerebellar ataxias |
title_fullStr |
Functionality and disease severity in spinocerebellar ataxias |
title_full_unstemmed |
Functionality and disease severity in spinocerebellar ataxias |
title_sort |
Functionality and disease severity in spinocerebellar ataxias |
author |
CRUZ,Geanison Castro da |
author_facet |
CRUZ,Geanison Castro da ZONTA,Marise Bueno MUNHOZ,Renato Puppi MELLO,Neliana Maria de MEIRA,Alex Tiburtino NUNES,Maria Cristina de Alencar ARANHA,Naiara Talita Guimarães CAMARGO,Carlos Henrique Ferreira LOPES NETO,Francisco Diego Negrão TEIVE,Hélio Afonso Ghizoni |
author_role |
author |
author2 |
ZONTA,Marise Bueno MUNHOZ,Renato Puppi MELLO,Neliana Maria de MEIRA,Alex Tiburtino NUNES,Maria Cristina de Alencar ARANHA,Naiara Talita Guimarães CAMARGO,Carlos Henrique Ferreira LOPES NETO,Francisco Diego Negrão TEIVE,Hélio Afonso Ghizoni |
author2_role |
author author author author author author author author author |
dc.contributor.author.fl_str_mv |
CRUZ,Geanison Castro da ZONTA,Marise Bueno MUNHOZ,Renato Puppi MELLO,Neliana Maria de MEIRA,Alex Tiburtino NUNES,Maria Cristina de Alencar ARANHA,Naiara Talita Guimarães CAMARGO,Carlos Henrique Ferreira LOPES NETO,Francisco Diego Negrão TEIVE,Hélio Afonso Ghizoni |
dc.subject.por.fl_str_mv |
Spinocerebellar Ataxias Severity of Illness Index Postural Balance Functional Residual Capacity |
topic |
Spinocerebellar Ataxias Severity of Illness Index Postural Balance Functional Residual Capacity |
description |
ABSTRACT Background: Spinocerebellar ataxias (SCAs) are a group of neurodegenerative diseases characterized by deterioration of balance and functionality that tends to follow disease progression. There is no established link between formal clinical markers for severity and functional/balance scores that could guide rehabilitation teams. Objective: To evaluate the relationship between functional scales and ataxia severity in order to identify cutoff landmarks for functional loss and estimate the mean SARA (Scale for Assessment and Rating of Ataxia) score for the risk ratings for falls on the BBS (Berg Balance Scale). Methods: Consecutive patients with a molecular diagnosis of SCA (total 89: 31 with SCA2 and 58 with SCA3) were assessed for functionality FIM-ADL (Functional Independence Measure-activities of daily living and Lawton-IADL (instrumental activities of daily living), balance (BBS) and disease severity (SARA). Results: The main disability cutoff landmarks were that the need for supervision for FIM-ADL starts with 12 points on SARA and the need for supervision for Lawton-IADL starts with 14 points on SARA. The first items to require assistance were “expression” and “shopping”, respectively. At 20 points on SARA, patients were dependent on all FIM and Lawton items. The item with the greatest impact on distinguishing dependents from independents was “means of transport” in Lawton-IADL and the domain “locomotion” in FIM-ADL. The mean SARA score for patients classified as low risk in the BBS was 9.9 points, and it was 17.4 for medium risk and 25.2 for high risk. Conclusions: Analysis on the correlation between the severity of ataxia and functional scales can form an important guide for understanding the progression of functional dependence among individuals with SCAs. |
publishDate |
2022 |
dc.date.none.fl_str_mv |
2022-02-01 |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2022000200137 |
url |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2022000200137 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
10.1590/0004-282x-anp-2020-0580 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
text/html |
dc.publisher.none.fl_str_mv |
Academia Brasileira de Neurologia - ABNEURO |
publisher.none.fl_str_mv |
Academia Brasileira de Neurologia - ABNEURO |
dc.source.none.fl_str_mv |
Arquivos de Neuro-Psiquiatria v.80 n.2 2022 reponame:Arquivos de neuro-psiquiatria (Online) instname:Academia Brasileira de Neurologia instacron:ABNEURO |
instname_str |
Academia Brasileira de Neurologia |
instacron_str |
ABNEURO |
institution |
ABNEURO |
reponame_str |
Arquivos de neuro-psiquiatria (Online) |
collection |
Arquivos de neuro-psiquiatria (Online) |
repository.name.fl_str_mv |
Arquivos de neuro-psiquiatria (Online) - Academia Brasileira de Neurologia |
repository.mail.fl_str_mv |
||revista.arquivos@abneuro.org |
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1754212790581592064 |