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Functionality and disease severity in spinocerebellar ataxias
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 coul...
Autores principales: | , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Academia Brasileira de Neurologia -ABNEURO
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9648916/ https://www.ncbi.nlm.nih.gov/pubmed/35352753 http://dx.doi.org/10.1590/0004-282X-ANP-2020-0580 |
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author | da CRUZ, Geanison Castro ZONTA, Marise Bueno MUNHOZ, Renato Puppi de MELLO, Neliana Maria MEIRA, Alex Tiburtino NUNES, Maria Cristina de Alencar ARANHA, Naiara Talita Guimarães CAMARGO, Carlos Henrique Ferreira LOPES, Francisco Diego Negrão TEIVE, Hélio Afonso Ghizoni |
author_facet | da CRUZ, Geanison Castro ZONTA, Marise Bueno MUNHOZ, Renato Puppi de MELLO, Neliana Maria MEIRA, Alex Tiburtino NUNES, Maria Cristina de Alencar ARANHA, Naiara Talita Guimarães CAMARGO, Carlos Henrique Ferreira LOPES, Francisco Diego Negrão TEIVE, Hélio Afonso Ghizoni |
author_sort | da CRUZ, Geanison Castro |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-9648916 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Academia Brasileira de Neurologia -ABNEURO |
record_format | MEDLINE/PubMed |
spelling | pubmed-96489162022-12-08 Functionality and disease severity in spinocerebellar ataxias da CRUZ, Geanison Castro ZONTA, Marise Bueno MUNHOZ, Renato Puppi de MELLO, Neliana Maria MEIRA, Alex Tiburtino NUNES, Maria Cristina de Alencar ARANHA, Naiara Talita Guimarães CAMARGO, Carlos Henrique Ferreira LOPES, Francisco Diego Negrão TEIVE, Hélio Afonso Ghizoni Arq Neuropsiquiatr Article 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 -ABNEURO 2022-03-14 /pmc/articles/PMC9648916/ /pubmed/35352753 http://dx.doi.org/10.1590/0004-282X-ANP-2020-0580 Text en https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License |
spellingShingle | Article da CRUZ, Geanison Castro ZONTA, Marise Bueno MUNHOZ, Renato Puppi de MELLO, Neliana Maria MEIRA, Alex Tiburtino NUNES, Maria Cristina de Alencar ARANHA, Naiara Talita Guimarães CAMARGO, Carlos Henrique Ferreira LOPES, Francisco Diego Negrão TEIVE, Hélio Afonso Ghizoni Functionality and disease severity in spinocerebellar ataxias |
title | 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_short | Functionality and disease severity in spinocerebellar ataxias |
title_sort | functionality and disease severity in spinocerebellar ataxias |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9648916/ https://www.ncbi.nlm.nih.gov/pubmed/35352753 http://dx.doi.org/10.1590/0004-282X-ANP-2020-0580 |
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