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Fatigability in spinal muscular atrophy: validity and reliability of endurance shuttle tests
BACKGROUND: To determine construct validity and test-retest reliability of Endurance Shuttle Tests as outcome measures for fatigability of remaining motor functions in children and adults with Spinal Muscular Atrophy (SMA) across the severity spectrum. RESULTS: We assessed the Endurance Shuttle - Ni...
Autores principales: | , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7092552/ https://www.ncbi.nlm.nih.gov/pubmed/32293503 http://dx.doi.org/10.1186/s13023-020-1348-2 |
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author | Bartels, Bart de Groot, Janke F. Habets, Laura E. Wijngaarde, Camiel A. Vink, Wendy Stam, Marloes Asselman, Fay-Lynn van Eijk, Ruben P. A. van der Pol, W. Ludo |
author_facet | Bartels, Bart de Groot, Janke F. Habets, Laura E. Wijngaarde, Camiel A. Vink, Wendy Stam, Marloes Asselman, Fay-Lynn van Eijk, Ruben P. A. van der Pol, W. Ludo |
author_sort | Bartels, Bart |
collection | PubMed |
description | BACKGROUND: To determine construct validity and test-retest reliability of Endurance Shuttle Tests as outcome measures for fatigability of remaining motor functions in children and adults with Spinal Muscular Atrophy (SMA) across the severity spectrum. RESULTS: We assessed the Endurance Shuttle - Nine Hole Peg Test (ESNHPT), − Box and Block Test (ESBBT) and – Walk Test (ESWT) in 61 patients with SMA types 2–4, 25 healthy controls (HC) and 15 disease controls (DC). Convergent validity, discriminative validity and test-retest reliability were investigated. Additionally, we compiled the Endurance Shuttle Combined Score (ESTCS) by selecting the most relevant endurance test of each individual. 54, 70 and 73% of patients with SMA demonstrated increased fatigability on the ESNHPT, ESBBT and the ESWT. Endurance response in SMA was characterized by a decrease in muscle strength, an increase in muscle fatigue and an increase in motor adaptions, thereby confirming convergent validity. Patients with SMA showed increased drop-out rates and a shorter endurance time compared to HC and DC demonstrating good discriminative validity. Test-retest reliability was moderate to excellent (ICC’s ranging from .78 to .91) with a trend towards better performance on retest. The ESTCS increased sample size and drop-out rate up to 100 and 85%. CONCLUSIONS: Fatigability is an important additional dimension of physical impairments across the severity spectrum in children and adults with SMA. The EST’s are reliable and valid to document fatigability of walking, proximal- and distal arm function in SMA and thus are promising outcome measures for use in clinical trials. |
format | Online Article Text |
id | pubmed-7092552 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-70925522020-03-27 Fatigability in spinal muscular atrophy: validity and reliability of endurance shuttle tests Bartels, Bart de Groot, Janke F. Habets, Laura E. Wijngaarde, Camiel A. Vink, Wendy Stam, Marloes Asselman, Fay-Lynn van Eijk, Ruben P. A. van der Pol, W. Ludo Orphanet J Rare Dis Research BACKGROUND: To determine construct validity and test-retest reliability of Endurance Shuttle Tests as outcome measures for fatigability of remaining motor functions in children and adults with Spinal Muscular Atrophy (SMA) across the severity spectrum. RESULTS: We assessed the Endurance Shuttle - Nine Hole Peg Test (ESNHPT), − Box and Block Test (ESBBT) and – Walk Test (ESWT) in 61 patients with SMA types 2–4, 25 healthy controls (HC) and 15 disease controls (DC). Convergent validity, discriminative validity and test-retest reliability were investigated. Additionally, we compiled the Endurance Shuttle Combined Score (ESTCS) by selecting the most relevant endurance test of each individual. 54, 70 and 73% of patients with SMA demonstrated increased fatigability on the ESNHPT, ESBBT and the ESWT. Endurance response in SMA was characterized by a decrease in muscle strength, an increase in muscle fatigue and an increase in motor adaptions, thereby confirming convergent validity. Patients with SMA showed increased drop-out rates and a shorter endurance time compared to HC and DC demonstrating good discriminative validity. Test-retest reliability was moderate to excellent (ICC’s ranging from .78 to .91) with a trend towards better performance on retest. The ESTCS increased sample size and drop-out rate up to 100 and 85%. CONCLUSIONS: Fatigability is an important additional dimension of physical impairments across the severity spectrum in children and adults with SMA. The EST’s are reliable and valid to document fatigability of walking, proximal- and distal arm function in SMA and thus are promising outcome measures for use in clinical trials. BioMed Central 2020-03-23 /pmc/articles/PMC7092552/ /pubmed/32293503 http://dx.doi.org/10.1186/s13023-020-1348-2 Text en © The Author(s). 2020 Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data. |
spellingShingle | Research Bartels, Bart de Groot, Janke F. Habets, Laura E. Wijngaarde, Camiel A. Vink, Wendy Stam, Marloes Asselman, Fay-Lynn van Eijk, Ruben P. A. van der Pol, W. Ludo Fatigability in spinal muscular atrophy: validity and reliability of endurance shuttle tests |
title | Fatigability in spinal muscular atrophy: validity and reliability of endurance shuttle tests |
title_full | Fatigability in spinal muscular atrophy: validity and reliability of endurance shuttle tests |
title_fullStr | Fatigability in spinal muscular atrophy: validity and reliability of endurance shuttle tests |
title_full_unstemmed | Fatigability in spinal muscular atrophy: validity and reliability of endurance shuttle tests |
title_short | Fatigability in spinal muscular atrophy: validity and reliability of endurance shuttle tests |
title_sort | fatigability in spinal muscular atrophy: validity and reliability of endurance shuttle tests |
topic | Research |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7092552/ https://www.ncbi.nlm.nih.gov/pubmed/32293503 http://dx.doi.org/10.1186/s13023-020-1348-2 |
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