Fatigability in spinal muscular atrophy: validity and reliability of endurance shuttle tests
Abstract 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 Shutt...
| Main Authors: | , , , , , , , , |
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| Format: | Article |
| Language: | English |
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BMC
2020-03-01
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| Series: | Orphanet Journal of Rare Diseases |
| Subjects: | |
| Online Access: | http://link.springer.com/article/10.1186/s13023-020-1348-2 |
| _version_ | 1828323154620055552 |
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| author | Bart Bartels Janke F. de Groot Laura E. Habets Camiel A. Wijngaarde Wendy Vink Marloes Stam Fay-Lynn Asselman Ruben P. A. van Eijk W. Ludo van der Pol |
| author_facet | Bart Bartels Janke F. de Groot Laura E. Habets Camiel A. Wijngaarde Wendy Vink Marloes Stam Fay-Lynn Asselman Ruben P. A. van Eijk W. Ludo van der Pol |
| author_sort | Bart Bartels |
| collection | DOAJ |
| description | Abstract 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. |
| first_indexed | 2024-04-13T18:49:32Z |
| format | Article |
| id | doaj.art-eac1a0ba8eba4fefb1c4f3a6043dbd46 |
| institution | Directory Open Access Journal |
| issn | 1750-1172 |
| language | English |
| last_indexed | 2024-04-13T18:49:32Z |
| publishDate | 2020-03-01 |
| publisher | BMC |
| record_format | Article |
| series | Orphanet Journal of Rare Diseases |
| spelling | doaj.art-eac1a0ba8eba4fefb1c4f3a6043dbd462022-12-22T02:34:30ZengBMCOrphanet Journal of Rare Diseases1750-11722020-03-011511910.1186/s13023-020-1348-2Fatigability in spinal muscular atrophy: validity and reliability of endurance shuttle testsBart Bartels0Janke F. de Groot1Laura E. Habets2Camiel A. Wijngaarde3Wendy Vink4Marloes Stam5Fay-Lynn Asselman6Ruben P. A. van Eijk7W. Ludo van der Pol8Wilhelmina Children’s Hospital, University Medical Center Utrecht, Child Development and Exercise CenterResearch Group Lifestyle and Health, HU University of Applied Health Sciences UtrechtWilhelmina Children’s Hospital, University Medical Center Utrecht, Child Development and Exercise CenterUniversity Medical Center Utrecht, UMC Utrecht Brain CenterRijndam Rehabilitation CenterUniversity Medical Center Utrecht, UMC Utrecht Brain CenterUniversity Medical Center Utrecht, UMC Utrecht Brain CenterUniversity Medical Center Utrecht, Biostatistics & Research Support, Julius Center for Health Sciences and Primary Care, Utrecht, The Netherlands and University Medical Center Utrecht, UMC Utrecht Brain CenterUniversity Medical Center Utrecht, UMC Utrecht Brain CenterAbstract 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.http://link.springer.com/article/10.1186/s13023-020-1348-2Spinal muscular atrophySMAFatigabilityEnduranceOutcome measureMuscle fatigue |
| spellingShingle | Bart Bartels Janke F. de Groot Laura E. Habets Camiel A. Wijngaarde Wendy Vink Marloes Stam Fay-Lynn Asselman Ruben P. A. van Eijk W. Ludo van der Pol Fatigability in spinal muscular atrophy: validity and reliability of endurance shuttle tests Orphanet Journal of Rare Diseases Spinal muscular atrophy SMA Fatigability Endurance Outcome measure Muscle fatigue |
| 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 | Spinal muscular atrophy SMA Fatigability Endurance Outcome measure Muscle fatigue |
| url | http://link.springer.com/article/10.1186/s13023-020-1348-2 |
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