This study was supported by the Department of Defense; USAMRAA Grant/Cooperative award number: 09131005(W81XWH-10-1-0127) and the SMA Foundation. AKR received research support from NICHD (K01 HD060912).
Leg muscle function and fatigue during walking in spinal muscular atrophy type 3
Version of Record online: 5 MAY 2014
Copyright © 2013 Wiley Periodicals, Inc.
Muscle & Nerve
Volume 50, Issue 1, pages 34–39, July 2014
How to Cite
Montes, J., Dunaway, S., Garber, C. E., Chiriboga, C. A., De Vivo, D. C. and Rao, A. K. (2014), Leg muscle function and fatigue during walking in spinal muscular atrophy type 3. Muscle Nerve, 50: 34–39. doi: 10.1002/mus.24081
The sponsors had no role in the conduct of this study.
- Issue online: 19 JUN 2014
- Version of Record online: 5 MAY 2014
- Accepted manuscript online: 1 OCT 2013 03:27AM EST
- Manuscript Accepted: 10 SEP 2013
- Manuscript Revised: 6 SEP 2013
- Manuscript Received: 11 JUN 2013
- The Department of Defense
- USAMRAA . Grant Number: 09131005(W81XWH-10-1-0127)
- The SMA Foundation
- AKR received research support from NICHD . Grant Number: K01 HD060912
- muscle strength;
- outcome measure;
- six-minute walk test;
- spinal muscular atrophy
Introduction: Spinal muscular atrophy (SMA) causes muscle weakness and fatigue. Better understanding of the relationship between weakness and fatigue may help identify potential targets for rehabilitation. Methods: Gait and surface electromyography (EMG) from 4 muscle groups were measured during the Six-Minute Walk Test (6MWT) in 10 ambulatory participants, aged 9–49 years. Average root mean square amplitude (RMS) of muscle activity was calculated. Strength was assessed using manual and quantitative methods. Results: RMS, stride length, and velocity decreased during the 6MWT. Knee flexor and hip abductor strength was associated with fatigue-related changes; overall strength correlated with disease duration; and leg strength was associated with 6MWT distance. Conclusions: Clinical measures are valid in assessing fatigue and function in SMA, and these assessments can be enhanced by use of gait analysis and EMG. Disease duration and strength measures may represent further stratification refinements when enrolling patients in clinical trials. Muscle Nerve 50: 34–39, 2014