Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2015 Oct 7;2(4):463-470.
doi: 10.3233/JND-150101.

Single-Blind, Randomized, Controlled Clinical Trial of Exercise in Ambulatory Spinal Muscular Atrophy: Why are the Results Negative?

Jacqueline Montes  1   2 , Carol Ewing Garber  3 , Samantha S Kramer  1 , Megan J Montgomery  1 , Sally Dunaway  1   2 , Shirit Kamil-Rosenberg  3 , Brendan Carr  3 , Rosangel Cruz  1 , Nancy E Strauss  2 , Douglas Sproule  1 , Darryl C De Vivo  1
Affiliations

Single-Blind, Randomized, Controlled Clinical Trial of Exercise in Ambulatory Spinal Muscular Atrophy: Why are the Results Negative?

Jacqueline Montes et al. J Neuromuscul Dis. .

Abstract

Background: The benefits of exercise on long-term health and well-being are well established. The possible benefits of exercise in Spinal Muscular Atrophy (SMA) have not been explored in a controlled clinical trial format.

Objective: To assess the effects of exercise on measures of function, strength, and exercise capacity in ambulatory SMA patients.

Methods: Fourteen participants, ages 10-48 years, were randomized to control and exercise cohorts after a 1 month lead-in period. The exercise group received 6 months of intervention. Thereafter, both groups received the intervention for the remaining 12 months. Participants were monitored for a total of 19 months. Exercise included individualized home-based cycling and strengthening. The primary outcome measure was distance walked during the six-minute walk test (6MWT). Secondary outcomes included strength, function, exercise capacity, quality of life and fatigue.

Results: Twelve participants completed the first 7 months of the study, and 9 completed all 19 months. At baseline, the groups were similar on all clinical variables. There were no group changes at any time point in the 6MWT, fatigue, or function. Percent-predicted VO2 max improved 4.9% in all participants in 6 months (p = 0.036) (n = 10).

Conclusion: Daily exercise is safe in ambulatory SMA and should be encouraged. We did not uncover any deleterious effects on strength, function, or fatigue. Our study documented a reduction in oxidative capacity and a blunted conditioning response to exercise possibly representing an important insight into underlying pathophysiological mechanisms. These findings also may be linked causally to mitochondrial depletion in SMA and warrant further study.

Keywords: Spinal muscular atrophy; aerobic; exercise; six minute walk test; strength.

PubMed Disclaimer

Figures

Fig.1
Fig.1
Percent change in scores over 6 months for strength and functional measures for the exercise (black bars) and control (gray bars) groups.
See this image and copyright information in PMC

References

    1. Darras BT, Markowitz JA, Monani UR, De Vivo DC. Chapter 8 - Spinal Muscular Atrophies. In: Basil T, Darras HRJ, Ryan Monique M, De Vivo Darryl C, editors. Neuromuscular Disorders of Infancy, Childhood, and Adolescence (Second Edition) San Diego: Academic Press; 2015. pp. 117–145.
    1. Deymeer F, Serdaroglu P, Poda M, Gulsen-Parman Y, Ozcelik T, Ozdemir C. Segmental distribution of muscle weakness in SMA III: Implications for deterioration in muscle strength with time. Neuromuscul Disord. 1997;7(8):521–528. - PubMed
    1. Deymeer F, Serdaroglu P, Parman Y, Poda M. Natural history of SMA IIIB. Neurology. 2008;71:664–449. - PubMed
    1. Garber CE, Blissmer B, Deschenes MR, Franklin BA, Lamonte MJ, Lee IM, et al. American College of Sports Medicine position stand. Quantity and quality of exercise for developing and maintaining cardiorespiratory, musculoskeletal, and neuromotor fitness in apparently healthy adults: Guidance for prescribing exercise. Med Sci Sports Exerc. 2011;43(7):1334–1359. - PubMed
    1. Sveen ML, Jeppesen T, Hauerslev S, Kober L, Krag TO, Vissing J. Endurance training improves fitness and strength in patients with becker muscular dystrophy. Brain. 2008. pp. 2824–2831. - PubMed

LinkOut - more resources