Literature DB >> 25418505

Training improves oxidative capacity, but not function, in spinal muscular atrophy type III.

Karen Lindhardt Madsen1, Regitze Sølling Hansen1, Nicolai Preisler1, Frank Thøgersen1, Martin Peter Berthelsen1, John Vissing1.   

Abstract

INTRODUCTION: In this study we investigated the effect of 12 weeks of cycle ergometer training in patients with spinal muscular atrophy type III (SMA III), a hereditary motor neuron disease with progressive muscle weakness and atrophy.
METHODS: Six SMA III patients and 9 healthy subjects completed a 12-week training program, performing 42 30-minute sessions exercising at 65-70% of maximal oxygen uptake (VO2max ). VO2max , muscle strength, functional tests, and self-reported activities of daily living were assessed before and after the training.
RESULTS: Training induced a 27 ± 3% increase in VO2max (17 ± 2 to 21 ± 2 ml/kg/min, P < 0.001) in patients. However, fatigue was a major complaint and caused 1 patient to drop out, increased the need for sleep in 3 patients, and led to training modifications in 2 patients.
CONCLUSIONS: Cycle exercise improves VO2max in SMA III without causing muscle damage, but it also induces significant fatigue. This warrants study into alternative training methods to improve exercise capacity in SMA III patients.
© 2014 Wiley Periodicals, Inc.

Entities:  

Keywords:  SMA III; exercise; fatigue; motor neuron disease; neuromuscular disorders; spinal muscular atrophy type III; training

Mesh:

Year:  2015        PMID: 25418505     DOI: 10.1002/mus.24527

Source DB:  PubMed          Journal:  Muscle Nerve        ISSN: 0148-639X            Impact factor:   3.217


  17 in total

1.  Chronic Treatment with the AMPK Agonist AICAR Prevents Skeletal Muscle Pathology but Fails to Improve Clinical Outcome in a Mouse Model of Severe Spinal Muscular Atrophy.

Authors:  Clàudia Cerveró; Neus Montull; Olga Tarabal; Lídia Piedrafita; Josep E Esquerda; Jordi Calderó
Journal:  Neurotherapeutics       Date:  2016-01       Impact factor: 7.620

Review 2.  Exercise Therapy in Spinobulbar Muscular Atrophy and Other Neuromuscular Disorders.

Authors:  Julia Rebecka Dahlqvist; John Vissing
Journal:  J Mol Neurosci       Date:  2015-11-19       Impact factor: 3.444

3.  Diminished muscle oxygen uptake and fatigue in spinal muscular atrophy.

Authors:  Jacqueline Montes; Ashley M Goodwin; Michael P McDermott; David Uher; Feliz Marie Hernandez; Kayla Coutts; Julia Cocchi; Margarethe Hauschildt; Kayla M Cornett; Ashwini K Rao; Umrao R Monani; Carol Ewing Garber; Darryl C De Vivo
Journal:  Ann Clin Transl Neurol       Date:  2021-03-31       Impact factor: 5.430

Review 4.  Physical exercise training for type 3 spinal muscular atrophy.

Authors:  Bart Bartels; Jacqueline Montes; W Ludo van der Pol; Janke F de Groot
Journal:  Cochrane Database Syst Rev       Date:  2019-03-01

5.  Aerobic Training in Patients with Congenital Myopathy.

Authors:  Gitte Hedermann; Christoffer Rasmus Vissing; Karen Heje; Nicolai Preisler; Nanna Witting; John Vissing
Journal:  PLoS One       Date:  2016-01-11       Impact factor: 3.240

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

Authors:  Jacqueline Montes; Carol Ewing Garber; Samantha S Kramer; Megan J Montgomery; Sally Dunaway; Shirit Kamil-Rosenberg; Brendan Carr; Rosangel Cruz; Nancy E Strauss; Douglas Sproule; Darryl C De Vivo
Journal:  J Neuromuscul Dis       Date:  2015-10-07

Review 7.  Emerging therapies and challenges in spinal muscular atrophy.

Authors:  Michelle A Farrar; Susanna B Park; Steve Vucic; Kate A Carey; Bradley J Turner; Thomas H Gillingwater; Kathryn J Swoboda; Matthew C Kiernan
Journal:  Ann Neurol       Date:  2017-02-17       Impact factor: 10.422

Review 8.  Advances in spinal muscular atrophy therapeutics.

Authors:  Valeria Parente; Stefania Corti
Journal:  Ther Adv Neurol Disord       Date:  2018-02-05       Impact factor: 6.570

9.  Vibration-Assisted Home Training Program for Children With Spinal Muscular Atrophy.

Authors:  Christina Stark; Ibrahim Duran; Sebahattin Cirak; Stefanie Hamacher; Heike-Katharina Hoyer-Kuhn; Oliver Semler; Eckhard Schoenau
Journal:  Child Neurol Open       Date:  2018-06-10

Review 10.  In Search of a Cure: The Development of Therapeutics to Alter the Progression of Spinal Muscular Atrophy.

Authors:  Kristine S Ojala; Emily J Reedich; Christine J DiDonato; Stephen D Meriney
Journal:  Brain Sci       Date:  2021-02-05
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