Aerobic conditioning: An effective therapy in McArdle's disease
- 22 May 2006
- journal article
- research article
- Published by Wiley in Annals of Neurology
- Vol. 59 (6), 922-928
- https://doi.org/10.1002/ana.20881
Abstract
Objective Susceptibility to exertional cramps and rhabdomyolysis in myophosphorylase deficiency (McArdle's disease [MD]) may lead patients to shun exercise. However, physical inactivity may worsen exercise intolerance by further reducing the limited oxidative capacity caused by blocked glycogenolysis. We investigated whether aerobic conditioning can safely improve exercise capacity in MD. Methods Eight MD patients (4 men and 4 women; age range, 33–61 years) pedaled a cycle ergometer for 30 to 40 minutes a day, 4 days a week, for 14 weeks, at an intensity corresponding to 60 to 70% of maximal heart rate. We monitored serum creatine kinase levels; changes in peak cycle work, oxygen uptake, and cardiac output; presence and magnitude of a spontaneous and glucose‐induced second wind; and citrate synthase and β‐hydroxyacyl coenzyme A dehydrogenase enzyme activities in quadriceps muscle. Results The prescribed exercise program increased average work capacity (36%), oxygen uptake (14%), cardiac output (15%), and citrate synthase and β‐hydroxyacyl coenzyme A dehydrogenase enzyme levels (80 and 62%, respectively) without causing pain or cramping or increasing serum creatine kinase. A spontaneous and glucose‐induced second wind was present and was of similar magnitude in each patient before and after training. Interpretation Moderate aerobic exercise is an effective means of improving exercise capacity in MD by increasing circulatory delivery and mitochondrial metabolism of bloodborne fuels. Ann Neurol 2006;59:922‐928Keywords
This publication has 27 references indexed in Scilit:
- Improved energy kinetics following high protein diet in McArdle's syndrome. A 31P magnetic resonance spectroscopy studyActa Neurologica Scandinavica, 2009
- Pharmacological and nutritional treatment for McArdle's disease (Glycogen Storage Disease type V)Published by Wiley ,2004
- A diagnostic cycle test for McArdle's diseaseAnnals of Neurology, 2003
- Spontaneous "Second Wind" and Glucose-Induced Second "Second Wind" in McArdle DiseaseArchives of Neurology, 2002
- Myophosphorylase Deficiency (Glycogenosis Type V McArdle Disease)Current Molecular Medicine, 2002
- Creatine Therapy in Myophosphorylase Deficiency (McArdle Disease)Archives of Neurology, 2000
- Metabolic causes of myoglobinuriaAnnals of Neurology, 1990
- THE SECOND WIND PHENOMENON IN McARDLE'S DISEASEBrain, 1986
- Myopathy in McArdle's SyndromeNew England Journal of Medicine, 1985
- Enzyme levels in pools of microdissected human muscle fibres of identified type: Adaptive response to exerciseActa Physiologica Scandinavica, 1984