Background Energy metabolism in muscles relies predominantlyon the breakdown of glycogen early in exercise. In patientswith McArdle's disease, blocked glycogenolysis in muscles resultsin low exercise tolerance and can lead to muscle injury, particularlyin the first minutes of exercise. We hypothesized that ingestingsucrose before exercise would increase the availability of glucoseand would therefore improve exercise tolerance in patients withMcArdle's disease.
Methods In a single-blind, randomized, placebo-controlled crossoverstudy, 12 patients with McArdle's disease drank 660 ml of abeverage that had been sweetened with artificial sweeteners(placebo) or with 75 g of sucrose after an overnight fast. Thirtyto 40 minutes later, the patients rode a stationary bicycleat a constant workload for 15 minutes while the heart rate,level of perceived exertion, and venous blood glucose levelswere monitored.
Results Supplemental sucrose increased the mean plasma glucoselevel by more than 36 mg per deciliter (2.0 mmol per liter)and resulted in a marked improvement in exercise tolerance inall patients. The mean (±SE) heart rate dropped by amaximum of 34±3 beats per minute (P<0.001), and thelevel of perceived exertion fell dramatically when the patientsingested glucose as compared with when they received the placebo.
Conclusions This study suggests that the ingestion of sucrosebefore exercise can markedly improve exercise tolerance in patientswith McArdle's disease. The treatment takes effect during thetime when muscle injury commonly develops in these patients.In addition to increasing the patients' exercise capacity andsense of well-being, the treatment may protect against exercise-inducedrhabdomyolysis.
Source Information
From the Department of Neurology and the Copenhagen Muscle Research Center, National University Hospital, Rigshospitalet, Copenhagen, Denmark (J.V.); and the Departments of Neurology at Veterans Affairs Medical Center and University of Texas Southwestern Medical Center, and the Neuromuscular Center, Institute for Exercise and Environmental Medicine, Presbyterian Hospital (R.G.H.) all in Dallas.
Address reprint requests to Dr. Vissing at the Neuromuscular Clinic, Department of Neurology 2082, National University Hospital, Rigshospitalet, Blegdamsvej 9, DK-2100 Copenhagen, Denmark, or at vissing{at}rh.dk.
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