Genetics of Glycogen-Storage Disease Type V Treatment & Management

Updated: Nov 10, 2015
  • Author: Edward J Cupler, MD, FAAN; Chief Editor: Maria Descartes, MD  more...
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Medical Care

Therapies in patients with McArdle disease (glycogen-storage disease type V) are used in an attempt to bypass the metabolic block by providing glucose or fructose yield inconsistent results. Similarly, injection of glucagons yields inconsistent results.



Acute renal failure may occur with rhabdomyolysis, necessitating consultation with a nephrologist.

Monitor renal function in all patients with McArdle disease. This may be performed in conjunction with a nephrologist.



A high carbohydrate diet may improve maximal work capacity and exercise tolerance. A randomized controlled trial comparing a carbohydrate rich (20% fat, 15% protein, 65% carbohydrate) and a protein rich diet (15% fat, 55% protein, 30% carbohydrate) surprisingly showed that subjects on the carbohydrate rich diet improved exercise tolerance and maximum oxidative capacity compared with subjects on the protein rich diet. [11]



Regular, moderate aerobic activity has been shown to improve exercise capacity in patients with McArdle disease. Patients should exercise using a heart rate monitor, keeping the heart rate to 60-70% of maximum. [12] Exercise should be preceded by a warm-up period and dose of sucrose prior to exertion if not contraindicated. [13]

Strenuous isometric exercises may cause symptoms or rhabdomyolysis.