To the Editor.
—Recent articles have held forth hope that certain metabolic myopathies might undergo improvement with proper dietary therapy.1-3 In particular, the simple expedient of a high protein diet has appeared to be remarkably effective in the disparate disorders of McArdle's disease,1 debrancher deficiency,2 and acid maltase deficiency.3 With respect to McArdle's disease, a single patient, described by Slonim and Goans, demonstrated marked improvement after consuming a high protein diet.1 Indeed, 45 minutes after a high protein (25% to 35%) meal, his exercise tolerance improved 400%. Over a 3-year period, the patient also experienced a decrease in muscular atrophy and an increase in exercise tolerance. The investigators discussed the possibility that increased availability of branched-chain amino acids (BCAAs) contributed to the improvement by serving as an alternative energy source for exercising muscle. To focus on this possibility, we studied the functional and metabolic muscle
Kushner RF, Berman SA. Are High-Protein Diets Effective in McArdle's Disease? Arch Neurol. 1990;47(4):383–384. doi:10.1001/archneur.1990.00530040023014
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