Creatine for McArdle Disease
Abstract & Commentary
Source: Vorgerd M, et al. Creatine therapy in myophosphorylase deficiency. Arch Neurol 2000;57:956-963.
Creatine therapy benefits in mitochondrial cytopathies (Muscle Nerve 1997;20:1502-1509), as well as various forms of muscular dystrophy (MD), including facioscapulohumeral, Becker, Duchenne (n = 8), and sarcoglycan-deficient MD (Neurology 2000; 54:1848-1850). It also improves exercise performance in myophosphorylase deficiency (McArdle disease). Nine McArdle patients, including six women and three men (mean age, 39.1 years) entered into a double-blind, placebo-controlled, crossover study using placebo or creatine, 150 mg/kg/d, for one week followed by 60 mg/kg/d for four weeks. A four-week washout period separated the active and placebo phases. Measurements included maximal ergometric bicycle-exercise workload and duration, fatigue severity scale (Arch Neurol 1989;46:1121-1123), grading of exercise-induced muscle pain, and frequency of muscle pain per week. Phosphorous 31 NMR spectroscopy of the right calf (Neuromusc Disord 1998;8:480-488), and surface electromyographic (sEMG) spectral distribution were obtained (Ergonomics 1996;39:298-313). Statistical analysis was performed with two sample t tests and the sign test.
All nine patients completed the study. FSS and maximal ergometric bicycle-exercise workload and duration did not differ between the two study arms. Four persons, however, reported reduced frequency, and five reported subjective improvement of muscle pain and wished to continue creatine treatment. Creatine resulted in a significantly increased force-time integral on 31P-NMR data during ischemic, but not aerobic, exercise, and increased EMG amplitude was recorded during sustained contraction. Other than transient mild headache and dizziness in a single patient, no adverse effects were reported. Creatine is safe and often beneficial for McArdle disease. —michael rubin
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