Effect of High-Dose Creatine Therapy on Symptoms of Exercise Intolerance in McArdle Disease

Vorgerd, Matthias; Zange, Jochen; Kley, Rudolf A.; Grehl, Torsten; Hüsing, Anika; Jäger, Matthias; Müller, Klaus; Schröder, Rolf; Mortier, W.; Fabian, K.; Malin, J.‐P.; Luttmann, Alwin

doi:10.1001/archneur.59.1.97

Cited by 92 publications

(60 citation statements)

References 25 publications

(9 reference statements)

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“…28 Even if we observed a small increase in the PCr/ATP ratio in our patients receiving Cr, this change did not reach the significance threshold, possibly due to the small number of patients investigated. We calculated that 12 subjects would have been necessary to detect a statistical increase (P Ͻ 0.05) in PCr/ATP ratio.…”

Section: Discussioncontrasting

confidence: 61%

Beneficial effects of creatine supplementation in dystrophic patients

et al. 2003

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The effect of creatine (Cr) supplementation on muscle function and body composition of 12 boys with Duchenne muscular dystrophy and three with Becker dystrophy was evaluated by a randomized double-blind cross-over study (3 g Cr or maltodextrin daily for 3 months, with wash-out period of 2 months). After placebo, no change was observed in maximal voluntary contraction (MVC) and resistance to fatigue, whereas total joint stiffness (TJS) was increased by approximately 25% (P < 0.05). The patients receiving Cr did not show any change in TJS, improved MVC by 15% (P = 0.02), and almost doubled their resistance to fatigue (P < 0.001). In patients still independent of a wheelchair (n = 5), bone mineral density increased by 3% (P < 0.05), and urinary excretion of collagen type I cross-linking N-telopeptide declined to about one third (P < 0.001) after Cr. No adverse effect was observed. Thus, Cr may provide some symptomatic benefit in these patients.

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Section: Discussioncontrasting

confidence: 61%

Beneficial effects of creatine supplementation in dystrophic patients

et al. 2003

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“…Attempts to boost muscle energy metabolism in McArdle patients by treatment with D-ribose, pyridoxine, creatine, or branched-chain amino acids have failed to improve exercise tolerance, [8][9][10][11] and gene therapy is far from being applied to humans. 12 Pre-exercise ingestion of sucrose and aerobic conditioning are therefore currently the most effective treatments presently available for patients with McArdle disease.…”

Section: Commentmentioning

confidence: 99%

Effect of Oral Sucrose Shortly Before Exercise on Work Capacity in McArdle Disease

Andersen¹,

Haller²,

Vissing³

2008

Arch Neurol

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“…However, in patients with McArdle disease, oral creatine supplementation in both high (150 mg/kg daily) and low (60 mg/kg daily) doses did not result in elevated PCr/ATP levels. 6,7 With low-dose creatine, working capacity for ischemic exercise improved, and we found a higher consumption of PCr during aerobic and ischemic low-level exercise in patients with McArdle disease. However, high-dose creatine cannot be recommended because it worsened clinical symptoms of exercise intolerance; patients reported an increase in both the number of pain episodes and the myalgia pain score.…”

Section: Creatine Treatmentmentioning

confidence: 68%

Therapeutic Options in Other Metabolic Myopathies

Vorgerd

2008

Neurotherapeutics

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Summary: Adult patients with metabolic myopathies typically present with exercise-induced pain, cramps, fatigue, and myoglobinuria. The current therapeutic options of glycogen and lipid storage myopathies include dietary treatments, excersise training, and pharmacological supplementations. Herein is a review of evidence from randomized controlled trials in McArdle disease (glycogen storage disease type V, muscle phosphorylase deficiency) and carnitine palmitoyltransferase (CPT) 2 deficiency. A brief overview on current treatment options in rhabdomyolysis is also included because patients with McArdle disease and CPT 2 often experience such potentially life-threatening complications. Key Words: McArdle disease, rhabdomyolysis, metabolic myopathy, carnitine palmitoyltransferase (CPT) 2 deficiency, glycogen storage disease, lipid storage disease.

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Effect of High-Dose Creatine Therapy on Symptoms of Exercise Intolerance in McArdle Disease

Cited by 92 publications

References 25 publications

Beneficial effects of creatine supplementation in dystrophic patients

Beneficial effects of creatine supplementation in dystrophic patients

Effect of Oral Sucrose Shortly Before Exercise on Work Capacity in McArdle Disease

Therapeutic Options in Other Metabolic Myopathies

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