Permanent muscle weakness in MCArdle disease
✍ Scribed by Aleksandra A. Nadaj-Pakleza; Carlo M. Vincitorio; Pascal Laforêt; Bruno Eymard; Elisabeth Dion; Susana Teijeira; Irene Vietez; Marc Jeanpierre; Carmen Navarro; Tanya Stojkovic
- Publisher
- John Wiley and Sons
- Year
- 2009
- Tongue
- English
- Weight
- 233 KB
- Volume
- 40
- Category
- Article
- ISSN
- 0148-639X
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McArdle's disease or myophosphorylase deficiency is one of the most common muscle glycogenoses and typically presents in childhood or adolescence with exercise intolerance, myalgia, myoglobinuria, and cramps in exercising muscle. We describe an elderly man who developed asymmetric proximal arm weakn
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Impairment of muscle glycogenolysis in McArdle's disease (myophosphorylase deficiency) leads to exercise intolerance and exercise-induced myalgia. The pathophysiology of these symptoms is not entirely clear. We used phosphorus magnetic resonance spectroscopy to measure muscle phosphate metabolite co