Sports in metabolic myopathies

The primary hereditary metabolic myopathies are a heterogeneous group of di sorders which usually impair muscle energy metabolism. They include defects of glycolysis and glycogenolysis, defects of mitochondrial fatty acid beta -oxidation and defects of the mitochondrial respiratory chain. Episodes of metabolic decompensation may lead to rhabdomyolysis in all of these disord ers. According to the underlying pathophysiological events, different recom mendations regarding exercise have to be made for the various disorders, be cause in principle any physical exertion can trigger an episode of rhabdomy olysis. On the other hand, muscle strength and endurance are improved by ap propriate physical exercise. The patient must be well informed about his di sease in order to avoid potentially life-threatening complications due to e xcessive physical exercise.