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Exercise training in metabolic myopathies.

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  • 1Copenhagen Neuromuscular Center, section 6921, Department of Neurology, University of Copenhagen, Rigshospitaletn, Juliane Maries Vej 28, DK-2100 Copenhagen, Danemark.

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Metabolic myopathies, including muscle glycogenoses (GSD) and fatty acid oxidation disorders (FAOD), cause exercise intolerance due to energy deficiency. Moderate aerobic exercise and specific fuel strategies can improve performance and are recommended adjunct therapies.

Keywords:
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Area of Science:

  • Neurology
  • Metabolic Disorders
  • Exercise Physiology

Background:

  • Metabolic myopathies (GSD, FAOD) cause exercise intolerance due to impaired ATP production.
  • Unlike structural myopathies, exercise limitations stem from energy deficiency, not muscle damage.
  • Exercise testing is crucial for diagnosing limitations in work capacity and endurance.

Purpose of the Study:

  • To review exercise responses and training recommendations for metabolic myopathies.
  • To explore the role of exercise and nutritional interventions in managing these conditions.
  • To highlight the benefits of exercise despite historical contraindications.

Main Methods:

  • Review of clinical phenotypes of GSD and FAOD.
  • Analysis of exercise responses based on energy substrate utilization (glycogen vs. fat).
  • Evaluation of existing literature on exercise training and dietary interventions.

Main Results:

  • GSD patients experience early exercise symptoms due to glycogen reliance.
  • FAOD patients develop symptoms later in exercise due to fat oxidation dependence.
  • Moderate aerobic exercise and short, low-intensity resistance training are generally well-tolerated.
  • Fuel supplementation and dietary interventions can enhance exercise performance.

Conclusions:

  • Exercise intolerance in metabolic myopathies is linked to ATP supply limitations.
  • Contrary to previous advice, exercise, particularly moderate aerobic training, is beneficial.
  • Training may improve metabolic pathways and exercise capacity.
  • Combined exercise and nutritional strategies offer promising therapeutic approaches.