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Related Experiment Videos

McArdle disease: another systemic low-inflammation disorder?

Alejandro Lucia1, Lucille Smith, Melissa Naidoo

  • 1Universidad Europea de Madrid, 28670 Madrid, Spain. alejandro.lucia@uem.es

Neuroscience Letters
|December 29, 2007
PubMed
Summary

McArdle disease patients exhibit low-level systemic inflammation, with elevated cytokines at rest. However, exercise triggers a similar anti-inflammatory response in patients and controls, suggesting supervised training is beneficial.

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Area of Science:

  • Exercise physiology
  • Immunology
  • Metabolic myopathies

Background:

  • McArdle disease results from a genetic defect in muscle glycogen phosphorylase, impairing glycogenolysis.
  • Patients typically experience exercise intolerance and chronic skeletal muscle damage.
  • This study investigates systemic inflammation markers in McArdle disease patients.

Purpose of the Study:

  • To compare circulating cytokine levels in McArdle disease patients and healthy controls.
  • To assess the impact of acute exercise on cytokine profiles in both groups.
  • To explore the potential benefits of exercise for managing inflammation in McArdle disease.

Main Methods:

  • Recruited 31 adult McArdle patients and 29 healthy controls.
  • Measured 27 circulating cytokines and neutrophil counts at rest and after a non-damaging cycling exercise bout.
  • Assessed serum creatine kinase activity to confirm ongoing muscle breakdown in patients.

Main Results:

  • McArdle patients showed higher resting neutrophil counts and elevated levels of TNF-alpha, IL-1ra, IL-10, IL-12, and IL-17 compared to controls.
  • The myokine IL-6 significantly increased post-exercise in both groups.
  • Patients exhibited baseline muscle breakdown (elevated creatine kinase) even when sedentary.

Conclusions:

  • McArdle disease is associated with a state of low-grade systemic inflammation.
  • Supervised exercise elicits a comparable anti-inflammatory response (increased IL-6) in patients and controls.
  • These findings support exercise training as a therapeutic strategy for McArdle disease.