European Journal of Nuclear Medicine

, Volume 22, Issue 11, pp 1256–1260

Impairment of the exercise-induced increase in muscle perfusion in McArdle's disease

  • Philippe Jehenson
  • Anne Leroy-Willig
  • Eric de Kerviler
  • Pascal Merlet
  • Denis Duboc
  • André Syrota
Original Article

DOI: 10.1007/BF00801609

Cite this article as:
Jehenson, P., Leroy-Willig, A., de Kerviler, E. et al. Eur J Nucl Med (1995) 22: 1256. doi:10.1007/BF00801609

Abstract

In McArdle's disease (myophosphorylase deficiency) exercise intolerance is generally attributed to a lack of glycogenolysis, which decreases energy production during exercise. Magnetic resonance imaging data have recently suggested an impairment of the increase in muscle perfusion during exercise in these patients. We have tested this hypothesis by direct measurement of local muscle perfusion increase. Increase in muscle perfusion was assessed by positron emission tomography with oxygen-15 labelled water in five patients with McArdle's disease and five age- and sex-matched healthy volunteers. Radioactivity was measured in both forearms before and after exercise of the right forearm. The exercise intensity was biochemically assessed by in vivo phosphorus-31 magnetic resonance spectroscopy. The estimated increase in muscle perfusion with exercise was 5.7±5.5-fold in the patients (range 1.5–12.8) and 22.3±12.0-fold in the healthy subjects (range 10.1–37) (P=0.022). The results show a significant impairment of increase in muscle perfusion with exercise in McArdle's disease. Thus patients may suffer not only from a direct lack of glycogenolysis but also from indirectly impaired vasodilation.

Key words

McArdle's disease Muscle perfusion Positron emission tomography Magnetic resonance spectroscopy 

Copyright information

© Springer-Verlag 1995

Authors and Affiliations

  • Philippe Jehenson
    • 1
  • Anne Leroy-Willig
    • 1
  • Eric de Kerviler
    • 1
  • Pascal Merlet
    • 1
  • Denis Duboc
    • 2
  • André Syrota
    • 1
  1. 1.Service Hospitalier Frédéric JoliotCommissariat à l'Energie AtomiqueOrsayFrance
  2. 2.INSERM U153 and Service de CardiologieHôpital CochinParisFrance

Personalised recommendations