Impairment of the exercise-induced increase in muscle perfusion in McArdle's disease
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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.
- McArdle B. Myopathy due to a defect in muscle glycogen breakdown.Clin Sci 1951; 10: 13–35.
- Tsujino S, Shanske S, Di Mauro S. Molecular genetics heterogeneity of myophosphorylase deficiency (McArdle's disease).N Engl JMed 1993; 329: 241–245.
- Porte D, Crawford DW, Jennings DB, Aber C, McIlroy MB. Cardiovascular and metabolic responses to exercise in a patient with McArdle's syndrome.N Engl J Med 1966; 275: 406–412.
- Barcroft H, Greenwood B, McArdle B, et al. The effect of exercise on forearm blood perfusion and on venous blood pH, pCO2 and lactate in a subject with phosphorylase deficiency in skeletal muscle (McArdle's syndrome) [abstract]J Physiol (Lond) 166; 189: 44P–46P.
- Lewis SF, Haller RG, Cook JD, Bloomqvist CG. Metabolic control of cardiac output response to exercise in McArdle's disease. J Appl Physiol 1984; 57: 1749–1753.
- Lewis SF, Haller RG, Henrikssen KG, Areskos NH, Jorfeldt L. Availability of oxydative substrate and leg blood perfusion in McArdle's disease [abstract]Fed Proc 1986; 45: 783.
- Lewis SF, Haller RG. Disorders of muscle glycogenolysis/glycolysis: the consequences of substrate-limited oxidative metabolism in humans. In: Taylor A, Gollnick PD, Green HJ, et al., eds.Biochemistry of exercise VII. Champaign, Ill: Human kinetics Publisher; 1990: 211–226.
- de Kerviler E, Leroy-Willig A, Jehenson P, Duboc D, Eymard B, Syrota A. Exercise-induced muscle modifications: study of healthy subjects and patients with metabolic myopathies with MR imaging and P-31 spectroscopy.Radiology 1991; 181: 259–264.
- Fleckenstein JL, Haller RF, Lewis SF, et al. Absence of exercise-induced MRI enhancement of skeletal muscle in McArdle disease.J Appl Physiol 1991; 71: 961–969.
- Jehenson P, Leroy-Willig A, de Kerviler E, Duboc D, Syrota A. MR imaging as a potential diagnostic test for metabolic myopathies: importance of variations in the T2 of muscle with exercise.AJR 1993; 161:347–351.
- Herscovitch P, Markham J, Raichle ME. Brain blood perfusion measured with intravenous H2 15O,J Nucl Med 1983; 24: 782–798.
- Bendriem B, Soussaline F, Campagnolo R, Verrey B, Wajnberg P, Syrota A. A technique for the correction of scattered radiation in a PET system using time-of-flight information.J Comput Assist Tomogr 1986; 10: 287–295.
- Trebossen R, Mazoyer B. Count rate performances of TTV03. The CEA-LETI time-of-flight positron emission tomograph.IEEE Trans Med Imag 1991; 10: 261–266.
- Duboc D, Jehenson P, Tran Dinh S, Marsac C, Syrota A, Fardeau M. Phosphorus NMR spectroscopy study of muscular enzyme deficiencies involving glycogenosis and glycolysis.Neurology 1987; 37: 663–671.
- Lassen NA, Lindbjerg J, Munck O. Measurement of bloodperfusion through skeletal muscle by intramuscular injection of xenon-133.Lancet 1964; 28: 686–689.
- Squire LR, Ojemann JG, Miezin FM, Petersen SE, Videen TO, Raichle ME. Activation of the hippocampus in normal humans: a functional anatomical study of memory.Proc Natl Acad Sci USA 1992; 89: 1837–1841.
- Iida H, Kanno I, Takahashi A, et al. Measurement of absolute myocardial blood perfusion with H2 15O and dynamic positron emission tomography.Circulation 1988; 78: 104–115.
- Bergmann SR, Herrero P, Markham J, Weinheimer CJ, Walsh MN. Non-invasive quantification of myocardial blood perfusion in human subjects with O-15 labeled water and positron emission tomography.J Am Coll Cardiol 1989; 14: 639–642.
- Araujo LI, Lammertsma AA, Rhodes CG, et al. Non-invasive quantification of regional myocardial blood perfusion in normal volunteers and patients with coronary artery disease using oxygen-15 labeled carbon dioxide inhalation and positron emission tomography.Circulation 1991; 83: 875–885.
- Lammertsma A, Mazoyer B. EEC concerted action on cellular degeneration and regeneration studied with PET. Modelling expert meeting blood flow measurement with PET.Eur J Nucl Med 1990; 16: 807–812.
- Donald KW, Lind AR, McNicol GW, Humphreys PW, Taylor SH, Stauton HP. Cardiovascular response to sustained static contractions.Circ Res 1967; 20: 15–30.
- Minotti JR, Johnson EC, Hudson TL, et al. Forearm metabolic asymmetry detected by 31P-NMR during submaximal exercise.J Appl Physiol 1989; 67: 324–329.
- Fleckenstein JL, Haller RG, Bertocci LA, Parkey RW, Peshock RM. Glycogenolysis, not perfusion, is the critical mediator of exercise-induced muscle modifications on MR imaging.Radiology 1992; 183: 25–26.
- Di Mauro S. Metabolic myopathies. In: Vinken PJ, Bruyn GW, Ringel SP, eds.Handbook of clinical neurology, vol 41. Diseases of muscle. Amsterdam: North Holland; 1979: 175–234.
- Impairment of the exercise-induced increase in muscle perfusion in McArdle's disease
European Journal of Nuclear Medicine
Volume 22, Issue 11 , pp 1256-1260
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- Online ISSN
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- McArdle's disease
- Muscle perfusion
- Positron emission tomography
- Magnetic resonance spectroscopy
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- Author Affiliations
- 1. Service Hospitalier Frédéric Joliot, Commissariat à l'Energie Atomique, 4 place du Général Leclerc, F-91406, Orsay, France
- 2. INSERM U153 and Service de Cardiologie, Hôpital Cochin, F-75014, Paris, France