Jehenson P, Leroy-Willig A, de Kerviler E, Merlet P, Duboc D, Syrota A
Service Hospitalier Frédéric Joliot, Commissariat à l'Energie Atomique, 4 place du Général Leclerc, F-91406 Orsay, France.
Eur J Nucl Med. 1995 Nov;22(11):1256-60. doi: 10.1007/BF00801609.
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.
在麦克尔迪氏病（肌磷酸化酶缺乏症）中，运动不耐受通常归因于糖原分解缺乏，这会减少运动期间的能量产生。磁共振成像数据最近表明，这些患者在运动期间肌肉灌注增加存在受损情况。我们通过直接测量局部肌肉灌注增加来检验这一假设。在5例麦克尔迪氏病患者和5名年龄及性别匹配的健康志愿者中，使用氧-15标记水的正电子发射断层扫描来评估肌肉灌注增加情况。在右侧前臂运动前后，测量双侧前臂的放射性。通过体内磷-31磁共振波谱对运动强度进行生化评估。患者运动时估计的肌肉灌注增加为5.7±5.5倍（范围1.5 - 12.8），健康受试者为22.3±12.0倍（范围10.1 - 37）（P = 0.022）。结果显示，麦克尔迪氏病患者运动时肌肉灌注增加存在显著受损。因此，患者不仅可能直接因糖原分解缺乏而受苦，还可能因间接的血管舒张受损而受苦。
