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THE SECOND WIND PHENOMENON IN McARDLE'S DISEASE

J. P. BRAAKHEKKE, M. I. DE BRUIN, D. F. STEGEMAN, R. A. WEVERS, R. A. BINKHORST, E. M. G. JOOSTEN
DOI: http://dx.doi.org/10.1093/brain/109.6.1087 1087-1101 First published online: 1 December 1986

Summary

Three patients with McArdle's disease exercised for 2 h at 30% VO2max. During exercise two phases occurred During the first 15 min they experienced progressive fatigue and weakness of exercised muscles, with a rapid and complete recovery (adaptation phase). Following this, all 3 patients were able to continue exercise without difficulty (‘second wind’ phase). During the adaptation phase, patients have to cope with their inability to use muscle glycogen as a fuel Processes occurring during this phase are as follows. (1) An increase in cardiac output. This might be expected to increase muscle blood flow in order to supply exercising muscle with substrates that can substitute for muscle glycogen (free fatty acids (FFA), bloodborne glucose). (2) Changes in the metabolic pathways. These cause a sufficient amount of hexose phosphates to be present to overcome the first 2.5 min of exercise, and FFA and bloodborne glucose to play a major role in energy supply at an earlier stage in exercise than in control subjects. (3) An increase in EMG activity. This is most probably caused by the recruitment of more motor units to compensate for a failure of force generation in the muscle fibres.

Central command seems to play an important role in the regulation of cardiovascular processes during the adaptation phase. Despite these compensatory mechanisms, metabolic stress occurs during the adaptation phase During the ‘second wind’ phase there are no important differences between the metabolism of exercising muscle of patients with McArdle's disease and that of control subjects.