ABSTRACT
Patients with mitochondrial myopathies (MM) or myophosphorylase deficiency (McArdle's disease, McA) show impaired capacity for O(2) extraction, low maximal aerobic power, and reduced exercise tolerance. Non-invasive tools are needed to quantify the metabolic impairment. Six patients with MM, 6 with McA, 25 with symptoms of metabolic myopathy but negative biopsy (patient-controls, P-CTRL) and 20 controls (CTRL) underwent an incremental cycloergometric test. Pulmonary O(2) uptake (VO(2)) and vastus lateralis oxygenation indices (by near-infrared spectroscopy, NIRS) were determined. Concentration changes of deoxygenated hemoglobin and myoglobin (Delta[deoxy(Hb + Mb)]) were considered an index of O(2) extraction. Delta[deoxy(Hb + Mb)] peak (percent limb ischemia) was lower in MM (25.3 +/- 12.0%) and McA (18.7 +/- 7.3) than in P-CTRL (62.4 +/- 3.9) and CTRL (71.3 +/- 3.9) subjects. VO(2) peak and Delta[deoxy(Hb + Mb)] peak were linearly related (r(2) = 0.83). In these patients, NIRS is a tool to detect and quantify non-invasively the metabolic impairment, which may be useful in the follow-up of patients and in the assessment of therapies and interventions.
Subject(s)
Glycogen Storage Disease Type V/diagnosis , Glycogen Storage Disease Type V/metabolism , Mitochondrial Myopathies/diagnosis , Mitochondrial Myopathies/metabolism , Oxygen Consumption , Spectroscopy, Near-Infrared/methods , Adult , Aged , Energy Metabolism/physiology , Exercise/physiology , Exercise Test , Exercise Tolerance/physiology , Female , Glycogen Storage Disease Type V/physiopathology , Heart Rate/physiology , Hemoglobins/analysis , Hemoglobins/metabolism , Humans , Male , Mitochondrial Myopathies/physiopathology , Muscle, Skeletal/metabolism , Muscle, Skeletal/physiopathology , Myoglobin/metabolism , Oxidative Phosphorylation , Oxygen Consumption/physiology , Predictive Value of Tests , Reference Values , Spectroscopy, Near-Infrared/trendsABSTRACT
During exercises with relatively small muscle masses, limitations to exercise performance by the cardiovascular system should be significantly reduced, allowing one to fully-test the "oxidative potential" of the investigated muscles. Ten elderly males (E, 77.8 +/- 2.9 years [x +/- SD]) and eight young controls (Y, 26.6 +/- 3.0) underwent incremental exercises to voluntary exhaustion on a dynamic leg-extension (dominant limb) machine (knee-extension, KE) and on a cycloergometer (CYCLO). During KE the load was increased every 3 min to loads corresponding to 20, 40 and 60% of the force of one-repetition maximum (1RM). The following variables were determined (vastus lateralis muscle): concentration changes of deoxygenated haemoglobin and myoglobin (Delta[deoxy(Hb + Mb)]) by near-infrared spectroscopy (NIRS), expressed as percentage of the maximal value obtained during transient limb ischemia, and taken as an index of O2 extraction; root mean square (RMS) and median power frequency (MDF) by electromyography. The total lifted load during KE and peak workload during CYCLO were lower in E versus Y (620.4 +/- 321.9 kg vs. 1347.4 +/- 458.7; 113.5 +/- 23.9 W vs. 224.3 +/- 41.0, respectively). During CYCLO Delta[deoxy(Hb + Mb)] peak (i.e. the value determined at exhaustion) was lower in E (44.5 +/- 17.7%) versus Y (67.1 +/- 22.9), whereas during KE Delta[deoxy(Hb + Mb)] peak was higher in E (56.8 +/- 20.9%) versus Y (38.6 +/- 15.8). "Thresholds", that is abrupt increases in RMS slopes, were detected in Y but not in E, suggesting less recruitment or a preferential atrophy of type 2 fibers in the elderly. These findings, associated with the preserved capacity of O2 extraction, suggest a shift towards oxidative metabolism in skeletal muscles of 78 year-old subjects, which could preserve, at least in part, their capacity to carry out exercise.
