Exercise Testing

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Functional or exercise testing allows use of the most sophisticated techniques available to evaluate the metabolic machinery of working muscle. Exercise testing has been used for many years as a sensitive means of evaluating cardiac function, but only in the past decade have such methods been applied systematically to neuromuscular disorders. The team of physicians and scientists assembled as the faculty of the Neuromuscular Center have pioneered the application of such techniques to the study of human muscle disorders. Techniques used include:

1. measuring oxygen uptake and carbon dioxide production in relation to work intensity;
2. evaluating the delivery of oxygen to working muscle by monitoring changes in muscle blood flow and by measuring the increase in the amount of blood pumped by the heart with exercise;
3. monitoring patterns of metabolites released from working muscle into the bloodstream; and
4. using magnetic resonance spectroscopy in which magnetic properties of atoms found in muscle permit quantification of changes in the concentrations of molecules that are vitally involved in energy metabolism.

A unique capacity of the Neuromuscular Center is the ability to undertake comprehensive biochemical evaluation of the abnormalities discovered by exercise testing and to diagnose muscle disorders characterized by specific metabolic defects. These techniques include:

1. the microscopic evaluation of muscle structures important in energy production;
2. measuring levels of substances (such as vitamins) which normally activate certain energy pathways;
3. isolation of muscle mitochondria where oxidative energy production occurs and;
4. evaluation of individual proteins called enzymes which make up the metabolic machinery of energy production in the muscle cell.