Electrophysiologic properties of intercostal muscle fibers in human neuromuscular diseases

Abstract

Electrophysiologic properties of biopsied normal and diseased intercostal muscle fibers were examined using intracellular microelectrode techniques. The resting potentials of all diseased muscle fibers were found to be depolarized. Those from Duchenne dystrophy patients showed the largest depolarization, followed by those from patients with myotonic muscular dystrophy, myotonia congenita, and motor neuron disease. All of the diseased fibers except those from myotonia congenita patients demonstrated an imparied ability to generate action potentials. In the latter fibers, the higher-than-normal membrane resistance was associated with hyperexcitability. When the membrane was hyperpolarized to the normal range, however, action potential characteristics in all fibers were near normal, except in motor neuron disease. All action potentials were blocked by tetrodotoxin. These findings--i.e., that all fibers were capable of generating action potentials when hyperpolarized, and that all action potentials were blocked by tetrodotoxin--suggest the relative intactness, in the disease studied here of the regenerative sodium conductance mechanism.