Neuromuscular junction alterations in extraocular muscles of myotonic rats

Abstract

Rats treated with 20,25-diazacholesterol manifest clinical as well as physiological signs characteristic of human myotonic dystrophy. The extraocular muscles (EOMs) of such myotonic rats were shewn in a prior study to exhibit by electromyography, prolonged insertion activity, high frequency bizzare discharges, and myotonic responses, which are comparable to that observed in skeletal muscle. Light and electron microscopy of the EOMs revealed numerous fiber alterations, i.e., dense bodies, atrophic and angulated fibers, cell vacuolization, dilation and proliferation of the sarcoplasmic reticulum, mu11ilamllar membranous bodies, atypical mitochondrial clusters and disruptions, mitochondrial inclusions, excessive lipid accumulations, and myofibrillar degeneration. Many of these changes have been reported in human myotonic peripheral musculature. The most susceptible fiber populations in the EOMs were found to be the pale, intermediate, and red singly-innervated fibers of the global region; the pale fibers were the most affected.