Abnormalities of aneural and innervated cultured muscle fibers from patients with myotonic atrophy (dystrophy)

T. Kobayashi, V. Askanas, K. Saito, W. K. Engel and K. Ishikawa
Neuromuscular Center, University of Southern California School of Medicine, Los Angeles.

Innervation of human muscle cocultured in monolayer with explants of fetal rat spinal cord plus dorsal-root ganglia produces more mature fibers, which show spontaneous, neurogenic (d-tubocurarine-blocked) contractions. On the innervated myotonic atrophy (MA) muscle fibers, 96% of acetylcholinesterase-stained patches were simple, and only 4% appeared as complicated, pretzel-like, more mature-looking structures; on control innervated fibers, 37% of the acetylcholinesterase patches had the mature appearance. The normal trend from multifocal innervation toward unifocal innervation was decreased in innervated MA muscle fibers. Microelectrode studies compared parameters of cultured aneural muscle fibers and cultured innervated-contracting muscle fibers from 7 patients with MA and 10 control patients. The mean resting membrane potentials of the two groups (aneurally cultured MA muscle fibers and innervated-contracting cultured MA muscle fibers) were 8 and 9 mV lower, respectively, than those of their counterpart controls. The mean amplitude of action potentials, the maximum rate of rise of action potentials in innervated MA muscle fibers, and the action potential amplitude in aneural MA muscle fibers were significantly smaller than in corresponding control fibers.