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Nuclear inclusions in innervated cultured muscle fibers from patients with oculopharyngeal muscular dystrophy

Groupe de Recherche de Biologie et Pathologie Neuromusculaire, INSERM U 163. Paris, France (Dr. Tomé)
USC Neuromuscular Centre, Department of Neurology, University of Southern California School of Medicine, Los Angeles, CA (Drs. Askanas and Engel, Ms. Alvarez, and MS. Lee)

We established monolayer muscle fiber cultures from muscle biopsies of 3 patients with oculopharyngeal muscular dystrophy (OPMD) who had Characteristic intranuclear inclusions (INI-A) in their muscle fibers. Aneural cultures had normal morphology, except for a few muscle fibers that contained small vacuoles. Innervated cultures had large cytoplasmic vacuoles in a number of muscle fibers. Those muscle fibers were breaking easily, and could not be maintained longer them 2 months. Electron microscopy showed unusual intranuclear inclusions (INI-B) not previously reported in aneurally cultured muscle fibers of OPMD or in any normal or disease-control aneural or innervated cultured human muscle fibers. They resembled, but were not identical to, the INI-A, and they occurred in both the cultured fibers and the original muscle biopsies of all 3 patients. Our study demonstrates that (1) nuclear inclusions in OPMD reflect an intrinsic genetic defect; and (2) neuronal influence, advanced maturation, or both, seem to be essential for their induction in muscle fibers.

Address correspondence and reprint requests to Dr. Askanas, USC Neuromuscular Center, Hospital of the Good Samaritan, 637 South Lucas Avenue, Los Angeles, CA 90017.

Supported by grants from the Muscular Dystrophy Association and the National Institutes of Health.

Presented in part at the fortieth annual meeting of the American Academy of Neurology, Cincinnati, OH, April 1988.

Received December 9,1988. Accepted for publication in final form February 1,1989.

This article has been cited by other articles:

				O. Hardiman, J. J. Halperin, M. A. Farrell, B. E. Shapiro, S. H. Wray, and R. H. Brown Jr Neuropathic Findings in Oculopharyngeal Muscular Dystrophy: A Report of Seven Cases and a Review of the Literature Arch Neurol, May 1, 1993; 50(5): 481 - 488. [Abstract] [PDF]