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Familial progressive external ophthalmoplegia and ragged-red fibers

Departments of Neurology and Pediatrics, University of Rochester School of Medicine and Dentistry, Rochester, New York.

"Ragged-red fibers" have been found in limb muscles of patients with progressive ptosis and external ophthalmoplegia, but despite the frequent familial nature of the disease, none of the cases with ragged-red fibers have been noted to be familial. We are reporting a family with progressive external ophthalmoplegia and ragged red fibers in which the disease occurred in four generations and in which biopsy of two patients demonstrated numerous ragged-red fibers in skeletal muscle.

This study was supported in part by grants from the Muscular Dystrophy Associations of America, Inc., USPHS grant RR00044, and neurology training grant 5 TO1 NS–05084–13 NSRB. Dr. Griggs is an American College of Physicians Teaching and Research Scholar.

Reprint requests and correspondence should be addressed to Dr. lannac-cone at Department of Neurology, Strong Memorial Hospital, 260 Critten-den Blvd., Rochester, NY 14642.

Received for publication June 7, 1974.

This article has been cited by other articles:

				J. E. Riggs, S. S. Schochet Jr., G. R. Goldfarb, and R. A. Price Congenital Myopathy With Progressive External Ophthalmoplegia J Child Neurol, July 1, 1989; 4(3): 195 - 197. [Abstract] [PDF]