Familial progressive external ophthalmoplegia and ragged-red fibers

“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.