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November 1959

Progressive Dystrophic External Ophthalmoplegia with Abiotrophic Fundus Changes

Author Affiliations

Iowa City
From the Departments of Ophthalmology and Neurology, State University of Iowa College of Medicine, and the Divisions of Ophthalmology and Neurology, Veterans Administration Hospital.

AMA Arch Ophthalmol. 1959;62(5):833-838. doi:10.1001/archopht.1959.04220050095015

The association of progressive external ophthalmoplegia and retinal degeneration, or "retinitis pigmentosa," has been noted in 13 previously reported cases.1-4 This combination of ocular signs has been regarded as a syndrome of disease.1-3 Recently a report of two patients with progressive paralysis of ocular movement with ptosis, retinal degeneration, and heart block was held to establish still a new syndrome.5

We have recently studied a patient who manifests these abnormalities, and we believe that the combination of symptoms is more properly regarded as a coincidence of abiotrophic manifestations rather than as a unique syndrome.5

Progressive external ophthalmoplegia, although an uncommon disorder, has been a recognized clinical entity since von Graefe's original description.6 The condition was usually regarded as secondary to nuclear degeneration7,8 until recent definitive studies of the ocular muscles established primary myopathy as the cause of paralysis in most cases.9-13 The ocular myopathy