Third Degree Atrioventricular Block, Chronic Progressive External Ophthalmoplegia and Pigmentary Degeneration of Retina

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Abstract
A woman was noted to have chronic progressive external ophthalmoplegia, pigmentary degeneration of retina and 3rd degree AV [atrioventricular] block. She was admitted to hospital because of syncopes and was successfully treated with a permanent pacemaker. Since 1958, 12 similar cases were noted. Of 7 patients, 3 without pacemaker treatment died, and 5 were successfully treated with pacemaker. The disturbances in AV conduction are not thought to be a mere coincidence to the ocular disorder. Cardiomyopathy was suggested.