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Third degree atrioventricular block, chronic progressive external ophthalmoplegia and pigmentary degeneration of retina. Case report and survey of the literature.

A woman was noted to have chronic progressive external ophthalmoplegia, pigmentary degeneration of retina and third degree AV block. She was admitted to hospital because of syncopes and was successfully treated with a permanent pacemaker. Since 1958, 12 similar cases have been noted. Three of 7 patients 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 has been suggested.