Retinal degeneration without pigment alterations in progressive external ophthalmoplegia

A 56-year-old black man had annular visual field defects and retinal electrophysiologic dysfunction with chronic progressive external ophthalmoplegia and no pigmentary abnormalities in the fundus. Because the association of retinal disease with neurodegenerative states has generally been based on the observation of pigmentary changes, the patient's fields were erroneously interpreted as the nerve fiber bundle defects of glaucoma.