Progressive External Ophthalmoplegia and Benign Retinal Pigmentation

Abstract

A 17-year-old boy had progressive external ophthalmoplegia, normal visual acuity, and a pigmentary retinopathy. Neurologic evaluation showed an abnormal electroencephalogram with diffuse slow (theta) waves. Myasthenia gravis was excluded. Fluorescein angiography showed a normal vascular pattern with diffuse hyper- and hypopigmentation. Results of retinal testing, including color vision, visual fields, electroretinography, and dark adaptometry, were within normal limits except for a slightly subnormal electroretinogram with normal implicit times. Progressive external ophthalmoplegia may be associated with either progressive or non-progressive pigmentary retinopathies, and adequate evaluation of retinal function in these cases must be obtained.