WE PRESENT THE HISTOPATHOLOGIC AND ELECTRON MICROSCOPIC FINDINGS IN two post-mortem eyes from a man who had Kearns-Sayre syndrome (total external ophthalmoplegia, heart block, and atypical pigmentary retinopa-thy" 2). The nature and pattern of the outer retinal and retinal pigment epithelial abnormalities in this case suggest that the primary defect may reside in theretinal pigment epithelium.

CASE REPORT A 52-year-old white man was transferred to the Pennsylvania Hospital on August 10th, 1979 for neurological evaluation of progressive weakness and dysphagia. Ventricular tachycardia necessitated admission to another hospital one week previously. At age 7 the patient developed ptosis and hearing loss, following an auto accident. At age 8" neuromuscular dystrophy" was diagnosed during a neurologi-cal evaluation at Jefferson University Hospital. Dysarthria, progressive muscle weakness and difficulty swallowing occurred during the next decade. Visual loss developed gradually andwas bilateral. Five years prior to admission the patient was barely able to walk down stairs and could read only large print. During the two years prior to admission, the patient experienced several episodes of syncope secondary to a rapid ventricular arrhythmia. Increasing dysphagia led to weight loss due to inadequate food intake and several bouts of aspiration pneumonitis.