CHRONIC PROGRESSIVE EXTERNAL OPHTHALMOPLEGIA, PIGMENTARY RETINOPATHY, AND HEART BLOCK (KEARNS‐SAYRE SYNDROME)

Abstract

Chronic progressive external ophthalmoplegia associated with a pigmentary retinopathy and heart block was described by Kearns & Sayre in 1958. They suggested that the triad represented a syndrome. The cases recorded since then are reviewed and summarised, and several common features are noted. Little attention has previously been paid to the retinopathy. A similar case is presented, and the pigment changes, retinal function, and fluorescein angiography are examined. There is no clinical evidence for the diagnosis of classical retinitis pigmentosa. The prognosis and aetiology of the condition are discussed.