Chronic progressive external ophthalmoplegia, pigmentary retinopathy, and heart block (Kearns-Sayre syndrome). Report of a case.
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, annd 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.
D000293:Adolescent; D000328:Adult; D003623:Dark Adaptation; D004596:Electroretinography; D005260:Female; D005451:Fluorescein Angiography; D006327:Heart Block / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009886:Ophthalmoplegia / Q000150:complications / Q000175:diagnosis*; D009887:Ophthalmoscopy; D011859:Radiography; D012171:Retinal Vessels / Q000000981:diagnostic imaging; D012174:Retinitis Pigmentosa / Q000150:complications / Q000175:diagnosis*; D014792:Visual Acuity; D014794:Visual Fields