Kearns-Sayre syndrome. A review of a multisystem disorder of children and young adults.

Abstract

The syndrome of a slowly progressive external ophthalmoplegia, pigment retinopathy, and disorder of cardiac conduction was described by Kearns and Sayre in 1958. In patients with this triad, other neurological deficits may occur with associated abnormalities of the electrocardiogram, electroencephalogram, audiogram, and an elevation of protein in cerebrospinal fluid. The onset of a potentially lethal cardiac dysrhythmia in a patient with this slowly progressive degeneration of the nervous system can be anticipated and prevented by the use of an artivicial cardiac pacemaker.