A mitochondrial disorder featuring the triad of chronic progressive EXTERNAL OPHTHALMOPLEGIA, cardiomyopathy (CARDIOMYOPATHIES) with conduction block (HEART BLOCK), and RETINITIS PIGMENTOSA. Disease onset is in the first or second decade. Elevated CSF protein, sensorineural deafness, seizures, and pyramidal signs may also be present. Ragged-red fibers are found on muscle biopsy. (Adams et al., Principles of Neurology, 6th ed, p984)

Synonyms: Kearn Syndrome, Ophthalmoplegia, Pigmentary Degeneration of Retina, and Cardiomyopathy, Kearn Sayre Mitochondrial Cytopathy, Kearns-Sayre-Shy-Daroff Syndrome, Syndrome, Kearns, Ophthalmoplegia-Plus Syndrome, Cytopathy, Kearn-Sayre Mitochondrial, Myopathies, CPEO with, Kearns Sayre Shy Daroff Syndrome, Oculocraniosomatic Syndromes, Kearns-Sayre Syndrome, Kearns Syndrome, Kearns-Sayre Mitochondrial Cytopathy, CPEO with Myopathy, CPEO with Ragged Red Fibers, Syndrome, Oculocraniosomatic, Kearns Sayre Syndrome, Mitochondrial Cytopathy, Kearn-Sayre, Sayre Syndrome, Kearns, Kearns' Syndrome, Syndrome, Ophthalmoplegia Plus, Kearn-Sayre Mitochondrial Cytopathy, Ophthalmoplegia Plus Syndromes, CPEO with Myopathies, Syndrome, Kearns-Sayre-Shy-Daroff, Myopathy, CPEO with, Syndrome, Kearns-Sayre, Syndrome, Kearns Sayre, Ophthalmoplegia Plus Syndrome, Syndrome, Kearns', Chronic Progressive External Ophthalmoplegia with Myopathy, Oculocraniosomatic Syndrome, Cpeo With Ragged-Red Fibers, Ophthalmoplegia, Progressive External, With Ragged-Red Fibers