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, Kearns Syndrome, Oculocraniosomatic Syndromes, Kearns-Sayre Syndrome, Kearns-Sayre Mitochondrial Cytopathy, CPEO with Ragged Red Fibers, CPEO with Myopathy, 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, Ophthalmoplegia Plus Syndrome, Syndrome, Kearns Sayre, Syndrome, Kearns', Chronic Progressive External Ophthalmoplegia with Myopathy, Oculocraniosomatic Syndrome, Ophthalmoplegia, Progressive External, With Ragged-Red Fibers, Cpeo With Ragged-Red Fibers