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