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| Kearns Syndrome Kearn-Sayre Mitochondrial Cytopathy | Kearns' | Syndrome | Kearns-Sayer Syndrome | Kearns-Sayre-Shy-Daroff Syndrome | Cytopathy, Kearn-Sayre Mitochondrial | Kearn Sayre Mitochondrial Cytopathy | Kearn Syndrome | Kearns Sayer Syndrome A mitochondrial disorder featuring the triad of chronic progressive external ophthalmoplegia, cardiomyopathy (with conduction 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) |