Canadian Medical Guide > Diseases > Musculoskeletal Diseases > Muscular Diseases > Mitochondrial Myopathies > Ophthalmoplegia, Chronic Progressive External > Kearns Syndrome Terms and Definitions




Kearns Syndrome

Medical Definition: 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)
Previously Indexed: Heart Block (1966-1982),Ophthalmoplegia (1966-1982),Retinitis Pigmentosa (1966-1982)

Kearns Syndrome Medical Definitions and Terms

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