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Kearnsov-Sayreov syndróm

  1. Subject h.Kearnsov-Sayreov syndróm
    Subject h.Kearns-Sayre Syndrome
    Entry termsKearnsov syndróm
    Kearns-Sayreov syndróm
    English X referencesKearns Syndrome
    Scope note in EnglishA 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)
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