Kearns-Sayer Syndrome

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Is a:
  • Disease or Syndrome
Definition:
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:
  • Cytopathy, Kearn-Sayre Mitochondrial
  • Kearn Sayre Mitochondrial Cytopathy
  • Kearn Syndrome
  • Kearn-Sayre Mitochondrial Cytopathy
  • Kearns Sayer Syndrome
  • Kearns Sayre Shy Daroff Syndrome
  • Kearns Syndrome
  • Kearns' Syndrome
  • Kearns-Sayre-Shy-Daroff Syndrome
  • Mitochondrial Cytopathy, Kearn-Sayre
  • Syndrome, Kearns
  • Syndrome, Kearns'
  • Syndrome, Kearns-Sayer
  • Syndrome, Kearns-Sayre-Shy-Daroff
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Narrower Topics:
  • None
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