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Kearns Syndrome

2 Diseases & Disorders / 2 Eye Diseases, Hereditary / 2 Retinitis Pigmentosa /
 ► Kearns Syndrome

2 Diseases & Disorders / 2 Ocular Motility Disorders / 2 Ophthalmoplegia /
2 Ophthalmoplegia, Chronic Progressive External / ► Kearns Syndrome

2 Diseases & Disorders / 2 Retinal Diseases / 2 Retinal Degeneration /
2 Retinitis Pigmentosa / ► Kearns Syndrome

 
  MeSH description: 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)
 
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