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Kearns-Sayre 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)

Synonym(s): Kearns Syndrome / Kearn-Sayre Mitochondrial Cytopathy / Kearns Sayre Syndrome / Kearns' Syndrome / Kearns-Sayre-Shy-Daroff Syndrome / Oculocraniosomatic Syndrome / Ophthalmoplegia Plus Syndrome / Ophthalmoplegia, Pigmentary Degeneration of Retina, and Cardiomyopathy / Cytopathy, Kearn-Sayre Mitochondrial / Kearn Sayre Mitochondrial Cytopathy / Kearn Syndrome / Kearns Sayre Shy Daroff Syndrome / Mitochondrial Cytopathy, Kearn-Sayre / Oculocraniosomatic Syndromes / Ophthalmoplegia Plus Syndromes / Sayre Syndrome, Kearns / Syndrome, Kearns / Syndrome, Kearns Sayre / Syndrome, Kearns' / Syndrome, Kearns-Sayre / Syndrome, Kearns-Sayre-Shy-Daroff / Syndrome, Oculocraniosomatic / Syndrome, Ophthalmoplegia Plus
MeSH 2010 © U.S. National Library of Medicine®.
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  Web resources for "Kearns-Sayre Syndrome"
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Broader term(s): - Ophthalmoplegia, Chronic Progressive External - Mitochondrial Myopathies - Muscular Diseases - Musculoskeletal Diseases - Retinitis Pigmentosa - Eye Diseases - Cardiomyopathies - Heart Diseases - Cardiovascular Diseases

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  Medical image(s) for "Kearns-Sayre Syndrome"
click below to enlargePtosis de la maladie de Kearns-Sayre
www.snof.org
Syndicat National des Ophtalmologistes de France Ptosis de la maladie de Kearns-Sayre
Copyright © Pr Alain Bron (CHU Dijon France)
Broader term(s): - Retinitis Pigmentosa - Heart Diseases
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  Medical News for "Kearns-Sayre Syndrome"
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Broader term(s): - Muscular Diseases - Eye Diseases

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  Scientific articles from MEDLINE for "Kearns-Sayre Syndrome"
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Subheadings: complications / diagnosis / embryology / epidemiology / etiology / metabolism / microbiology / surgery / therapy

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Broader term(s): - Cardiovascular Diseases

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Subheadings: complications / diagnosis / embryology / epidemiology / etiology / metabolism / microbiology / surgery / therapy


  Broader term(s):
      Diseases
          Nutritional and Metabolic Diseases
              Metabolic Diseases
                  Mitochondrial Diseases
                      Mitochondrial Myopathies
                          Ophthalmoplegia, Chronic Progressive External 
      Diseases
          Cardiovascular Diseases
              Heart Diseases
                  Cardiomyopathies 
      Diseases
          Eye Diseases
              Retinal Diseases
                  Retinal Degeneration
                      Retinitis Pigmentosa 
              Ocular Motility Disorders
                  Ophthalmoplegia, Chronic Progressive External 
      Diseases
          Nervous System Diseases
              Neuromuscular Diseases
                  Muscular Diseases
                      Mitochondrial Myopathies
                          Ophthalmoplegia, Chronic Progressive External 
              Cranial Nerve Diseases
                  Ocular Motility Disorders
                      Ophthalmoplegia, Chronic Progressive External 
      Diseases
          Musculoskeletal Diseases
              Muscular Diseases
                  Mitochondrial Myopathies
                      Ophthalmoplegia, Chronic Progressive External 

MeSH 2010 © U.S. National Library of Medicine®.

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