Kearns Sayre Syndrome

It is feasible that the primary title of the report Kearns Sayre Disorder is not the name you expected. Kindly inspect the synonyms listing to discover the alternating name(s) as well as condition class(s) covered by this record.


  • KSS
  • persistent progressive external ophthalmoplegia and myopathy
  • chronic modern outside ophthalmoplegia with ragged red fibers
  • CPEO with myopathy
  • CPEO with ragged red fibers
  • mitochondrial cytopathy
  • occulocraniosomatic syndrome (out-of-date)
  • ophthalmoplegia, pigmentary weakening of the retina and also cadiomyopathy
  • ophthalmoplegia plus syndrome

Condition Neighborhoods

  • None

General Conversation
Kearns-Sayre syndrome (KSS) is an unusual neuromuscular problem characterized by three primary findings: dynamic paralysis of particular eye muscle mass (persistent progressive exterior ophthalmoplegia [CPEO]; uncommon buildup of colored (pigmented) material on the nerve-rich membrane layer lining the eyes (irregular retinitis pigmentosa), bring about persistent swelling, progressive degeneration, and also wearing away of particular eye structures (pigmentary deterioration of the retina); and also heart disease (cardiomyopathy) such as heart block. Various other findings may consist of muscle weakness, short stature, hearing loss, and/or the loss of capability to collaborate volunteer motions (ataxia) due to problems affecting component of the human brain (cerebellum). In many cases, KSS may be associated with other disorders and/or conditions.

KSS belongs (partially) to a group of unusual neuromuscular conditions referred to as mitochondrial encephalomyopathies. Mitochondrial encephalomyopathies are problems in which a problem in hereditary material occurs from a component of the cell framework that launches power (mitochondria), causing the mind as well as muscle mass to operate poorly (encephalomyopathies). In these disorders, unusually high varieties of faulty mitochondria are present. In about 80 percent of situations of KSS, examinations will reveal missing genetic material (removal) entailing the distinct DNA in mitochondria (mtDNA).

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