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CPEO (Chronic Progressive External Ophthalmoplegia), CPEO with Cardiomyopathy (Kearns-Sayre Syndrome) Print E-mail

Dr. Kennedy CPEO (chronic progressive external ophthalmoplegia.) is a slowly progressive paralysis of the lateral eye muscles.

CPEO with cardio myopathy (progressive weakness of the heart) is known as Kearns-Sayre syndrome: It is a neuromuscular disorder characterized by:

  • Progressive paralysis of the lateral rectus muscles leading to chronic progressive external ophthalmoplegia (an inability to look to the side without turning the head),
  • Abnormal accumulation of colored (pigmented) material on the retina (atypical retinitis pigmentosa), leading to chronic inflammation and progressive degeneration of the retina; and
  • Heart disease (cardiomyopathy) such as cardiac conduction defects and heart block.

Other findings in the syndrome may include:

  • muscle weakness
  • short stature
  • hearing loss
  • loss of ability to coordinate voluntary movements (ataxia) due to problems in the cerebellum

Kearns-Sayre syndrome is one of the mitochondrial encephalomyopathies. These disorders are due to defects in the DNA of the mitochondria, the cell structures that produce energy. These defects cause the brain and muscles to function abnormally (encephalomyopathy). In about 80% of cases of Kearns-Sayre syndrome, tests reveal deletions in mitochondrial DNA (mtDNA).

The information in this article is not meant to be medical advice.�Treatment for a medical condition should come at the recommendation of your personal physician.

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