Diseases

Kearns-Sayre Syndrome

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)

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

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)

MeSH Record

Classification

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MeSH Record

Synonyms

42 entry terms
  • CPEO with Myopathy
  • CPEO with Ragged Red Fibers
  • Chronic Progressive External Ophthalmoplegia with Myopathy
  • Cpeo With Ragged-Red Fibers
  • Kearn-Sayre Mitochondrial Cytopathy
  • Kearns Sayre Syndrome
  • Kearns Syndrome
  • Kearns' Syndrome
  • Kearns-Sayre Mitochondrial Cytopathy
  • Kearns-Sayre-Shy-Daroff Syndrome
  • Oculocraniosomatic Syndrome
  • Ophthalmoplegia Plus Syndrome
  • Ophthalmoplegia, Pigmentary Degeneration of Retina, and Cardiomyopathy
  • Ophthalmoplegia, Progressive External, With Ragged-Red Fibers
  • Ophthalmoplegia-Plus Syndrome
  • CPEO with Myopathies
  • Cytopathies, Kearns-Sayre Mitochondrial
  • Cytopathy, Kearn-Sayre Mitochondrial
  • Cytopathy, Kearns-Sayre Mitochondrial
  • Kearn Sayre Mitochondrial Cytopathy
  • Kearn Syndrome
  • Kearns Sayre Mitochondrial Cytopathy
  • Kearns Sayre Shy Daroff Syndrome
  • Kearns-Sayre Mitochondrial Cytopathies
  • Mitochondrial Cytopathies, Kearns-Sayre
  • Mitochondrial Cytopathy, Kearn-Sayre
  • Mitochondrial Cytopathy, Kearns-Sayre
  • Myopathies, CPEO with
  • Myopathy, CPEO with
  • Oculocraniosomatic Syndromes
  • Ophthalmoplegia Plus 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
  • Syndrome, Ophthalmoplegia-Plus
  • Syndromes, Ophthalmoplegia-Plus

MeSH Record

Aspects Covered

34 allowable subheadings

Indexed with the subheadings blood, cerebrospinal fluid, chemically induced, classification, complications, diagnosis, diagnostic imaging, diet therapy, drug therapy, economics, embryology, enzymology, epidemiology, ethnology, etiology, genetics, history, immunology, metabolism, microbiology, mortality, nursing, parasitology, pathology, physiopathology, prevention & control, psychology, radiotherapy, rehabilitation, surgery, therapy, urine, veterinary, virology.

MeSH Record

History Note

2010 (1983)

MeSH Record

Previous Indexing

  • Heart Block (1966-1982)
  • Ophthalmoplegia (1966-1982)
  • Retinitis Pigmentosa (1966-1982)

MeSH Hierarchy

Tree Numbers

AMA Style

References

  1. National Library of Medicine. Kearns-Sayre Syndrome. Medical Subject Headings (MeSH). 2026. Unique ID D007625. http://id.nlm.nih.gov/mesh/2026/D007625
  2. Kearns-Sayre Syndrome. In: Wikipedia. https://en.wikipedia.org/wiki/Kearns%E2%80%93Sayre_syndrome
  3. Kearns-Sayre Syndrome. In: Wikidata. https://www.wikidata.org/wiki/Q2605012