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Mitochondrial neurogastrointestinal encephalopathy syndrome

Other Names for this Disease
  • MNGIE syndrome
  • Myoneurogastrointestinal encephalopathy syndrome
  • Oculogastrointestinal muscular dystrophy
More Names
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Mitochondrial neurogastrointestinal encephalopathy (MNGIE) syndrome is a condition that particularly affects the digestive system and nervous system. Signs and symptoms of this condition most often begin by age 20 and worsen with time. Almost all people with MNGIE have gastrointestinal dysmotility, in which the muscles and nerves of the digestive system do not move food through the digestive tract efficiently. Affected individuals also experience peripheral neuropathy, droopy eyelids (ptosis), weakness of the muscles that control eye movement (ophthalmoplegia), and hearing loss. Leukoencephalopathy, which is the deterioration of a type of brain tissue known as white matter, is a hallmark of MNGIE; however it does not usually cause symptoms in people with this disorder. Mutations in the TYMP gene cause MNGIE, and this condition is inherited in an autosomal recessive pattern.[1]
Last updated: 12/22/2009


  1. Mitochondrial neurogastrointestinal encephalopathy disease. Genetics Home Reference. June 2008; Accessed 12/22/2009.
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Basic Information

  • Genetics Home Reference (GHR) contains information on Mitochondrial neurogastrointestinal encephalopathy syndrome. This website is maintained by the National Library of Medicine.
  • The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.

In Depth Information

  • The Online Mendelian Inheritance in Man (OMIM) is an catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Mitochondrial neurogastrointestinal encephalopathy syndrome. Click on the link to view a sample search on this topic.