Orphanet: Mitochondrial neurogastrointestinal encephalomyopathy

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Mitochondrial neurogastrointestinal encephalomyopathy

Disease definition

Mitochondrial NeuroGastroIntestinal Encephalomyopathy (MNGIE) syndrome is characterized by the association of gastrointestinal dysmotility, peripheral neuropathy, chronic progressive external ophthalmoplegia and leukoencephalopathy.


Classification level: Disorder
  • Synonym(s):
    • MNGIE
  • Prevalence: 1-9 / 1 000 000
  • Inheritance: Autosomal recessive 
  • Age of onset: Adolescent, Childhood, Adult
  • ICD-10: G71.3
  • OMIM: 603041  612075  613662
  • UMLS: C0872218
  • MeSH: -
  • GARD: 9920
  • MedDRA: -

Detailed information


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