Orphanet: Leigh syndrome

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Leigh syndrome

Disease definition

A progressive neurological disease defined by specific neuropathological features associating brainstem and basal ganglia lesions.


Classification level: Group of disorders
  • Synonym(s):
    • Infantile subacute necrotizing encephalopathy
    • Leigh disease
  • Prevalence: 1-9 / 100 000
  • Inheritance: Autosomal recessive or X-linked recessive or Mitochondrial inheritance 
  • Age of onset: All ages
  • ICD-10: G31.8
  • OMIM: 256000
  • UMLS: C0023264  C0751267
  • MeSH: D007888
  • GARD: 6877
  • MedDRA: 10062950

Detailed information

Article for general public


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