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Acute necrotizing encephalopathy of childhood

Disease definition

A rare neurologic disease characterized by a rapid onset of seizures, an altered state of consciousness, neurologic decline, and variable degrees of hepatic dysfunction following a respiratory or gastrointesitnal infection (e.g. mycoplasma, influenza virus) in a previously healthy child. Brain MRI of patients reveals bilateral, multiple, symmetrical lesions predominantly observed in thalami and brainstem, but also in periventricular white matter and cerebellum in some cases.


Classification level: Disorder
  • Synonym(s):
    • ANEC
    • Isolated ANE
    • Isolated acute necrotizing encephalopathy
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: Childhood
  • ICD-10: G31.8
  • OMIM: 614212
  • UMLS: -
  • MeSH: -
  • GARD: -
  • MedDRA: -

Detailed information


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