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Acute necrotizing encephalopathy of childhood

Disease definition

Acute necrotizing encephalopathy of childhood is 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.

ORPHA:263524

  • 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: -

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