Cranial MRI in acute hyperammonemic encephalopathy

Pediatr Neurol. 2009 Aug;41(2):139-42. doi: 10.1016/j.pediatrneurol.2009.02.012.


Cranial magnetic resonance imaging was performed in three cases of acute hyperammonemic encephalopathy with three diverse etiologies: infantile citrullinemia, acute hepatic encephalopathy, and proximal urea cycle disorder. All three patients exhibited diffuse extensive cortical signal changes and swelling. Neurologic outcome was poor in all three cases. Knowledge of the magnetic resonance imaging findings of hyperammonemic encephalopathy may help in early diagnosis and treatment and could influence the neurologic outcome.

Publication types

  • Case Reports

MeSH terms

  • Brain / pathology*
  • Brain Diseases, Metabolic / diagnosis*
  • Brain Diseases, Metabolic / etiology
  • Brain Diseases, Metabolic / pathology*
  • Child, Preschool
  • Citrullinemia / complications
  • Diagnosis, Differential
  • Diffusion Magnetic Resonance Imaging
  • Early Diagnosis
  • Female
  • Hepatic Encephalopathy / diagnosis
  • Hepatic Encephalopathy / pathology
  • Humans
  • Hyperammonemia / diagnosis*
  • Hyperammonemia / etiology
  • Hyperammonemia / pathology*
  • Infant
  • Magnetic Resonance Imaging
  • Male