Extensive cortical magnetic resonance signal change in proximal urea cycle disorder

P. S. Bindu, Ssanjib Sinha*, A. B. Taly, H. S. Chandrasekhar, R. Christopher, G. R. Arunodaya, T. Shetty

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

6 Citations (Scopus)


The authors report a 3-year 8-month-old girl presenting with episodic hyperammonemic encephalopathy probably due to a proximal urea cycle disorder. The magnetic resonance imaging (MRI) of the brain performed during the third episode revealed extensive and diffuse cerebral cortical signal changes with sparing of occipital cortex. It is believed that intracerebral accumulation of glutamine mainly in astrocytes is the major cause of the encephalopathy. This results in astrocyte swelling, brain edema, intracranial hypertension, and cerebral hypoperfusion.

Original languageEnglish
Pages (from-to)238-239
Number of pages2
JournalJournal of Child Neurology
Issue number2
Publication statusPublished - Feb 2007
Externally publishedYes


  • Urea cycle disorder

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology


Dive into the research topics of 'Extensive cortical magnetic resonance signal change in proximal urea cycle disorder'. Together they form a unique fingerprint.

Cite this