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

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5 Citations (Scopus)

Abstract

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
Volume22
Issue number2
DOIs
Publication statusPublished - Feb 2007

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Keywords

  • Urea cycle disorder

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health

Cite this

Bindu, P. S., Sinha, S., Taly, A. B., Chandrasekhar, H. S., Christopher, R., Arunodaya, G. R., & Shetty, T. (2007). Extensive cortical magnetic resonance signal change in proximal urea cycle disorder. Journal of Child Neurology, 22(2), 238-239. https://doi.org/10.1177/0883073807300308