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

doi:10.1177/0883073807300308

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

Medicine

Research output: Contribution to journal › Article › peer-review

6 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 language	English
Pages (from-to)	238-239
Number of pages	2
Journal	Journal of Child Neurology
Volume	22
Issue number	2
DOIs	https://doi.org/10.1177/0883073807300308
Publication status	Published - Feb 2007

Keywords

Urea cycle disorder

ASJC Scopus subject areas

Clinical Neurology
Pediatrics, Perinatology, and Child Health

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AU - Bindu, P. S.

AU - Sinha, Ssanjib

AU - Taly, A. B.

AU - Chandrasekhar, H. S.

AU - Christopher, R.

AU - Arunodaya, G. R.

AU - Shetty, T.

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AB - 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.

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