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 | |
| Publication status | Published - Feb 2007 |
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Keywords
- Urea cycle disorder
ASJC Scopus subject areas
- Clinical Neurology
- Pediatrics, Perinatology, and Child Health
Cite this
Extensive cortical magnetic resonance signal change in proximal urea cycle disorder. / Bindu, P. S.; Sinha, Ssanjib; Taly, A. B.; Chandrasekhar, H. S.; Christopher, R.; Arunodaya, G. R.; Shetty, T.
In: Journal of Child Neurology, Vol. 22, No. 2, 02.2007, p. 238-239.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Extensive cortical magnetic resonance signal change in proximal urea cycle disorder
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.
PY - 2007/2
Y1 - 2007/2
N2 - 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.
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.
KW - Urea cycle disorder
UR - http://www.scopus.com/inward/record.url?scp=34249862180&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=34249862180&partnerID=8YFLogxK
U2 - 10.1177/0883073807300308
DO - 10.1177/0883073807300308
M3 - Article
C2 - 17621492
AN - SCOPUS:34249862180
VL - 22
SP - 238
EP - 239
JO - Journal of Child Neurology
JF - Journal of Child Neurology
SN - 0883-0738
IS - 2
ER -