RT Journal Article
SR Electronic
T1 Brain MR Imaging in Acute Hyperammonemic Encephalopathy Arising from Late-Onset Ornithine Transcarbamylase Deficiency
JF American Journal of Neuroradiology
JO Am. J. Neuroradiol.
FD American Society of Neuroradiology
SP 390
OP 393
VO 24
IS 3
A1 Takanashi, Jun-ichi
A1 Barkovich, A. James
A1 Cheng, Sabrina F.
A1 Kostiner, Dana
A1 Baker, John C.
A1 Packman, Seymour
YR 2003
UL http://www.ajnr.org/content/24/3/390.abstract
AB Summary: The brain MR imaging characteristics of three patients with acute hyperammonemic encephalopathy resulting from late-onset ornithine transcarbomylase deficiency are presented. MR images revealed injury to the cortex, especially the cingulate gyrus and insular cortex, with sparing of the perirolandic and occipital cortices. These findings presumably reflect the distribution of brain injury from hypoperfusion secondary to hyperammonemia. Knowledge of the MR findings may help expedite diagnosis and treatment and prevent chronic impairment.