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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 24:390-393, March 2003
© 2003 American Society of Neuroradiology

Case Report
PEDIATRICS

Brain MR Imaging in Acute Hyperammonemic Encephalopathy Arising from Late-Onset Ornithine Transcarbamylase Deficiency

Jun-ichi Takanashi^a, A. James Barkovich^a, Sabrina F. Cheng^b, Dana Kostiner^b, John C. Baker^c and Seymour Packman^b

^a Neuroradiology Section, Department of Radiology, University of California San Francisco
^b Division of Medical Genetics, Department of Pediatrics, University of California San Francisco
^c Department of Medical Genetics, Kaiser Permanente, Oakland, CA

Address correspondence to Jun-ichi Takanashi, MD, Department of Pediatrics, Graduate School of Medicine, Chiba University, 1–8-1 Inohana, Chuo-ku, Chiba-shi, Chiba 260-8677, Japan

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.

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