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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 24:1184-1187, June-July 2003
© 2003 American Society of Neuroradiology

Case Report
PEDIATRIC

Brain MR Imaging in Neonatal Hyperammonemic Encephalopathy Resulting from Proximal Urea Cycle Disorders

Jun-ichi Takanashi^a, A. James Barkovich^a, Sabrina F. Cheng^b, Kara Weisiger^b, Carol O. Zlatunich^b, Christine Mudge^c, Philip Rosenthal^c, Mendel Tuchman^d and Seymour Packman^b

^a Neuroradiology Section, Department of Radiology, University of California San Francisco
^b Division of Medical Genetics, University of California San Francisco
^c the Liver Transplant Program, University of California San Francisco
^d Department of Pediatrics, University of California San Francisco; and the Children’s Research Institute, Children’s National Medical Center, George Washington University, Washington DC

Address reprint requests 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: We present brain MR images in three patients with neonatal-onset hyperammonemic encephalopathy resulting from urea-cycle disorders (two sisters with deficiency of the carbamyl phosphate synthetase I reaction step and one boy with an ornithine transcarbamylase deficiency). MR imaging revealed almost identical findings of injury to the bilateral lentiform nuclei and the deep sulci of the insular and perirolandic regions; to our knowledge, this pattern has not been previously reported. We hypothesize that these lesions presumably reflect the distribution of brain injury due to hypoperfusion secondary to hyperammonemia and hyperglutaminemia in the neonatal period.

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