Brain MR Imaging in Acute Hyperammonemic Encephalopathy Arising from Late-Onset Ornithine Transcarbamylase Deficiency
Jun-ichi Takanashia,
A. James Barkovicha,
Sabrina F. Chengb,
Dana Kostinerb,
John C. Bakerc and
Seymour Packmanb
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
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FIG 1. MR image in patient 1 obtained 7 days after the onset of symptoms. Spin-echo echo-planar diffusion-weighted image (TR, 7000, b = 800) reveals reduced diffusion in posterior portion of the left insular cortex (arrow). Standard spin-echo and fluid-attenuated inversion recovery images were normal.
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FIG 2. MR images in patient 2 obtained 2 days after the onset of symptoms.
A and B, Spin-echo images (2500/80/1) at the level of the basal ganglia (A) and centrum semiovale (B) demonstrate extensive swelling and T2 prolongation in the cerebral cortex, especially in the insular cortex and cingulate gyrus. The perirolandic and occipital cortices are spared. The deep gray matter, white matter, and cerebellum are normal.
C, Diffusion-weighted image (TR, 7000, b = 800) shows reduced diffusion in the cerebral cortex, especially in the cingulate gyrus and insular cortex.
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FIG 3. MR imaging in patient 3 obtained 10 days after the onset of symptoms.
A, Spin-echo image (2500/80/1) of the brain demonstrates swelling and T2 prolongation in the cingulate gyri and the right insular cortex (arrowheads).
B, Coronal fluid-attenuated inversion recovery image (10,000/140/2200) image shows hyperintense lesions in the right cingulate gyrus and bilateral insulae (arrowheads), with foci of hyperintensity in the left cerebral white matter.
C, Apparent diffusion coefficient map reveals reduced diffusion in the bilateral cingulate gyri, in the entire right insular cortex, and in the most anterior aspect of the left insular cortex.
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