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Case: Hyperammonemia on MRI

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This was a child with primary biliary cirrhosis

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and hepatic failure who presented with obtundation.

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Looking at the FLAIR scan, I must admit that although

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I see some focal areas of high signal intensity in the

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subcortical white matter, I'm not all that impressed

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with anything to account for the patient's obtundation.

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Certainly, with these bright

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areas of high signal intensity.

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This would not be normal for a child,

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and I'd be worried about a vasculopathy.

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This might be a pattern that is seen with

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migraines, but it seems like a lot of injuries.

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I wanna know exactly what you know, whether

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the hepatic disease could be causing some

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sort of vasculopathy associated with it.

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On the diffusion-weighted scan, you notice that there

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is this accentuation once again of the difference

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between the gray matter and the white matter.

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So I'm looking at the DWI saying, is this just a really

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good-looking DWI with good gray-white differentiation?

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One would go back to the FLAIR scan, and you know,

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it does look like there's some thickening

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of the gray matter here, that there may be some

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cerebral gray matter edema that may be pathologic,

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and may account for the patient's obtundation.

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The next step would be to look at the ADC map.

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So this is the ADC map that corresponds

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to the diffusion-weighted imaging.

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And what I see on the ADC map is dark signal intensity

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in the cortex diffusely, which corroborates my concern

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that there is too much hyperintensity to the cortex

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on the DWI image. So remember, this is DWI.

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This is ADC.

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On the ADC image, dark signal is abnormal.

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On the DWI, bright signal is abnormal.

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Here, I'm seeing cortex, which is darker than

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expected, and therefore there is cytotoxic

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edema being demonstrated in this patient.

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This patient's—it’s gonna be seen over here as well

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in the inferior frontal lobe, there's a difference

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in the dark signal of the cortex here versus the

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remainder of the brain, where it's not as dark.

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And that's seen in the parietal regions here,

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and most impressively high up along the vertex.

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This was another patient who had hyperammonemia,

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secondary to primary biliary cirrhosis.

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And the inability to clear the ammonia

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from the blood, leading to cytotoxic edema.

Report

Faculty

David M Yousem, MD, MBA

Professor of Radiology, Vice Chairman and Associate Dean

Johns Hopkins University

Tags

Neuroradiology

Metabolic

MRI

Emergency

Brain

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