Hepatic hyperammonemic encephalopathy

Case contributed by Derek Smith
Diagnosis almost certain

Presentation

Known metastatic fibrolamellar HCC. On Sorafenib. Off food and lethargic for a week, vomiting. Now acute agitation with fluctuating LOC. BM 2.3 mmoll-1, responded to simple sugar drink. Deteriorated in ED requiring intubation.

Patient Data

Age: 25 years
Gender: Male

Normal appearances of acute unenhanced CT head. No infarct, bleed or mass. No hyperdense vessels or features of PRES. 

Subtle abnormality best seen on the diffusion sequences - mainly the ADC - with abnormal restriction in the insular cortices and posterior thalami. This can be seen in toxic / metabolic insults.

Case Discussion

This patient who had a complex history of hepatocellular carcinoma and recent short illness developed an acutely altered conscious level and required intubation. The MRI was performed a few days into admission as blood workup was awaited which demonstrated acutely abnormal ALT / AST suggesting hepatocellular damage, as well as an ammonia level of over 200 microg/dL (normal range 0-80).

These findings were sufficient to make a diagnosis of hyperammonemic encephalopathy.

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