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
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.