Acquired hepatocerebral degeneration

Case contributed by Paul Simkin
Diagnosis almost certain


Increasing confusion and chronic liver disease. History of falls, normal CT brain. Signs of encephalitis? Evidence of injury?

Patient Data

Age: 55 years
Gender: Male

Increased T1 signal is seen within both globus pallidi, extending into the anteromedial cerebral peduncles. The anterior pituitary also shows abnormally high T1 signal. There is increased FLAIR signal in the insular cortex and amygdala bilaterally, the right more so than left, as well as along the corticospinal tracts.

The cortical sulci and ventricles are within normal limits for the patient's stated age, and there is good cerebellar volume, with no evidence of accelerated atrophy. Patchy foci of subcortical T2 hyperintensity in both cerebral hemispheres are keeping with small vessel ischemic change, within normal limits for the patient's age.

No evidence of intracranial hemorrhage or other traumatic sequelae.


Increased basal ganglia T1 signal and increased T2 in the insular cortex and amygdala are in keeping with acquired hepatocerebral degeneration. 

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