2017 Articles

Recent Updates on Acquired Hepatocerebral Degeneration

Shin, Hae-Won; Park, Hee Kyung

Background: Acquired hepatocerebral degeneration (AHD) refers to a chronic neurological syndrome in patients with advanced hepatobiliary diseases. This comprehensive review focuses on the pathomechanism and neuroimaging findings in AHD.

Methods: A PubMed search was performed using the terms “acquired hepatocerebral degeneration,” “chronic hepatocerebral degeneration,” “Non-Wilsonian hepatocerebral degeneration,” “cirrhosis-related parkinsonism,” and “manganese and liver disease.”

Results: Multiple mechanisms involving the accumulation of toxic substances such as ammonia or manganese and neuroinflammation may lead to widespread neurodegeneration in AHD. Clinical characteristics include movement disorders, mainly parkinsonism and ataxia-plus syndrome, as well as cognitive impairment with psychiatric features. Neuroimaging studies of AHD with parkinsonism show hyperintensity in the bilateral globus pallidus on T1-weighted magnetic resonance images, whereas molecular imaging of the presynaptic dopaminergic system shows variable findings. Ataxia-plus syndrome in AHD may demonstrate high-signal lesions in the middle cerebellar peduncles on T2-weighted images.

Discussion: Future studies are needed to elucidate the exact pathomechanism and neuroimaging findings of this heterogeneous syndrome.