SHM Converge Scientific Abstract ePoster Gallery-Ahd: Not Your Average Hepatic Encephalopathy

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This case describes a 51-year-old man with NASH cirrhosis and prior hepatic encephalopathy who developed 3 weeks of slowed speech, bradykinesia, confusion, and abnormal involuntary facial movements. Although his mental status improved after treatment with lactulose and rifaximin, his movement disorder and dysarthria persisted. Workup for common metabolic, toxic, nutritional, and structural causes was unrevealing, including normal ammonia, B12, folate, TSH, copper studies, manganese, and urine toxicology. Brain MRI showed bilateral T2/FLAIR abnormalities in the caudate and globus pallidus, and CT abdomen demonstrated small portosystemic shunts. Neurology diagnosed acquired hepatocerebral degeneration (AHD).<br /><br />AHD is a rare complication of chronic liver disease, often associated with prior hepatic encephalopathy and portosystemic shunting. It can present with parkinsonism, ataxia, cognitive slowing, psychiatric symptoms, and sometimes myelopathy. Diagnosis is primarily clinical, supported by characteristic neuroimaging findings, especially basal ganglia changes. Proposed mechanisms include neurotoxicity from manganese and ammonia accumulation with chronic neuroinflammation and neurodegeneration.<br /><br />There is no definitive cure. Treatment is mainly symptomatic, including lactulose and rifaximin for encephalopathy and, depending on the movement disorder, agents such as levodopa or other neurologic medications. In selected cases, shunt obliteration or liver transplantation may improve symptoms and imaging findings. This case highlights the importance of recognizing AHD as an uncommon but important cause of neurologic decline in patients with chronic liver disease to avoid unnecessary testing and to prompt consideration of transplant evaluation.

Asset Subtitle

Evan Raff

Meta Tag

Author List Amy Tierney, Evan Raff, Mukhtar Adem, Spencer Scott

Category Clinical Vignettes

Concept Acquired Hepatocerebral Degeneration

Concept Parkinsonism

Concept Chronic Liver Disease

Concept Portosystemic Shunt

Concept Involuntary Movement

Distinguished Non-Finalist

Presenter Organization University of North Carolina School of Medicine

Presenting Author Evan Raff

Track Adult

Keywords

acquired hepatocerebral degeneration

NASH cirrhosis

hepatic encephalopathy

portosystemic shunt

parkinsonism

basal ganglia MRI

dysarthria

bradykinesia

liver disease

lactulose rifaximin

Acquired Hepatocerebral Degeneration

Parkinsonism

Chronic Liver Disease

Portosystemic Shunt

Involuntary Movement