Acquired hepatocerebral degeneration

Acquired (non-Wilsonian) hepatocerebral degeneration is a rare irreversible neurological syndrome that occurs in patients with chronic liver disease associated with multiple metabolic insults. Van Woerkom was the first to describe acquired hepatocerebral degeneration in 1914 followed by the landmark article by Victor et al in 1965. Multiple bouts of hepatic coma are the only known risk factors that trigger this devastating neurodegenerative disease with features suggesting toxic exposure to the brain. Clinically and pathologically the disorder is similar to Wilson's disease although subtle differences in immunostaining of glial fibrillar acidic protein have been documented. Acquired hepatocerebral degeneration occurs in 0.8-2% of patients with cirrhosis. As acquired hepatocerebral degeneration is relatively rare, we are reporting one such case from our hospital in a 50-year-old male patient who had long-standing portal systemic shunt and presented with progressive cognitive decline, bradykinesia, tremors and bilateral extrapyramidal signs.