OBJECTIVES: Acquired (non-Wilsonian) hepato-cerebral degeneration is an infrequent neurologic disorder in patients with liver dysfunction and longstanding portal-systemic shunting. The clinical manifestations include dysarthria, ataxia, tremor, and cognitive dysfunction. Typically, patients with acquired hepatocerebral degeneration respond poorly to medical therapy as the underlying end-stage liver disease remains. Information regarding the effect of orthotopic liver transplant on acquired hepatocerebral degeneration, however, is limited and conflicting. MATERIALS AND METHODS: We conducted a review of literature via a PubMed search to summarize the effect of orthotopic liver transplant on acquired hepatocerebral degeneration. RESULTS: We present a 56-year-old man with compensated hepatitis C cirrhosis who developed acquired hepatocerebral degeneration with Parkinsonian symptoms refractory to conventional Parkinson medical therapy. Orthotopic liver transplant led to marked clinical improvement of the acquired hepatocerebral degeneration. However, the patient developed recurrence of acquired hepatocerebral degeneration 6-week postorthotopic liver transplant as he developed graft failure from aggressive progressive hepatitis C recurrence. Our review found a heterogeneous group of case series, suggesting that the experience with orthotopic liver transplant is variable. CONCLUSIONS: Our experience demonstrates that orthotopic liver transplant may lead to resolution of acquired hepatocerebral degeneration; however, acquired hepatocerebral degeneration may return with recurrent liver disease. Future studies with long-term follow-up are needed.
OBJECTIVES: Acquired (non-Wilsonian) hepato-cerebral degeneration is an infrequent neurologic disorder in patients with liver dysfunction and longstanding portal-systemic shunting. The clinical manifestations include dysarthria, ataxia, tremor, and cognitive dysfunction. Typically, patients with acquired hepatocerebral degeneration respond poorly to medical therapy as the underlying end-stage liver disease remains. Information regarding the effect of orthotopic liver transplant on acquired hepatocerebral degeneration, however, is limited and conflicting. MATERIALS AND METHODS: We conducted a review of literature via a PubMed search to summarize the effect of orthotopic liver transplant on acquired hepatocerebral degeneration. RESULTS: We present a 56-year-old man with compensated hepatitis C cirrhosis who developed acquired hepatocerebral degeneration with Parkinsonian symptoms refractory to conventional Parkinson medical therapy. Orthotopic liver transplant led to marked clinical improvement of the acquired hepatocerebral degeneration. However, the patient developed recurrence of acquired hepatocerebral degeneration 6-week postorthotopic liver transplant as he developed graft failure from aggressive progressive hepatitis C recurrence. Our review found a heterogeneous group of case series, suggesting that the experience with orthotopic liver transplant is variable. CONCLUSIONS: Our experience demonstrates that orthotopic liver transplant may lead to resolution of acquired hepatocerebral degeneration; however, acquired hepatocerebral degeneration may return with recurrent liver disease. Future studies with long-term follow-up are needed.