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.
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Ann Indian Acad Neurol
January 2025
Department of Neurology, Princess Esra Hospital, Deccan College of Medical Sciences, Hyderabad, Telangana, India.
Cureus
October 2024
Department of Acute Medicine, University Hospitals Plymouth NHS Trust, Plymouth, GBR.
Manganese is an essential trace mineral that has a vital role in maintaining various body functions. Manganese toxicity, referred to as Manganism, causing parkinsonism is a well-known phenomenon that occurs secondary to chronic environmental and occupational exposure to manganese. Patients with underlying chronic liver disease are also susceptible to the toxic accumulation of manganese since it essentially undergoes biliary excretion.
View Article and Find Full Text PDFFront Neurol
September 2024
Department of Neurology, University Medical Centre Ljubljana, Ljubljana, Slovenia.
Rev Esp Enferm Dig
August 2024
Aparato Digestivo, Hospital Universitario Torrecárdenas, España.
Acquired chronic hepatocerebral degeneration (CAHD) is a rare and irreversible neurological disorder that can occur in patients with chronic liver disease. It is characterized by neurological symptoms similar to parkinsonism and the presence of brain damage secondary to manganese deposition. We present the case of a 60-year-old patient with episodes of recurrent hepatic encephalopathy and diagnosis of CAHD.
View Article and Find Full Text PDFJ Clin Exp Hepatol
June 2024
Department of Liver Transplant, Shifa International Hospital, Islamabad, Pakistan.
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