Acquired hepatocerebral degeneration in a patient with HCV cirrhosis: complete resolution with subsequent recurrence after liver transplantation.
Acquired (non-Wilsonian) hepatocerebral degeneration (AHD) is a chronic brain disorder caused by liver dysfunction and long-standing portal-systemic shunting. It typically presents with dysathria, ataxia, tremor, involuntary movements and altered mental status, and often does not respond to conventional medical therapy for hepatic encephalopathy. There is scarce and conflicting information regarding the clinical course of AHD after liver transplantation (OLT). We present a case of a 47-year-old woman with hepatitis C (HCV) cirrhosis who developed severe manifestations of AHD after multiple bouts of hepatic encephalopathy. Her first OLT was complicated with primary nonfunction requiring immediate retransplantation. The second OLT led to complete clinical and radiological resolution of the AHD. However the patient developed recurrence of AHD 11 months post-transplant due to recurrent HCV and chronic rejection leading to cirrhosis of the graft. The patient developed severe neurological symptoms, despite mild synthetic graft dysfunction. A third OLT led again to disappearance of the clinical and radiological manifestations of AHD. AHD may show complete resolution after OLT; however it may rapidly recur following recurrent liver disease or graft dysfunction.
Servin-Abad
Luis
L
Division of Hepatology, Center for Liver Diseases, Miller School of Medicine, University of Miami, Miami, FL 33136, USA.
Tzakis
Andreas
A
Schiff
Eugene R
ER
Regev
Arie
A
D005260:Female; D016174:Hepacivirus / Q000502:physiology*; D006526:Hepatitis C / Q000150:complications / Q000000981:diagnostic imaging* / Q000601:surgery* / Q000821:virology; D006527:Hepatolenticular Degeneration / Q000000981:diagnostic imaging* / Q000209:etiology / Q000601:surgery* / Q000821:virology; D006801:Humans; D008103:Liver Cirrhosis / Q000000981:diagnostic imaging* / Q000209:etiology / Q000601:surgery* / Q000821:virology; D016031:Liver Transplantation; D008875:Middle Aged; D011877:Radionuclide Imaging; D012008:Recurrence