A Case of Acquired Hepatocerebral Degeneration Presenting as Hemichorea Post-Liver Transplant

Abstract

Acquired hepatocerebral degeneration (AHCD) is a rare but morbid neurological manifestation of decompensated cirrhosis. Portosystemic shunting leads to manganese deposition in the basal ganglion which is identified as bilateral hyperintense signals on T1-weighted MRI. The resulting neuronal loss leads to involuntary movements, cognitive dysfunction, Parkinsonism, or cerebellar ataxia in the absence of hepatic encephalopathy. We present a case of AHCD with several unique features: 1) initial presentation post-liver transplant (OLT), 2) no clinical or radiographical evidence of portosystemic shunting, and 3) unilateral basal ganglia hyperintensity. A 57-year-old man with hypertension and chronic kidney disease (stage III) underwent OLT for hepatitis C and alcoholic cirrhosis 3 years prior to presentation. His post-transplant course was complicated by continued alcohol abuse, steroid-induced hyperglycemia, and acute cellular rejection without fibrosis 4 months prior. Prescriptions included tacrolimus, mycophenolate mofetil, prednisone, nifedipine, and insulin with questionable adherence. He had no personal or family history of movement disorders. His chief complaint was one month of progressive, uncontrollable right-sided arm and leg movements in a writhing, non-rhythmic pattern. He was afebrile. Physical exam was notable for right-sided hemichorea with normal strength, finger-to-nose test, reflexes, and sensation. He displayed no altered-mental status, asterixis, nystagmus, hepatosplenomegaly or ascites. Labs were notable for total bilirubin 1.3 mg/dL, Alk Phos 43 U/L, AST/ALT 117/65 U/L, blood alchohol level 103 mg/dL. INR 1.0 and platelets 141 x103/μL. Electrolytes, glucose and creatinine were normal. HgbA1c 6.0%. Tacrolimus level undetectable. Serum iron, zinc, lead, and manganese levels were normal. MRI brain notable for T1 hyperintensities in the left globus pallidus without evidence of hemorrhage or acute ischemia (Figure 1). He was treated with haloperidol 1mg twice daily which mildly suppressed the hemichorea and allowed discharge with close neurology follow-up. Symptoms of ACHD often improve with resolution of portosystemic shunting post-OLT. However, we present a case of new onset ACHD in a post-transplant patient without evidence of portosystemic shunting. This illustrates that ACHD remains a poorly understood disorder that should be considered in patients with typical findings and a history of cirrhosis, even after liver transplantation.Figure: T1 weighted magnetic resonance imaging (MRI) of the brain without contrast in axial view (A) and sagittal view (B) of hyperintensity in the left globus without evidence of hemorrhage or acute ischemia.