Acquired hepatocerebral degeneration (AHD) is a rare, underdiagnosed neurological syndrome in patients with chronic liver disease with limited treatment options. It presents with hypo and hyperkinetic movements. Its pathogenesis has been associated with manganese accumulation in the basal ganglia. It is often unnoticed under-diagnosed as a cause of cognitive impairment in patients with liver disease.  

A 54-year-old female with a history of Budd-Chiari syndrome status post-TIPS placement experienced multiple hospitalizations for altered mental status and oral buccal movements, presumably thought to be secondary to hepatic encephalopathy (HE). A CT head was remarkable for a stable subdural hematoma embolized in a prior admission after a fall. Involuntary movements were diagnosed as Tardive Dyskinesia (TD) secondary to SSRI and Metoclopramide, which were discontinued during prior admissions. Four weeks later, the patient presented with worsening involuntary appendicular movements now involving the arms, legs, in addition to other facial muscles. A repeat head CT showed stable subdural hematoma. Patient's exam exhibited nonrhythmic generalized choreiform movements. MRI brain showed extensive periventricular, peritrigonal white matter flair hyperintensities without enhancement, and mineral deposition in the globus pallidus. Low-dose Ativan was trialed, as it is not metabolized by the liver. SSRI was re-started with ongoing depression which were helpful. The patient showed a reduced frequency in hyperkinetic movements, particularly in the arms and legs. VMAT inhibitors and/or anticholinergics were under consideration for future use as needed.

AHD is a heterogeneous syndrome with symptoms that may be attributed to other potential causes. A high index of suspicion for an underlying neurological dysfunction must be maintained when treating cirrhotic patients complicated by multiple episodes of HE. AHD commonly presents with Parkinsonian features; however individuals may exhibit hyperkinetic disorders, such as our patient. Broad differential should be maintained; imaging findings can be helpful & multidisciplinary management will ensure favorable outcomes.