A Case of chronic acquired hepatocerebral degeneration caused by a porto-systemic shunt by multiple anomalous vessels

ABSTRACT

BACKGROUND AND SIGNIFICANCE: Chronic acquired hepatocerebral degeneration(CAHD) is a heterogenous disorder that can occur with a primary neurologic, hepatic, or combined presentation. Characteristic radiologic finding is high signal in globus pallidus on T1WI MRI, and which is associated with increased level of Manganese. We experienced a patient with CAHD presenting various involuntary movements. Our patient had multiple anomalous vessels with a porto-systemic shunt in abdomen in absence of liver cirrhosis. CASE A 74-years-old female was admitted because of gradually progressive buccal and lingual choreiform movements with moderate generalized chorea. In our patient, T1 weighted MRI of the brain showed symmetric high signal intensity in both globus pallidus and subthalamus. Increased ammonia level(165umol/L) and Manganese level(7.75ug/dl) in whole blood, pancytopopenia in peripheral blood smear and a multiple vessel anomaly with a porto-systemic shunt on abdominal ultrasonography and CT were found. These involuntary movements had a dramatic response to neuroleptics and nearly disappeared within 5 days. CONCLUSION: We report one patient with chronic acquired hepatocerebral degeneration which had a porto-systemic shunt by anomalous vessels and various involuntary movements.