Acquired hepatocerebral degeneration without overt liver disease

Abstract

Acquired hepatocerebral degeneration (AHCD) is usually described in patients with chronic liver diseases with portal-systemic shunting and previous episodes of acute hepatic encephalopathy. We describe an atypical case with no overt evidence of hepatic disease in which the characteristic neuroradiologic findings first called attention to the diagnosis. A 45-year-old man had progressive quadriparesis, dysphagia, and dysphonia for 18 months. He then developed back pain, urinary retention, and weight loss. There was no history of alcoholism or electrolyte disturbances. The general physical examination was unremarkable. The neurologic examination disclosed mental slowing, asymmetric spastic quadriparesis predominating in the lower limbs, mild arm ataxia, dysarthria, and left palate paresis. EMG, CSF, and slitlamp examination were normal. Cranial CT showed moderate corticosubcortical atrophy; MRI revealed symmetric focal lesions in the cerebellar peduncles and white matter (figure, A through D). The patient developed recurrent fever from Staphylococcus aureus infection of undetermined origin. Liver enzymes were normal (alanine aminotransferase, 38 U/L; aspartate aminotransferase, 41 U/L), and ammonia level was not measured. IV …