Delayed myelopathic presentation of the acquired hepatocerebral degeneration syndrome

Patients with chronic liver disease frequently experience neurologic problems. The most common and widely recognized is the reversible syndrome of hepatic encephalopathy. There are also rare and largely irreversible conditions such as acquired hepatocerebral degeneration (AHCD)1 and hepatic myelopathy (HM).2 We describe a patient who presented with a spastic paraparesis but where investigations suggested that he had HM and AHCD. A 45-year-old man was admitted with a 12-month history of progressive difficulty walking, mild dysarthria, a tremor of his right hand, and impaired short-term memory. Twenty-eight years previously the patient underwent the surgical formation of a porta-caval shunt after the diagnosis of congenital hepatic fibrosis and recurrent esophageal variceal bleeds. For the next 27 years he remained well and had not experienced any episodes of acute hepatic encephalopathy. The patient had hypertension, which was well controlled on atenolol, 100 mg once daily. He admitted to drinking 20 units of alcohol per week, and …