A Case of Acquired Hepatocerebral Degeneration

Kim, Sung Soo; Lee, Chang Don; Kim, Jae Young; Kim, Soung Kyun; Hang, Sang Won; Yoon, Seung Kew; Choi, Sang Wook; Park, Doo Ho; Kim, Boo Sung; Kim, Yeong In; Lee, Kwang Soo

Abstract

The acquired hepatocerebral degeneration is a irreversible neurologic syndrome that occurs occasionally in patients who have chronic liver disease and portasystemic shunting. Clinically, the patients present with dementia, dysarthria, ataxia, tremor and extrapyramidal symptoms that must be distinguished from the symptorns of the patients with hepatic cirrhosis complicating hepatic encephalopathy. Tl-weighted images of MR in patients with hepatocerebral degeneration demonstrate characteristic increased signal intensity in the basal ganglia, pituitary gland, and mesencephalon, while T2-weighted images demonstrated no corresponding alteration in signal intensity. It is known that the symptoms of acquired hepatocerebral degenerafion is not irnproved by the conventional therapy for the hepatic encephalopathy such as hypertonic glucose enema or lactulose administration. Recently, we experienced a case of acquired hepatocerebral degeneration diagnosed by brain MRI. A 54-year-old man who had been diagnosed and treated as hepatic cirrhosis for 5 years at our hospital have complained stuporous mental status, dysarthria, fine tremor, and motor disturbance. Under the impression of hepatic encephalopathy, we treated this patient with lactulose and hypertonic glucose enema.. There was some clinical improvement in mental status, but the symptoms such as dysarthria, tremor, and motor disturbance were not changed during hospitalization. Tl-weighted images of MR demonstrate characteristic increased signal intensity in the basal ganglia, while T2-weighted images demonstrated no corresponding alteration in signal intensity.