MR Imaging of Acquired Hepatocerebral Degeneration

Acquired hepatocerebral degeneration is a well-known irreversible neurologic syndrome that occurs occasionally in patients who have chronic liver disease and portocaval shunting (1, 2]. Affected patients have some combination of coarse postural and action tremor, dyski-nesias of cranial predominance, dysarthria, gait and limb ataxia, and variable impairment of intellectual function (1, 2). Few neuroimaging studies of this condition, which affects the cortex, basal ganglia, and cerebellum, have been performed. We present a patient whose symptomatology correlates with MR abnormalities of the basal gan-glia, a finding not previously described. Case Report A 66-year-old woman had a 3-year history of progressive incapa-citating tremor of the limbs, speech difficulties, and involuntary oro-facial movements. Chronic hepatitis (positive test for hepatitis B surface antigen) had been diagnosed when she was 47 years old. When she was 58, she had her first attack of hepatic encephalopathy. Ten more episodes occurred in the following years before the onset of fixed neurologic symptoms. On repeated examinations, she had a normal mental status, choreic movements of the face and neck, a coarse dysarthria, mild hypokinesia, a large-amplitude postural and action tremor of the limbs, and truncal ataxia. Muscular tone was normal; reflexes were brisk, but plantar responses were flexor. No Kayser-Fleischer rings were present. Liver function tests were altered, but other blood studies, including levels of copper and ceruloplasmin, were normal. CT of the head (Fig. 1A) showed mild cortical atrophy. MR showed abnormal increased signal on T1-weighted images (Fig. 1 B), 575/20/2 (TRfTEfexcitations), in the corpus striatum, mainly in the globus pallidus, and subthalamic region. On T2-weighted images, 2000f40f90, the same area again showed abnormal signal hyperin-tensity relative to the brain on the early-echo sequence (Fig. 1 C) that faded in the later echo (Fig. 1 D). No signal abnormalities were found in the cerebellum