The patient came to our observation in April 1992 when he was 44 years old. He had a history of chronic hepatic disease, which had begun some years before. At 42 years, he had noticed a subtle onset of slowness, mental impairment, and bilateral hand tremor, with a gradual progressive course and episodic worsening related to temporary liver insufficiency. He had no family members with neurological problems; his mother had had an undiagnosed liver disease. When seen, he was recovering from a hepatic decompensation, with still-elevated ammonia serum levels (199 µmol/L). He was alert, oriented, and cooperative, but with a slight slowness of psychomotor speed. The neurological examination showed bradyarthria and dysarthria, bilateral hand postural tremor that was more severe on the left side, head and left arm dystonic movements, and mild ataxia. Mild asterixis was superimposed on the tremor. An electroencephalogram showed a bilateral posterior activity of 7 Hz, without focal abnormalities. After therapy with lactulose, ammonia levels decreased, and his general condition improved, asterixis disappeared, and no other remarkable changes were seen in neurological status. Neuropsychological examination showed a full-scale IQ of 62 (verbal, 70; performance, 55) and a moderate impairment of information processing control (attention, vigilance, psychomotor speed, intelligence) and verbal and visuospatial abilities (Table 1). Memory was grossly preserved. T2-weighted magnetic resonance imaging (MRI) revealed bilateral lucencies on lentiform nuclei (Figure 1). Kayser-Fleischer (K-F) rings and an initial sunflower cataract were found by slitlamp examination. Laboratory tests showed a low plasma copper level (8.3 µmol/L) and high urinary copper level (61 µg/d). Wilson disease was diagnosed and treatment with penicillamine, 600 mg/d, was given, with initial improvement of motor impairment. After 6 months, his neurological status and liver function deteriorated rapidly. At this time, the patient exhibited a severe enhancement of the motor impairment, especially tremor and dysarthria, and a reduced psychomotor speed. Liver transplantation was performed, with a successful course and a rapid copper balance normalization. One month after LT, only a mild postural hand tremor persisted. Findings on cerebral MRI at this time were unchanged. The electroencephalogram was normal. Nine months later, results of the neurological examination were normal, K-F rings disappeared, and MRI showed attenuation of the basal ganglia lucencies, which were no longer present on a fourth MRI examination 18 months after orthotopic LT (Figure 2). Neuropsychological testing 22 months after LT showed a full-scale IQ of 71 (verbal, 76; performance, 69), with a slight amelioration of the performances on information processing control tasks and of some instrumental abilities (especially visuospatial) (Table 1). No other neurological problems have emerged during 5 years of follow-up. Table 2 summarizes the data of the longitudinal neurological examination, expressed by means of a severity index of 4 domains (tremor, dystonia, ataxia, and dysarthria), showing a dramatic improvement of the neurological symptoms after LT.