Cheon et al. Table 3. Association of Liver MR Imaging and Age at MR Examination Table 4. Association of Liver MR Imaging and various Clinical Manifestations Liver MR Findings Intrahepatic nodule Hypointense nodules on T2WI Hyperintense nodules on T1WI Hepatic Contour Surface nodularity Caudate hypertrophy Gallbladder fossa widening Others Ascites Venous collaterals Gallbladder wall edema Splenomegaly Mean Age (± Standard Deviation) Positive Negative P value 15.8 ± 4.6 11.9 ± 5.8 16.1 ± 5.4 12.9 ± 5.4 16.4 ± 4.8 11.2 ± 5.1 19.5 ± 3.8 12.4 ± 5.0 16.2 ± 4.6 10.6 ± 5.1 20.3 ± 6.7 12.8 ± 4.6 18.6 ± 6.4 12.7 ± 4.9 17.6 ± 6.4 12.6 ± 4.7 0.001 0.113 < 0.001 < 0.001 0.001 0.006 0.013 0.025 15.2 ± 5.1 08.4 ± 3.8 < 0.001 Macronodular lesions were observed in six patients (67%) (Fig. 4). Each MR imaging finding demonstrated a statisti- cally significant difference among the three groups of Child-Pugh classification except for splenomegaly (p = 0.243). A summary of the MR findings and association of MR findings with the severity of hepatic dysfunction are listed in Table 2. For each MR imaging finding, mean age at MR examination was greater in patients with a positive finding than those with negative findings, and were statisti- cally significant except in hyperintense nodules on T1- weighted images (p = 0.113) (Table 3). In the patients belonging to Child-Pugh class A (n = 36), MR findings such as intrahepatic nodules (p = 0.007), surface nodularity (p = 0.011), and gallbladder fossa widening (p < 0.001) demonstrated a statistically signifi- cant difference between patients with neurological presen- tation and non-neurological presentation. Other MR findings did not present a statistically significant difference between neurological presentation and non-neurological presentation (Table 4). Follow-Up MR Imaging Findings and Clinical Features Twenty follow-up MR examinations obtained from 17 patients demonstrated improved hepatic nodular infiltra- tions for eight patients, aggravated intrahepatic nodules and/or cirrhosis for four patients, and a stationary state of imaging features for five patients (Fig. 2B). In the eight patients with improved nodular lesions, all of them showed improved hepatic dysfunction (normalized serum transami- 670 Clinical Presentation Neurological (n = 12) Liver MR Findings in Patients with Child-Pugh Class A (n = 36) Intrahepatic nodules Hypointensity on T2WI 08 (67%) Hyperintense on T1WI 04 (33%) Hepatic Contour Surface nodularity 08 (67%) Caudate hypertrophy 0 Gallbladder fossa widening 11 (92%) Others Ascites 0 Venous collaterals 1 (8%) Gallbladder wall edema 0 Splenomegaly 11 (92%) Non- Neurological (n = 24) 04 (17%) 2 (8%) 05 (21%) 2 (8%) 1 (4%) 2 (8%) 2 (8%) 016 (67%)0 P value 0.007 0.149 0.011 0.543 1.000 1.000 0.543 0.108 06 (25%) < 0.001 nase level, n = 5; improved hyperbilirubinemia, n = 1; improved coagulation abnormality, n = 2). In four patients with aggravated nodular lesions and/or cirrhosis, newly appeared intrahepatic nodules were detected in two patients, whereas aggravated intrahepatic nodules and cirrhosis were observed in two patients. Among these four patients, two patients underwent liver transplantation (Fig. 4C). DISCUSSION In Wilson’s disease, asymptomatic hepatic copper deposi- tion in liver cells occurs early in the disease’s progression, predominantly in the periportal regions and along the hepatic sinusoids (17, 18). After episodes of acute hepatitis, which may be reflected by elevated plasma levels of liver enzymes, a fatty change and periportal inflammation can develop. Afterwards, piecemeal necrosis and fibrosis with inflammatory cell infiltrations induce chronic active hepati- tis. Finally, after several years, insidious development of irreversible cirrhosis occurs (19). In a large cohort study of patients with Wilson’s disease, liver biopsies revealed variable hepatic involvement in 37% of patients presenting cirrhosis, 36% at an unspecified stage of fibrosis, and 54% with steatosis (20). Considering the variable hepatic involvement, various imaging findings of the liver in Wilson’s disease are demonstrated (8-13). Recently, Akhan et al. (14) reported that Wilson’s disease should be considered as one of the leading diagnoses of young patients with a perihepatic fat Korean J Radiol 11(6), Nov/Dec 2010