American Journal of Neuroradiology 27:1373-1378, June-July 2006
© 2006 American Society of Neuroradiology
PEDIATRICS
MR Imaging of the Brain in Wilson Disease of Childhood: Findings Before and After Treatment with Clinical Correlation
a Department of Radiology, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Institute of Radiation Medicine, Seoul National University Medical Research Center
b Department of Radiology, Seoul National University Hospital, Seoul National University College of Medicine, Institute of Radiation Medicine, Seoul National University Medical Research Center
c Department of Pediatrics, Seoul National University Hospital, Seoul National University College of Medicine
Please address correspondence to: In-One Kim, MD, Department of Radiology, Seoul National University Hospital, #28 Yongon-dong, Chongno-gu, Seoul 110-744, Korea
BACKGROUND AND PURPOSE: Although brain MR imaging findings in adult Wilson disease have been described in considerable detail, a paucity of information currently exists regarding brain MR imaging findings in pediatric Wilson disease. The purpose of this study was to analyze the brain MR imaging findings in Wilson disease of childhood at the initial stage and during follow-up after treatment and to correlate these observations with clinical response.
METHODS: We evaluated 50 patients with pediatric Wilson disease. Fifty initial and 20 follow-up MR images from 15 patients following penicillamine treatment were analyzed retrospectively, and the data were correlated with clinical findings.
RESULTS: Patients were categorized into 3 groups on the basis of initial MR imaging findings. Group I (n = 23) showed normal MR imaging findings. Group II (n = 15) was characterized by T1-weighted images with increased signal intensity in the globus pallidus (n = 15, 100%) followed by the putamen, midbrain, and caudate nucleus. Group III (n = 12) demonstrated T2-weighted images with increased signal intensity in the putamen (n = 10, 83%), followed by the caudate nucleus, globus pallidus, thalamus, midbrain, and pons. There was a significant difference in mean age, the presence of neurologic symptoms, and Child-Pugh classification among the 3 groups (P < .001). Following copper chelating therapy, the changes on follow-up MR imaging were strongly correlated with clinical response to treatment (P < .001).
CONCLUSION: Brain MR imaging in children with Wilson disease can be categorized into distinct groups and demonstrated a significant correlation with clinical findings. Interval changes on follow-up MR imaging were also closely correlated with clinical findings and helpful in assessing the clinical response.