MR Imaging of the Brain in Wilson Disease of Childhood: Findings Before and After Treatment with Clinical Correlation
T.J. Kima,b,
I.O. Kimb,
W.S. Kimb,
J.E. Cheonb,
S.G. Moonb,
J.W. Kwonb,
J.K. Seoc and
K.M. Yeonb
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
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Fig 1. Wilson disease in a 13-year-old boy with abdominal distention.
A and B, T1-weighted axial MR images show bilateral increased signal intensity in the globus pallidus (arrows) and midbrain (arrowhead).
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Fig 2. High-signal-intensity lesions on T2-weighted MR images.
A, Wilson disease in a 12-year-old girl with dysarthria. T2-weighted axial MR image shows bilateral high signal intensity with central dark signal intensity in the putamen (arrows) and caudate nuclei (arrowheads).
B, Wilson disease in a 13-year-old girl with dysarthria. T2-weighted axial MR image shows bilateral symmetric high signal intensity in the putamen (black arrows) and caudate nuclei (white arrows). Slightly increased signal intensity in both thalami (black arrowheads) and globus pallidi (white arrowheads) is also noted.
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Fig 3. Wilson disease in an 11-year-old girl with abnormal findings on a liver function test.
A, Initial T1-weighted axial MR image shows a subtle increased signal intensity in both globus pallidi (arrows).
B, Follow-up T1-weighted axial MR image obtained after 5 years shows increased extent of the lesion (arrows). Patient’s Child-Pugh class was Child C, despite adequate penicillamine therapy, and she had intractable ascites and a bleeding tendency.
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Fig 4. Wilson disease in a 14-year-old girl with dysarthria.
A, Initial T2-weighted axial MR image shows increased signal intensity in both caudate nuclei (arrowheads) and putamen (arrows).
B, Follow-up T2-weighted axial MR image obtained after 3 years shows complete resolution of the lesions. Patient’s neurologic symptoms were also improved.
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Fig 5. Wilson disease in a 14-year-old girl with dystonia.
A, Initial T2-weighted axial MR image shows increased signal intensity in both caudate nuclei (arrowheads) and putamen (arrows).
B and C, Follow-up T2-weighted axial MR images obtained after 3 years show aggravated lesions, especially in the thalamus (white arrows) and midbrain (arrowheads). Clinically, the patient’s neurologic symptoms, including dystonia, were deteriorated, despite adequate treatment.
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