Acute putaminal necrosis and white matter demyelination in a child with subnormal copper metabolism in Wilson disease: MR imaging and spectroscopic findings.
Wilson disease (WD) that manifests solely with acute and severe neurological damage in the absence of hepatic disease and Kayser-Fleischer ring of the cornea is rare and difficult to diagnose at the acute setting. This report describes unusual diffusion and proton spectroscopic magnetic resonance (MR) imaging findings in a 12-year-old boy with WD who presented with hemichorea and subnormal copper metabolism. The MR imaging findings of lactate accumulation, decrease of N-acerylaspartate/creatinine (NAA/Cr) ratio and markedly increased apparent diffusion coefficient (ADC) value of the asymmetrical edematous putaminal lesions in the early stage were suggestive of acute necrosis with anaerobic metabolism of glucose leading to poor clinical outcome at follow-up.
Juan
Chun-Jung
CJ
Department of Electrical Engineering, College of Electrical Engineering and Computer Sciences, National Taiwan University, Taipei.
Chen
Cheng-Yu
CY
Liu
Yi-Jui
YJ
Chung
Hsiao-Wen
HW
Chin
Shy-Chy
SC
Hsueh
Chun-Jen
CJ
Chu
Hsin
H
Zimmerman
Robert A
RA
D002648:Child; D003300:Copper / Q000378:metabolism*; D003711:Demyelinating Diseases / Q000209:etiology*; D006527:Hepatolenticular Degeneration / Q000150:complications* / Q000378:metabolism*; D006801:Humans; D008279:Magnetic Resonance Imaging; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D009336:Necrosis / Q000209:etiology; D011699:Putamen / Q000378:metabolism* / Q000473:pathology*
D003300:Copper