title: Acute putaminal necrosis and white matter demyelination in a child with subnormal copper metabolism in Wilson disease: MR imaging and spectroscopic findings.
abstract: 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.
authors.affiliation: Department of Electrical Engineering, College of Electrical Engineering and Computer Sciences, National Taiwan University, Taipei.
authors.forename: Chun-Jung
authors.initials: CJ
authors.lastname: Juan
authors.forename: Cheng-Yu
authors.initials: CY
authors.lastname: Chen
authors.forename: Yi-Jui
authors.initials: YJ
authors.lastname: Liu
authors.forename: Hsiao-Wen
authors.initials: HW
authors.lastname: Chung
authors.forename: Shy-Chy
authors.initials: SC
authors.lastname: Chin
authors.forename: Chun-Jen
authors.initials: CJ
authors.lastname: Hsueh
authors.forename: Hsin
authors.initials: H
authors.lastname: Chu
authors.forename: Robert A
authors.initials: RA
authors.lastname: Zimmerman
mesh_terms: 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*
chemical_list: D003300:Copper