Acute putaminal necrosis and white matter demyelination in a child with subnormal copper metabolism in Wilson disease: MR imaging and spectroscopic findings.
Journal
Neuroradiology.
Journal Volume
47
Journal Issue
6
Pages
401-405
Date Issued
2005
Author(s)
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.
Other Subjects
copper; article; case report; child; demyelinating disease; human; male; metabolism; necrosis; nuclear magnetic resonance imaging; nuclear magnetic resonance spectroscopy; pathology; putamen; Wilson disease; Child; Copper; Demyelinating Diseases; Hepatolenticular Degeneration; Humans; Magnetic Resonance Imaging; Magnetic Resonance Spectroscopy; Male; Necrosis; Putamen
Type
journal article