Acute putaminal necrosis and white matter demyelination in a child with subnormal copper metabolism in Wilson disease: MR imaging and spectroscopic findings.

Chun Jung Juan, Cheng Yu Chen, Yi Jui Liu, Hsiao Wen Chung, Shy Chy Chin, Chun Jen Hsueh, Hsin Chu, Robert A. Zimmerman

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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.

Original languageEnglish
Pages (from-to)401-405
Number of pages5
JournalNeuroradiology
Volume47
Issue number6
Publication statusPublished - Jun 2005
Externally publishedYes

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ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Clinical Neurology
  • Radiological and Ultrasound Technology

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