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From the University Department of Clinical Neurosciences (Drs. Page, Davie, and Schapira), Royal Free and University College Medical School, Institute of Neurology (Drs. MacManus, Miller, and Schapira), and Department of Neuroradiology (Dr. Miszkiel), National Hospital for Neurology and Neurosurgery, University College of London, University College of London Hospitals Trust (Dr. Walshe), and Reta Lila Weston Institute of Neurological Studies (Dr. Lees), London, UK.
Address correspondence and reprint requests to Dr. C.A. Davie, University Department of Clinical Neurosciences, Royal Free and University College Medical School, UCL, London NW3 2QG, UK; e-mail: c.davie{at}rfc.ucl.ac.uk
Background: The progression of Wilson disease (WD), a disorder of copper metabolism, can be arrested by chelation therapy. However, neurologic deficits may persist despite adequate treatment. MRI is used to assess patients with WD, but previous attempts to correlate clinical progression with the investigation findings have often been unsuccessful.
Objective: To identify MR visible markers that could help stratify disease severity and to clarify the mechanism of persistent neurologic deficit after treatment.
Methods: MRI and proton MR spectroscopy (1H-MRS) were performed in 17 patients with WD. MRI was assessed semiquantitatively and used to locate volumes of interest (voxels) in the striatum for 1H-MRS.
Results: MRI showed abnormalities predominantly confined to those patients with neurologic features of WD. The 1H spectra demonstrated a reduction of N-acetylaspartate and N-acetylaspartylglutamate (2.05 mM; p < 0.01) in those patients with neurologic features but not in patients without clinical neurologic involvement (0.42 mM; p > 0.1) in comparison with age-matched normal control subjects. Choline was also reduced in both patient groups (0.08 mM; p < 0.01) compared with age-matched controls.
Conclusions: There may be a biochemical correlate of tissue-specific dysfunction in patients with Wilson disease who develop neurologic features. These changes appear to be present despite prior clinical improvement and may imply a need for additional treatment.
Received July 26, 2003. Accepted in final form April 26, 2004.
Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the August 24 issue to find the title link for this article.
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