MR spectroscopy in monitoring the treatment of Wilson's disease patients

Mov Disord. 2008 Aug 15;23(11):1560-6. doi: 10.1002/mds.22163.

Abstract

The aim of this study was to determine the effectiveness of brain proton magnetic resonance spectroscopy ((1)H-MRS) for monitoring therapy in Wilson's disease (WD) patients. Voxels were located in the globus pallidus (right, left). We followed 17 newly diagnosed WD cases for 1-year period. During this observation period, 6 neurological and 9 hepatic patients improved, while 2 neurological patients deteriorated. The pretreatment (1)H-MRS analysis showed a statistically significant lower level of mI/Cr, NAA/Cr, and higher Lip/Cr in all WD patients with improvement compared with controls. In patients with hepatic signs, a statistically significant increase of mI/Cr and Glx/Cr was observed in the second (1 year posttreatment) (1)H-MRS. In patients with neurological improvement after treatment in the follow-up (1)H-MRS, a statistically significant increase of NAA/Cr was noted. During neurological deterioration, a decrease of Glx/Cr and NAA/Cr was seen, in contrast to another neurologically impaired patient with liver failure exacerbation, where a decrease of mI/Cr and increase of Glx/Cr was observed. The alternations of NAA/Cr ratio in neurologically impaired patients and mI/Cr and Glx/Cr in patients with liver failure could be a sensitive marker of the clinical recovery and deterioration in those WD patients. (1)H-MRS is a technique that can be used for accurate monitoring of treatment efficacy in WD patients.

Publication types

  • Controlled Clinical Trial
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Antidotes / therapeutic use
  • Aspartic Acid / analysis
  • Brain / metabolism
  • Brain / pathology
  • Case-Control Studies
  • Choline / analysis
  • Creatine / analysis
  • Female
  • Follow-Up Studies
  • Glutamic Acid / analysis
  • Hepatolenticular Degeneration / diagnosis*
  • Hepatolenticular Degeneration / drug therapy
  • Hepatolenticular Degeneration / metabolism
  • Humans
  • Magnetic Resonance Imaging / methods
  • Magnetic Resonance Spectroscopy / methods*
  • Male
  • Penicillamine / therapeutic use
  • Protons*
  • Severity of Illness Index
  • Young Adult

Substances

  • Antidotes
  • Protons
  • Aspartic Acid
  • Glutamic Acid
  • Penicillamine
  • Creatine
  • Choline