Quantitative measurements with localized 1H MR spectroscopy in children with Canavan's disease

J Magn Reson Imaging. Nov-Dec 1996;6(6):889-93. doi: 10.1002/jmri.1880060609.


Canavan's disease is an autosomal recessive hereditary leukodystrophy resulting from deficiency of the enzyme aspartoacylase. Two children suffering from this metabolic brain disease were examined using image-guided localized proton spectroscopy. The absolute concentrations of metabolites were determined. These data demonstrate, for the first time, that the well known increase of the N-acetylaspartic acid (NAA)/Cho ratio in this disease may be not only due to a reduction of choline-containing compounds in brain tissue but, at least in specific cases, also due to an increase of the NAA concentration, which is a result of the enzyme defect.

Publication types

  • Case Reports
  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aspartic Acid / analogs & derivatives
  • Aspartic Acid / metabolism
  • Brain / metabolism
  • Canavan Disease / metabolism*
  • Choline / metabolism
  • Female
  • Humans
  • Infant
  • Magnetic Resonance Spectroscopy / instrumentation
  • Magnetic Resonance Spectroscopy / methods*
  • Male
  • Reference Values


  • Aspartic Acid
  • N-acetylaspartate
  • Choline