Localized 1H NMR spectroscopy in Canavan's disease: a report of two cases

Magn Reson Med. 1991 Jun;19(2):439-45. doi: 10.1002/mrm.1910190235.

Abstract

Two children with Canavan's Disease, an autosomal recessive leukodystrophy, were studied by localized 1H spectroscopy. The N-acetylaspartate (NAA) signal intensity was high relative to other metabolite signals, and the signal intensity from choline-containing compounds was low. These findings are discussed in relation to a possible role for NAA in normal myelination.

Publication types

  • Case Reports

MeSH terms

  • Aspartic Acid / analogs & derivatives
  • Aspartic Acid / metabolism
  • Basal Ganglia / metabolism
  • Brain / pathology
  • Child, Preschool
  • Choline / metabolism
  • Creatine / metabolism
  • Diffuse Cerebral Sclerosis of Schilder / diagnosis*
  • Diffuse Cerebral Sclerosis of Schilder / genetics
  • Diffuse Cerebral Sclerosis of Schilder / pathology
  • Female
  • Humans
  • Hydrogen
  • Infant
  • Magnetic Resonance Spectroscopy* / methods
  • Male
  • Occipital Lobe / metabolism

Substances

  • Aspartic Acid
  • Hydrogen
  • N-acetylaspartate
  • Creatine
  • Choline