Childhood adrenoleukodystrophy: assessment with proton MR spectroscopy

Radiology. 1993 Nov;189(2):467-80. doi: 10.1148/radiology.189.2.8210375.

Abstract

Purpose: Image-guided, single-voxel, localized proton magnetic resonance (MR) spectroscopy was performed to assess white matter in childhood adrenoleukodystrophy (ALD).

Materials and methods: Eleven X-linked ALD subjects, seven with neurologic symptoms or white matter lesions at MR imaging and four asymptomatic patients, were compared with nine aged-matched, healthy volunteers.

Results: Compared with those from normal white matter, MR spectra from white matter lesions (n = 6) showed 65% reduction in the ratio of N-acetyl aspartate (NAA) to total creatine (tCr) (P < .01); 55% increase in the ratio of choline-containing compounds (Cho) to tCr (P < .02); substantial levels of tCr; 94% increase in the ratio of glutamate, glutamine, or inositol to tCr (P < .02); and lactate accumulation in four patients. Patients without brain lesions (n = 4) exhibited a 51% increase in Cho-to-tCr ratio (P < .01) and 11% nonsignificant increase in NAA-to-tCr ratio.

Conclusion: Proton MR spectroscopy may prove a valuable technique for noninvasive diagnostic and prognostic assessment of ALD.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Adrenoleukodystrophy / diagnosis*
  • Adrenoleukodystrophy / genetics
  • Adrenoleukodystrophy / metabolism
  • Aspartic Acid / analogs & derivatives
  • Aspartic Acid / analysis
  • Child
  • Child, Preschool
  • Choline / analysis
  • Creatine / analysis
  • Genetic Linkage
  • Glucose / analysis
  • Glutamates / analysis
  • Glutamine / analysis
  • Glycine / analysis
  • Humans
  • Hydrogen
  • Inositol / analysis
  • Lipids / analysis
  • Magnetic Resonance Imaging
  • Magnetic Resonance Spectroscopy*
  • Male
  • Protons
  • X Chromosome
  • gamma-Aminobutyric Acid / analysis

Substances

  • Glutamates
  • Lipids
  • Protons
  • Glutamine
  • Aspartic Acid
  • Inositol
  • gamma-Aminobutyric Acid
  • Hydrogen
  • N-acetylaspartate
  • Glucose
  • Creatine
  • Choline
  • Glycine