MR-spectroscopic findings in juvenile-onset Huntington's disease

Mov Disord. 2008 Oct 15;23(13):1931-5. doi: 10.1002/mds.22245.

Abstract

Seven HD gene positive individuals under the age of 21 years are described with clinical examination and proton-MR-spectroscopy ((1)H-MRS) profiles of the putamen. Despite clinical variability, the predominate (1)H-MRS abnormality is elevated glutamate, expressed well beyond the confines of the basal ganglia, and low striatal creatine.

MeSH terms

  • Adolescent
  • Aspartic Acid / analogs & derivatives
  • Aspartic Acid / metabolism
  • Brain Mapping
  • Child
  • Child, Preschool
  • Creatine / metabolism
  • Female
  • Glutamic Acid / metabolism
  • Humans
  • Huntington Disease / diagnosis*
  • Huntington Disease / genetics
  • Magnetic Resonance Spectroscopy / methods*
  • Male
  • Protons
  • Putamen / metabolism
  • Trinucleotide Repeats / genetics
  • Young Adult

Substances

  • Protons
  • Aspartic Acid
  • Glutamic Acid
  • N-acetylaspartate
  • Creatine