Down syndrome with and without dementia: an in vivo proton Magnetic Resonance Spectroscopy study with implications for Alzheimer's disease

Neuroimage. 2011 Jul 1;57(1):63-68. doi: 10.1016/j.neuroimage.2011.03.073. Epub 2011 Apr 12.

Abstract

It is poorly understood why people with Down syndrome (DS) are at extremely high-risk of developing Alzheimer's disease (AD) compared to the general population. One explanation may be related to their extra copy of risk factors modulated by chromosome 21. Myo-inositol (mI), whose transporter gene is located on chromosome 21, has been associated with dementia in the non-DS population; however, nobody has contrasted brain mI in DS with (DS+) and without (DS-) dementia to other non-DS groups. Our primary aim was to compare the hippocampal concentration of mI ([mI]) and other brain metabolites such as N-acetylaspartate (NAA; a proxy measure of neuronal density and mitochondrial function) in DS+, DS-, and age-matched healthy controls using proton Magnetic Resonance Spectroscopy (((1))H-MRS). We compared hippocampal [mI] and other metabolites in 35 individuals with genetically-confirmed DS [DS+ (n=17, age=53±6) and DS- (n=18, age=47±8)] to age-matched healthy controls (n=13, age=51±10) adjusting for proportion of the MRS voxel occupied by cerebrospinal spinal fluid, and gray/white matter. DS+ had a significantly higher [mI] than both DS- and healthy controls. In contrast neither DS+ nor DS- differed significantly from controls in [NAA] (although NAA in DS+ was significantly lower than DS-). Our secondary aim of comparing brain metabolites in DS+ and DS- to Alzheimer's disease (AD; n=39; age=77±5) revealed that the DS+ group had significantly elevated [mI] compared to AD or DS-. [mI] may modify risk for dementia in this vulnerable population.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Alzheimer Disease / complications
  • Alzheimer Disease / metabolism*
  • Aspartic Acid / analogs & derivatives
  • Aspartic Acid / analysis
  • Brain Chemistry / genetics*
  • Case-Control Studies
  • Dementia / etiology
  • Dementia / metabolism
  • Down Syndrome / complications
  • Down Syndrome / genetics
  • Down Syndrome / metabolism*
  • Female
  • Hippocampus / chemistry*
  • Hippocampus / metabolism
  • Humans
  • Inositol / analysis
  • Magnetic Resonance Spectroscopy
  • Male
  • Middle Aged

Substances

  • Aspartic Acid
  • Inositol
  • N-acetylaspartate