Whole-brain atrophy as a measure of progression in premanifest and early Huntington's disease

Authors

  • Susie M.D. Henley PhD,

    Corresponding author
    1. Dementia Research Centre, Institute of Neurology, University College London, London, United Kingdom
    • Dementia Research Centre, Box 16, National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, United Kingdom
    Search for more papers by this author
    • Susie M.D. Henley and Edward J. Wild are equal first authors.

  • Edward J. Wild MRCP,

    1. Dementia Research Centre, Institute of Neurology, University College London, London, United Kingdom
    Search for more papers by this author
    • Susie M.D. Henley and Edward J. Wild are equal first authors.

  • Nicola Z. Hobbs MEng,

    1. Dementia Research Centre, Institute of Neurology, University College London, London, United Kingdom
    Search for more papers by this author
  • Chris Frost DipStat,

    1. Dementia Research Centre, Institute of Neurology, University College London, London, United Kingdom
    2. Medical Statistics Unit, London School of Hygiene and Tropical Medicine, London, United Kingdom
    Search for more papers by this author
  • David G. MacManus MSc,

    1. NMR Research Unit, Institute of Neurology, University College London, London, United Kingdom
    Search for more papers by this author
  • Roger A. Barker PhD,

    1. Department of Clinical Neurosciences, Brain Repair Centre, Addenbrooke's Hospital, Cambridge, United Kingdom
    Search for more papers by this author
  • Nick C. Fox PhD,

    1. Dementia Research Centre, Institute of Neurology, University College London, London, United Kingdom
    2. Department of Clinical Neurology, National Hospital for Neurology and Neurosurgery, Queen Square, London, United Kingdom
    Search for more papers by this author
    • Nick C. Fox and Sarah J. Tabrizi are equal senior authors.

  • Sarah J. Tabrizi PhD

    1. Department of Clinical Neurology, National Hospital for Neurology and Neurosurgery, Queen Square, London, United Kingdom
    2. Department of Neurodegenerative Disease, Institute of Neurology, University College London, United Kingdom
    Search for more papers by this author
    • Nick C. Fox and Sarah J. Tabrizi are equal senior authors.


  • Potential conflict of interest: None reported.

Abstract

Therapeutic trials in Huntington's disease (HD) are challenging as clinical progression is slow and variable and reliable biomarkers are lacking. We used magnetic resonance imaging and the brain boundary shift integral to quantify whole-brain atrophy rates over 1 year in early and premanifest HD subjects, and controls. Early HD subjects had statistically significantly (P = 0.007) increased (threefold higher) rates of whole-brain atrophy compared with controls. Higher atrophy rates were associated with longer CAG repeat length. MRI-based measures of whole-brain atrophy may have potential as a measure of progression in HD. © 2009 Movement Disorder Society

Ancillary