Article Text

Download PDFPDF

  1. M-C Porter1,2,
  2. E De Vita3,
  3. I Simpson4,
  4. E Franko1,2,
  5. N Bjurstrom1,2,
  6. P Rudge1,2,
  7. S Mead1,2,
  8. J Collinge1,2,
  9. J Thornton3,
  10. H Hyare1,2
  1. 1MRC Prion Unit, Department of Neurodegenerative Disease, UCL Institute of Neurology
  2. 2NHS National Prion Clinic, National Hospital for Neurology and Neurosurgery
  3. 3Academic Neuroradiological Unit, Department of Brain Repair and Rehabilitation, UCL
  4. 4UCL Centre for Medical Image Computing


Quantitative MRI measures can detect the presence of microstructural pathology in human prion disease not visible on conventional MR imaging. Few studies have examined longitudinal change and correlation with clinical measures. To test if longitudinal change could be identified on quantitative MRI, we performed a multiple-parameter analysis in the largest data set of patients with prion disease to date.

Multiple-parameter analysis of quantitative MRI sequences including diffusion tensor imaging, magnetisation transfer ratio and T2-weighted imaging was performed in 106 subjects (37 asymptomatic gene mutation carriers, 39 symptomatic patients and 30 healthy controls). The analysis included voxel-based morphometry, voxel-based analysis of diffusion tensor imaging and magnetisation transfer imaging measures, grey and white matter histograms and region of interest analysis.

Symptomatic patients showed significant longitudinal grey matter atrophy. Additionally a significant decrease in magnetisation transfer ratio and mean diffusivity was observed in grey matter structures; the decline in all MRI parameters strongly correlated with a decline in a functionally orientated outcome measure, the MRC Prion Disease Rating Scale. These data show that multi-parameter MRI analysis can identify longitudinal microstructural grey matter changes and may be able to provide objective and anatomically-specific biomarkers of disease progression in future clinical trials.


Statistics from

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.