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Research paper
Iron accumulation in the basal ganglia in Huntington's disease: cross-sectional data from the IMAGE-HD study
  1. Juan F Domínguez D1,
  2. Amanda C L Ng2,
  3. Govinda Poudel1,3,4,
  4. Julie C Stout1,
  5. Andrew Churchyard5,
  6. Phyllis Chua1,
  7. Gary F Egan1,3,
  8. Nellie Georgiou-Karistianis1
  1. 1School of Psychological Sciences, Monash University, Clayton, Victoria, Australia
  2. 2Department of Anatomy and Neuroscience, The University of Melbourne, Parkville, Victoria, Australia
  3. 3Monash Biomedical Imaging (MBI), Monash University, Melbourne, Victoria, Australia
  4. 4VLSCI Life Sciences Computation Centre, Melbourne, Victoria, Australia
  5. 5Department of Neurology, Monash Medical Centre, Clayton, Victoria, Australia
  1. Correspondence to Professor Nellie Georgiou-Karistianis, School of Psychological Sciences, Monash University, Clayton, VIC 3800, Australia; nellie.georgiou-karistianis{at}monash.edu

Abstract

Objectives To measure iron accumulation in the basal ganglia in Huntington's disease (HD) using quantitative susceptibility mapping (QSM), and to ascertain its relevance in terms of clinical and disease severity.

Methods In this cross-sectional investigation, Graphic weighted imaging was undertaken on 31 premanifest HD, 32 symptomatic HD and 30 control participants as part of the observational IMAGE-HD study. Group differences in iron accumulation were ascertained with QSM. Associations between susceptibility values and disease severity were also investigated.

Results Compared with controls, both premanifest and symptomatic HD groups showed significantly greater iron content in pallidum, putamen and caudate. Additionally, iron accumulation in both putamen and caudate was significantly associated with disease severity.

Conclusions These findings provide the first evidence that QSM is sensitive to iron deposition in subcortical target areas across premanifest and symptomatic stages of HD. Such findings could open up new avenues for biomarker development and therapeutic intervention.

  • HUNTINGTON'S
  • IRON DEPOSITION
  • MRI
  • NEUROPATHOLOGY
  • MOVEMENT DISORDERS

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