RT Journal Article
SR Electronic
T1 Iron accumulation in the basal ganglia in Huntington's disease: cross-sectional data from the IMAGE-HD study
JF Journal of Neurology, Neurosurgery & Psychiatry
JO J Neurol Neurosurg Psychiatry
FD BMJ Publishing Group Ltd
SP 545
OP 549
DO 10.1136/jnnp-2014-310183
VO 87
IS 5
A1 Juan F Domínguez D
A1 Amanda C L Ng
A1 Govinda Poudel
A1 Julie C Stout
A1 Andrew Churchyard
A1 Phyllis Chua
A1 Gary F Egan
A1 Nellie Georgiou-Karistianis
YR 2016
UL http://jnnp.bmj.com/content/87/5/545.abstract
AB 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, 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.