Quantitative susceptibility mapping suggests altered brain iron in premanifest Huntington disease

Jiri M G Van Bergen, J. Hua, P. G. Unschuld, Issel Anne L Lim, Craig Jones, Russell Louis Margolis, Christopher A Ross, Peter C Van Zijl, Xu Li

Research output: Contribution to journalArticle

Abstract

BACKGROUND AND PURPOSE: In patients with premanifest (nonsymptomatic) and advanced Huntington disease, changes in brain iron levels in the basal ganglia have been previously reported, especially in the striatum. Quantitative susceptibility mapping by using MR phase imaging allows in vivo measurements of tissue magnetic susceptibility, which has been shown to correlate well with iron levels in brain gray matter and is believed to be more specific than other imaging-based iron measures. The purpose of this study was to investigate the use of magnetic susceptibility as a biomarker of disease progression. MATERIALS AND METHODS: Fifteen subjects with premanifest Huntington disease and 16 age-matched healthy controls were scanned at 7T. Magnetic susceptibility, effective relaxation, and tissue volume in deep gray matter structures were quantified and compared with genetic and clinical measures. RESULTS: Subjects with premanifest Huntington disease showed significantly higher susceptibility values in the caudate nucleus, putamen, and globus pallidus, indicating increased iron levels in these structures. Significant decreases in magnetic susceptibility were found in the substantia nigra and hippocampus. In addition, significant volume loss (atrophy) and an increase effective relaxation were observed in the caudate nucleus and putamen. Susceptibility values in the caudate nucleus and putamen were found to be inversely correlated with structure volumes and directly correlated with the genetic burdens, represented by cytosine-adenine-guanine repeat age-product-scaled scores. CONCLUSIONS: The significant magnetic susceptibility differences between subjects with premanifest Huntington disease and controls and their correlation with genetic burden scores indicate the potential use of magnetic susceptibility as a biomarker of disease progression in premanifest Huntington disease.

Original languageEnglish (US)
Pages (from-to)789-796
Number of pages8
JournalAmerican Journal of Neuroradiology
Volume37
Issue number5
DOIs
StatePublished - May 1 2016

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Huntington Disease
Iron
Caudate Nucleus
Putamen
Brain
Disease Progression
Biomarkers
Globus Pallidus
Cytosine
Guanine
Adenine
Substantia Nigra
Basal Ganglia
Atrophy
Hippocampus
Gray Matter

ASJC Scopus subject areas

  • Clinical Neurology
  • Radiology Nuclear Medicine and imaging

Cite this

Quantitative susceptibility mapping suggests altered brain iron in premanifest Huntington disease. / Van Bergen, Jiri M G; Hua, J.; Unschuld, P. G.; Lim, Issel Anne L; Jones, Craig; Margolis, Russell Louis; Ross, Christopher A; Van Zijl, Peter C; Li, Xu.

In: American Journal of Neuroradiology, Vol. 37, No. 5, 01.05.2016, p. 789-796.

Research output: Contribution to journalArticle

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