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Altered brain iron content and deposition rate in Huntington’s disease as indicated by quantitative susceptibility MRI
Author(s) -
Chen Lin,
Hua Jun,
Ross Christopher A.,
Cai Shuhui,
van Zijl Peter C.M.,
Li Xu
Publication year - 2019
Publication title -
journal of neuroscience research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.72
H-Index - 160
eISSN - 1097-4547
pISSN - 0360-4012
DOI - 10.1002/jnr.24358
Subject(s) - putamen , globus pallidus , striatum , atrophy , medicine , caudate nucleus , basal ganglia , huntington's disease , glucocerebrosidase , magnetic resonance imaging , gastroenterology , endocrinology , psychology , cardiology , disease , central nervous system , dopamine , radiology
Altered brain iron content in the striatum of premanifest and manifest Huntington’s disease (HD) has been reported. However, its natural history remains unclear. This study aims to investigate altered brain iron content in premanifest and early HD, and the iron deposition rate in these patients through a longitudinal one‐year follow‐up test, with quantitative magnetic susceptibility as an iron imaging marker. Twenty‐four gene mutation carriers divided into three groups (further‐from‐onset, closer‐to‐onset and early HD) and 16 age‐matched healthy controls were recruited at baseline, and of these, 14 carriers and 7 controls completed the one‐year follow‐up. Quantitative magnetic susceptibility and effective transverse relaxation rate ( R 2∗ ) were measured at 7.0 Tesla and correlated with atrophy and available clinical and cognitive measurements. Higher susceptibility values indicating higher iron content in the striatum and globus pallidus were only observed in closer‐to‐onset ( N = 6, p < 0.05 in caudate and p < 0.01 in putamen) and early HD ( N = 9, p < 0.05 in caudate and globus pallidus and p < 0.01 in putamen). Similar results were found by R 2∗measurement. Such increases directly correlated with HD CAG–age product score and brain atrophy, but not with motor or cognitive scores. More importantly, a significantly higher iron deposition rate (11.9%/years in caudate and 6.1%/years in globus pallidus) was firstly observed in closer‐to‐onset premanifest HD and early HD as compared to the controls. These results suggest that monitoring brain iron may provide further insights into the pathophysiology of HD disease progression, and may provide a biomarker for clinical trials.