Iron accumulation in the basal ganglia in Huntington's disease: cross-sectional data from the IMAGE-HD study
Iron accumulation in the basal ganglia in Huntington's disease: cross-sectional data from the IMAGE-HD study
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DOI：
10.1136/jnnp-2014-310183
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发表时间：
2016-05-01
影响因子：
11
通讯作者：
Georgiou-Karistianis, Nellie
中科院分区：
文献类型：
--
作者：
Dominguez, Juan F. D.;Ng, Amanda C. L.;Georgiou-Karistianis, Nellie
目的应用定量易感图谱（QSM）测定亨廷顿病（HD）患者基底神经节铁积累，并探讨其与临床和疾病严重程度的相关性。方法在本横断面研究中，作为观察性IMAGE-HD研究的一部分，对31例先兆HD、32例症状性HD和30例对照进行T-2*加权成像。用QSM确定各组铁积累的差异。还调查了易感值与疾病严重程度之间的关系。结果与对照组相比，表现前组和症状组患者脑白质、壳核和尾状核铁含量均显著升高。此外，壳核和尾状核中的铁积累与疾病严重程度显著相关。这些研究结果首次证明，在HD的前期和症状期，QSM对皮质下靶区铁沉积敏感。这些发现可能为生物标志物的开发和治疗干预开辟新的途径。
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, T-2* 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.