Gregory Sarah, Cole James H, Farmer Ruth E, Rees Elin M, Roos Raymund A C, Sprengelmeyer Reiner, Durr Alexandra, Landwehrmeyer Bernhard, Zhang Hui, Scahill Rachael I, Tabrizi Sarah J, Frost Chris, Hobbs Nicola Z
Wellcome Trust Centre for Neuroimaging, UCL, London, WC1N 3BG, UK.
UCL Institute of Neurology, University College London, UK.
J Huntingtons Dis. 2015;4(4):333-46. doi: 10.3233/JHD-150173.
Huntington's disease is marked by progressive neuroanatomical changes, assumed to underlie the development of the disease's characteristic symptoms. Previous work has demonstrated longitudinal macrostructural white-matter atrophy, with some evidence of microstructural change focused in the corpus callosum.
To more accurately characterise longitudinal patterns, we examined white matter microstructural change using Diffusion Tensor Imaging (DTI) data from three timepoints over a 15 month period.
In 48 early-stage HD patients and 36 controls from the multi-site PADDINGTON project, diffusion tensor imaging (DTI) was employed to measure changes in fractional anisotropy (FA) and axial (AD) and radial diffusivity (RD) in 24 white matter regions-of-interest (ROIs).
Cross-sectional analysis indicated widespread baseline between-group differences, with significantly decreased FA and increased AD and RD found in HD patients across multiple ROIs. Longitudinal rates of change differed significantly between HD patients and controls in the genu and body of corpus callosum, corona radiata and anterior limb of internal capsule. Change in RD in the body of the corpus callosum was significantly associated with baseline disease burden, but other clinical associations were not significant.
We detected subtle longitudinal white matter changes in early HD patients. Progressive white matter abnormalities in HD may not be uniform throughout the brain, with some areas remaining static in the early symptomatic phase. Longer assessment periods across disease stages will help map this progressive trajectory.
亨廷顿舞蹈症以进行性神经解剖学变化为特征,这些变化被认为是该疾病特征性症状发展的基础。先前的研究已经证明了纵向宏观结构的白质萎缩,并有一些证据表明微观结构变化集中在胼胝体。
为了更准确地描述纵向模式,我们使用了15个月内三个时间点的扩散张量成像(DTI)数据来研究白质微观结构变化。
在多中心帕丁顿项目的48例早期亨廷顿舞蹈症患者和36例对照中,采用扩散张量成像(DTI)测量24个白质感兴趣区(ROI)的分数各向异性(FA)、轴向扩散率(AD)和径向扩散率(RD)的变化。
横断面分析表明,两组之间存在广泛的基线差异,在多个ROI中,亨廷顿舞蹈症患者的FA显著降低,AD和RD升高。亨廷顿舞蹈症患者和对照组在胼胝体膝部和体部、放射冠和内囊前肢的纵向变化率存在显著差异。胼胝体体部RD的变化与基线疾病负担显著相关,但其他临床相关性不显著。
我们在早期亨廷顿舞蹈症患者中检测到了细微的纵向白质变化。亨廷顿舞蹈症中进行性白质异常在整个大脑中可能并不均匀,在症状早期一些区域保持不变。跨疾病阶段的更长评估期将有助于描绘这种进行性轨迹。