Rowley Christopher D, Tabrizi Sarah J, Scahill Rachael I, Leavitt Blair R, Roos Raymund A C, Durr Alexandra, Bock Nicholas A
McMaster Integrative Neuroscience Discovery and Study Program, McMaster University, Hamilton, ON, Canada.
Huntington's Disease Centre, University College London Institute of Neurology, National Hospital for Neurology and Neurosurgery, London, United Kingdom.
Front Neurosci. 2018 Nov 5;12:805. doi: 10.3389/fnins.2018.00805. eCollection 2018.
Huntington's disease (HD) is a genetic neurodegenerative disorder that is characterized by neuronal cell death. Although medium spiny neurons in the striatum are predominantly affected, other brain regions including the cerebral cortex also degenerate. Previous structural imaging studies have reported decreases in cortical thickness in HD. Here we aimed to further investigate changes in cortical tissue composition in HD using standard clinical T-weighted (TW) and T-weighted (TW) magnetic resonance images (MRIs). 326 subjects from the TRACK-HD dataset representing healthy controls and four stages of HD progression were analyzed. The intracortical TW/TW intensity was sampled in the middle depth of the cortex over 82 regions across the cortex. While these previously collected images were not optimized for intracortical analysis, we found a significant increase in TW/TW intensity ( < 0.05 Bonferroni-Holm corrected) beginning with HD diagnosis. Increases in ratio intensity were found in the insula, which then spread to ventrolateral frontal cortex, superior temporal gyrus, medial temporal gyral pole, and cuneus with progression into the most advanced HD group studied. Mirroring past histological reports, this increase in the ratio image intensity may reflect disease-related increases in myelin and/or iron in the cortex. These findings suggest that future imaging studies are warranted with imaging optimized to more sensitively and specifically assess which features of cortical tissue composition are abnormal in HD to better characterize disease progression.
亨廷顿舞蹈症(HD)是一种遗传性神经退行性疾病,其特征是神经元细胞死亡。虽然纹状体中的中等棘状神经元受到的影响最为显著,但包括大脑皮层在内的其他脑区也会发生退化。以往的结构成像研究报告称,HD患者的皮层厚度会减小。在此,我们旨在利用标准临床T加权(TW)和T加权(TW)磁共振成像(MRI)进一步研究HD患者皮层组织成分的变化。我们分析了来自TRACK-HD数据集的326名受试者,这些受试者代表了健康对照以及HD进展的四个阶段。在整个皮层的82个区域,于皮层中间深度对皮层内TW/TW强度进行采样。虽然这些先前收集的图像并非为皮层内分析而优化,但我们发现,从HD诊断开始,TW/TW强度就有显著增加(经Bonferroni-Holm校正后P<0.05)。在脑岛发现强度比值增加,随后随着病情进展至所研究的最晚期HD组,强度比值增加扩散至腹外侧额叶皮层、颞上回、颞中回内侧极和楔叶。与过去的组织学报告一致,比值图像强度的这种增加可能反映了皮层中与疾病相关的髓磷脂和/或铁含量的增加。这些发现表明,未来有必要开展成像研究,采用经过优化的成像方法,以更敏感、更具体地评估HD患者皮层组织成分的哪些特征异常,从而更好地表征疾病进展。