A Critical Review of White Matter Changes in Huntington's Disease.

A Critical Review of White Matter Changes in Huntington's Disease.
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DOI:
10.1002/mds.28109
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发表时间:
2020-08
期刊:
影响因子:
8.6
通讯作者:
Metzler-Baddeley, Claudia
Metzler-Baddeley, Claudia
中科院分区:
医学1区
文献类型:
--
作者:
Casella, Chiara;Lipp, Ilona;Rosser, Anne;Jones, Derek K.;Metzler-Baddeley, Claudia

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亨廷顿氏病是一种遗传性神经退行性疾病。白质改变最近被确定为亨廷顿病的相关病理生理特征,但其病因和在疾病发病机制和进展中的作用尚不清楚。越来越多的证据表明,这种疾病中的白质变化归因于髓磷脂相关生物过程的改变。这篇综述首先讨论了神经化学研究的证据,这些证据支持亨廷顿病的脱髓鞘假说,证明了亨廷顿病大脑中异常的髓鞘形成和少突胶质细胞的变化。接下来,回顾了神经影像学研究的证据,讨论了所描述方法的局限性,并对已发表研究结果的建议解释提出了挑战。虽然我们对亨廷顿氏病相关的大脑病理变化的理解将越来越依赖于神经成像技术,但这些方法的缺点不能被遗忘。磁共振成像技术和组织建模的进步将使白质微观结构的生物特性在体内得到更好的纵向表征。这反过来将促进疾病相关生物标志物的识别和临床试验结果测量的规范。©2020作者。Wiley期刊公司代表国际帕金森和运动障碍学会出版的《运动障碍》。
Huntington’s disease is a genetic neurodegenerative disorder. White matter alterations have recently been identified as a relevant pathophysiological feature of Huntington’s disease, but their etiology and role in disease pathogenesis and progression remain unclear. Increasing evidence suggests that white matter changes in this disorder are attributed to alterations in myelin‐associated biological processes. This review first discusses evidence from neurochemical studies lending support to the demyelination hypothesis of Huntington’s disease, demonstrating aberrant myelination and changes in oligodendrocytes in the Huntington’s brain. Next, evidence from neuroimaging studies is reviewed, the limitations of the described methodologies are discussed, and suggested interpretations of findings from published studies are challenged. Although our understanding of Huntington’s associated pathological changes in the brain will increasingly rely on neuroimaging techniques, the shortcomings of these methodologies must not be forgotten. Advances in magnetic resonance imaging techniques and tissue modeling will enable a better in vivo, longitudinal characterization of the biological properties of white matter microstructure. This in turn will facilitate identification of disease‐related biomarkers and the specification of outcome measures in clinical trials. © 2020 The Authors. Movement Disorders published by Wiley Periodicals, Inc. on behalf of International Parkinson and Movement Disorder Society.
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