当前位置： SCI文献检索 > ANNALS OF NEUROLOGY期刊下所有文献 > In vivo characterization of white matter pathology in premanifest huntington's disease.

In vivo characterization of white matter pathology in premanifest huntington's disease.

Abstract：

OBJECTIVE:Huntington's disease (HD) is a monogenic, fully penetrant neurodegenerative disorder, providing an ideal model for understanding brain changes occurring in the years prior to disease onset. Diffusion tensor imaging (DTI) studies show widespread white matter disorganization in the early premanifest stages (pre-HD). However, although DTI has proved informative, it provides only limited information about underlying changes in tissue properties. Neurite orientation dispersion and density imaging (NODDI) is a novel magnetic resonance imaging (MRI) technique for characterizing axonal pathology more specifically, providing metrics that separately quantify axonal density and axonal organization. Here, we provide the first in vivo characterization of white matter pathology in pre-HD using NODDI. METHODS:Diffusion-weighted MRI data that support DTI and NODDI were acquired from 38 pre-HD and 45 control participants. Using whole-brain and region-of-interest analyses, NODDI metrics were compared between groups and correlated with clinical scores of disease progression. Whole-brain changes in DTI metrics were also examined. RESULTS:The pre-HD group displayed widespread reductions in axonal density compared with control participants; this correlated with measures of clinical disease progression in the body and genu of the corpus callosum. There was also evidence in the pre-HD group of increased coherence of axonal packing in the white matter surrounding the basal ganglia. INTERPRETATION:Our findings suggest that reduced axonal density is one of the major factors underlying white matter pathology in pre-HD, coupled with altered local organization in areas surrounding the basal ganglia. NODDI metrics show promise in providing more specific information about the biological processes underlying HD and neurodegeneration per se. Ann Neurol 2018;84:497-504.

journal_name

Ann Neurol

journal_title

Annals of neurology

authors

Zhang J,Gregory S,Scahill RI,Durr A,Thomas DL,Lehericy S,Rees G,Tabrizi SJ,Zhang H,TrackOn-HD investigators.

doi

10.1002/ana.25309

subject

Has Abstract

pub_date

2018-10-01 00:00:00

pages

497-504

issue

eissn

0364-5134

issn

1531-8249

journal_volume

pub_type

杂志文章,多中心研究

在线工具

In vivo characterization of white matter pathology in premanifest huntington's disease.