Related Experiment Video
Updated: Apr 17, 2026
09:06
Whole-brain Segmentation and Change-point Analysis of Anatomical Brain MRI—Application in Premanifest Huntington's Disease
Published on: June 9, 2018
12.8K
Microstructural brain abnormalities in Huntington's disease: A two-year follow-up
Omar F F Odish1, Alexander Leemans, Robert H A M Reijntjes
1Department of Neurology, Leiden University Medical Center, Leiden, The Netherlands.
Human Brain Mapping
|February 4, 2015
Summary
Diffusion tensor imaging reveals early brain changes in Huntington's disease (HD). Axial diffusivity alterations in white matter and striatum indicate microstructural changes even before symptom onset, correlating with disease progression.
Area of Science:
- Neuroimaging
- Neuroscience
- Medical Imaging
Background:
- Huntington's disease (HD) is a neurodegenerative disorder characterized by progressive motor, cognitive, and psychiatric symptoms.
- Understanding the early microstructural brain changes in HD is crucial for developing effective interventions.
- Diffusion tensor imaging (DTI) offers a non-invasive method to assess white matter (WM) and gray matter (GM) integrity.
Purpose of the Study:
- To investigate cross-sectional and longitudinal microstructural brain changes in different stages of Huntington's disease (HD).
- To utilize diffusion tensor imaging (DTI) to examine striatal and whole-brain alterations in premanifest HD (preHD), early manifest HD, and control groups.
- To identify sensitive DTI markers for early detection of HD-related neuropathology.
Main Methods:
- The study analyzed data from the TRACK-HD cohort, including premanifest gene carriers (preHD), early manifest HD patients, and healthy controls.
- Participants underwent DTI scans at baseline and a 2-year follow-up.
- Age-corrected diffusion measures (mean, axial, and radial diffusivity; fractional anisotropy) were computed for whole-brain WM, GM, and striatum, normalized by voxel count.
Main Results:
- Manifest HD groups showed significantly higher WM and GM diffusivities compared to preHD and control groups.
- PreHD individuals exhibited elevated WM axial diffusivity (AD) compared to controls, particularly those closer to predicted disease onset (preHD-B).
- Striatal AD was also higher in preHD-B compared to controls and preHD-A. Fractional anisotropy (FA) did not effectively differentiate groups. Diffusion measures correlated significantly with neurocognitive function.
Conclusions:
- Cross-sectional DTI reveals significant microstructural alterations in both whole-brain and striatum in manifest HD.
- Axial diffusivity (AD) emerges as a sensitive marker for detecting early, pre-symptomatic microstructural changes in HD.
- Individual diffusion measures, particularly AD, are more effective than FA in identifying pathological brain alterations and correlate with clinical status in HD.
Related Concept Videos
01:21Huntington Disease l: Introduction
2
Huntington disease or HD is a progressive, fatal neurodegenerative disorder inherited in an autosomal dominant pattern.PathophysiologyIt is caused by expansion of the CAG trinucleotide repeat in the HTT gene on chromosome 4 (4p16.3), producing an abnormal huntingtin protein with an expanded polyglutamine tract. This misfolded protein disrupts cellular function, leading to neuronal death. Normal alleles have ≤26 repeats, 27–35 are intermediate (risk of expansion), 36–39 show...
2
01:23Alzheimer Disease ll: Pathophysiology
1
Alzheimer disease involves structural changes in the brain that begin long before symptoms appear. The most distinctive features are extracellular neuritic plaques and intracellular neurofibrillary tangles.Neuritic plaques form in the cerebral cortex and around blood vessels. These plaques contain a dense core of beta-amyloid (Aβ)—a toxic protein fragment that clumps outside neurons. The core is surrounded by damaged neuronal extensions, as well as reactive astrocytes and...
1