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Related Concept Videos

Huntington Disease l: Introduction01:21

Huntington Disease l: Introduction

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 reduced penetrance,...

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Diffusion Tensor Magnetic Resonance Imaging in the Analysis of Neurodegenerative Diseases
09:33

Diffusion Tensor Magnetic Resonance Imaging in the Analysis of Neurodegenerative Diseases

Published on: July 28, 2013

Longitudinal diffusion tensor imaging in Huntington's Disease.

Kurt E Weaver1, Todd L Richards, Olivia Liang

  • 1Department of Radiology, University of Washington School of Medicine, Box #357115, Seattle, WA 98195, USA. weaverk@u.washington.edu

Experimental Neurology
|March 26, 2009
PubMed
Summary
This summary is machine-generated.

Huntington's Disease (HD) shows white matter (WM) degeneration, evidenced by reduced fractional anisotropy (FA). Longitudinal DTI scans reveal axonal injury as a key factor, suggesting FA changes can serve as a biomarker for HD progression.

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Area of Science:

  • Neuroscience
  • Radiology
  • Genetics

Background:

  • Huntington's Disease (HD) is a neurodegenerative disorder with poorly understood white matter (WM) pathology.
  • Diffusion Tensor Imaging (DTI) offers insights into WM integrity and microstructural changes.

Purpose of the Study:

  • To investigate the longitudinal neurodegenerative profile of white matter (WM) in individuals with the Huntington's Disease (HD) gene mutation.
  • To determine if DTI metrics can serve as neuropathological biomarkers for HD.

Main Methods:

  • Serial DTI scans were performed at baseline and 1-year follow-up in 7 HD gene mutation carriers and 7 age/gender-matched controls.
  • Analysis focused on fractional anisotropy (FA) and diffusivity measures (axial and radial) to assess WM integrity and axonal stability.

Main Results:

  • HD subjects exhibited significant whole-brain reduction in FA between baseline and follow-up, unlike controls.
  • Axial diffusivity showed significant longitudinal decreases in HD subjects, correlating with FA changes.
  • Radial diffusivity increases, indicative of demyelination, were less pronounced than axial diffusivity declines.

Conclusions:

  • These findings provide the first longitudinal DTI evidence of WM degeneration in HD.
  • FA abnormalities in HD may stem from axonal injury or withdrawal.
  • Longitudinal FA changes show potential as a neuropathological biomarker for Huntington's Disease.