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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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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 microglia. Abnormal...

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Related Experiment Video

Updated: May 19, 2026

Whole-brain Segmentation and Change-point Analysis of Anatomical Brain MRI—Application in Premanifest Huntington's Disease
09:06

Whole-brain Segmentation and Change-point Analysis of Anatomical Brain MRI—Application in Premanifest Huntington's Disease

Published on: June 9, 2018

Brain structure in preclinical Huntington's disease: a multi-method approach.

Robert Christian Wolf1, Philipp Arthur Thomann, Anne Kerstin Thomann

  • 1Center of Psychosocial Medicine, Department of General Psychiatry, University of Heidelberg, Heidelberg, Germany. christian.wolf@med.uni-heidelberg.de

Neuro-Degenerative Diseases
|August 22, 2012
PubMed
Summary

Detecting early Huntington's disease (HD) biomarkers in preHD individuals is crucial. Advanced MRI techniques reveal subtle striatal changes, offering a more reliable marker for disease progression than cortical changes.

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Area of Science:

  • Neuroimaging
  • Neurodegenerative diseases
  • Biomarker discovery

Background:

  • Structural magnetic resonance imaging (MRI) shows promise for identifying early biomarkers in pre-symptomatic Huntington's disease (preHD) gene mutation carriers.
  • Previous studies found structural brain changes mainly in preHD individuals closer to motor onset, with inconclusive findings in those further from onset.

Purpose of the Study:

  • To assess the sensitivity of various structural MRI methods in detecting changes in preHD individuals far from estimated motor onset.
  • To investigate the relationship between brain structure, clinical variables, and cognitive function in early preHD.

Main Methods:

  • Acquired high-resolution 3 Tesla MRI data from 20 preHD individuals and 20 healthy controls.
  • Analyzed MRI data using voxel-based morphometry (VBM), cortical surface modeling, and subcortical segmentation.

Main Results:

  • VBM analysis showed no significant group differences.
  • Cortical surface modeling and subcortical segmentation revealed significant regional cortical thinning and striatal changes in preHD.
  • Striatal structure correlated with estimated time to motor onset and executive function, unlike cortical changes.

Conclusions:

  • A combined methodological approach to structural MRI enhances sensitivity for detecting subtle neurobiological changes in early preHD.
  • Striatal structure changes appear to be a more robust marker of Huntington's disease progression than cortical changes, correlating with clinical measures.