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Related Concept Videos

Huntington Disease l: Introduction01:21

Huntington Disease l: Introduction

166
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...
166

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Whole-brain Segmentation and Change-point Analysis of Anatomical Brain MRI—Application in Premanifest Huntington's Disease
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Functional changes during working memory in Huntington's disease: 30-month longitudinal data from the IMAGE-HD study.

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Area of Science:

  • Neuroscience
  • Neuroimaging
  • Neurodegenerative Diseases

Background:

  • Huntington's disease (HD) is a progressive neurodegenerative disorder.
  • Early detection of HD is crucial for potential interventions.
  • Functional brain changes precede clinical symptoms in HD.

Purpose of the Study:

  • To investigate longitudinal changes in brain activation and connectivity during working memory in premanifest and symptomatic Huntington's disease.
  • To identify early biomarkers of HD progression.

Main Methods:

  • Longitudinal case-control study over 30 months.
  • Functional magnetic resonance imaging (fMRI) during working memory tasks (1-BACK and 2-BACK).
  • Analysis of blood-oxygen-level-dependent (BOLD) activity and functional connectivity between dorsolateral prefrontal cortex (DLPFC) and caudate.

Main Results:

  • Pre-HD group showed increased longitudinal activation in DLPFC and frontal cortex (1-BACK) and further increased activation in caudate, putamen, and temporal cortex (2-BACK).
  • Symptomatic HD group showed no significant longitudinal changes compared to controls.
  • Pre-HD group exhibited decreased functional connectivity between DLPFC and caudate over time.
  • Longitudinal changes in pre-HD correlated with disease burden and years to onset.

Conclusions:

  • Longitudinal changes in brain activity during working memory occur before clinical HD onset.
  • Increased prefrontal cortex activation in pre-HD may represent an early compensatory mechanism.
  • These findings suggest potential early biomarkers for Huntington's disease.