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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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Related Experiment Video

Updated: May 7, 2026

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

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Published on: June 9, 2018

Longitudinal task-negative network analyses in preclinical Huntington's disease.

Robert Christian Wolf1, Fabio Sambataro, Nenad Vasic

  • 1Center for Psychosocial Medicine, Department of General Psychiatry, University of Heidelberg, Voßstraße 4, 69115, Heidelberg, Germany, christian.wolf@med.uni-heidelberg.de.

European Archives of Psychiatry and Clinical Neuroscience
|September 28, 2013
PubMed
Summary

Huntington's disease (HD) gene mutation carriers show stable task-negative network connectivity changes over two years. These neural alterations in preclinical HD may reflect disease progression and motor function changes.

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Area of Science:

  • Neuroscience
  • Neuroimaging
  • Genetics

Background:

  • Preclinical Huntington's disease (preHD) shows altered task-related brain activation.
  • Task-negative network (TNN) deactivation patterns and their stability in preHD are largely unknown.
  • Understanding these changes is crucial for tracking disease progression.

Purpose of the Study:

  • To investigate task-related deactivation and TNN functional connectivity in preHD over two years.
  • To assess the stability of these neural patterns and their relationship with disease burden and motor function.

Main Methods:

  • Functional magnetic resonance imaging (fMRI) was used in 13 preHD individuals and 13 controls.
  • A verbal working memory task was employed.
  • Uni- and multivariate analyses examined TNN functional connectivity at baseline and 2-year follow-up.

Main Results:

  • Behavioral performance remained similar between groups and stable over time.
  • Task-related deactivation was comparable between preHD and controls at both time points.
  • PreHD showed altered functional connectivity in TNNs: lower in posterior cingulate cortex, higher in left anterior prefrontal cortex, with these changes being stable over 2 years.
  • Anterior prefrontal connectivity correlated with disease burden.
  • Over time, preHD exhibited increased dorsal cingulate connectivity, inversely associated with motor function changes.

Conclusions:

  • This study provides the first evidence of stable TNN connectivity alterations in preHD over a 2-year period.
  • Altered anterior prefrontal and dorsal cingulate connectivity in preHD may serve as sensitive biomarkers for disease progression and motor function.
  • Task-negative activity patterns offer insights into time-sensitive neural and functional changes in preclinical Huntington's disease.