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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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A Fine Motor Task to Study Joint Kinematics in a Preclinical Model of Neurodegenerative Disease
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Increased cortical recruitment in Huntington's disease using a Simon task.

Nellie Georgiou-Karistianis1, Anusha Sritharan, Maree Farrow

  • 1School of Psychology, Psychiatry and Psychological Medicine, Monash University, Clayton, Victoria 3800, Australia. nellie.georgiou-karistianis@med.monash.edu.au

Neuropsychologia
|February 27, 2007
PubMed
Summary

Huntington's disease (HD) patients show altered brain activity in frontal and parietal regions during a cognitive task. This increased activation may indicate primary dysfunction or compensatory mechanisms in these areas.

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Area of Science:

  • Neuroscience
  • Cognitive Neurology
  • Neuroimaging

Background:

  • Cognitive deficits in Huntington's disease (HD) are often linked to striatal degeneration.
  • However, studies indicate broader morphological brain changes beyond the striatum in HD patients.

Purpose of the Study:

  • To investigate the impact of HD on brain activation patterns in regions outside the striatum.
  • To compare functional magnetic resonance imaging (fMRI) activation between HD patients and healthy controls during a cognitive task.

Main Methods:

  • Utilized fMRI on 20 mild to moderate stage HD patients and 17 healthy controls.
  • Employed a Simon task involving congruent and incongruent arrow stimuli requiring button press responses.
  • Analyzed data using statistical parametric mapping with a random effects model to identify group differences in brain activation.

Main Results:

  • HD patients exhibited recruitment of frontal and parietal cortical regions during the task.
  • Significantly greater activation was observed in HD patients compared to controls in areas including the caudal anterior cingulate, insula, inferior parietal lobules, and superior temporal gyrus.

Conclusions:

  • Increased activation in the anterior cingulate-frontal-motor-parietal cortex in HD may stem from direct cortical damage.
  • Alternatively, this heightened activation could represent a compensatory response to underlying striatal deficits in Huntington's disease.