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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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Behavioral Assessment of Manual Dexterity in Non-Human Primates
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Objectively characterizing Huntington's disease using a novel upper limb dexterity test.

Samuel Woodgate1,2, Philippa Morgan-Jones1,3, Susanne Clinch4

  • 1Cardiff School of Engineering, Cardiff University, Cardiff, United Kingdom.

Journal of Neurology
|February 8, 2021
PubMed
Summary

The Clinch Token Transfer Test (C3t) accurately predicts Huntington's disease severity and impairment. This simple, objective measure supports remote monitoring and clinical trial screening.

Keywords:
Convergent validityHuntington’s diseaseOutcome measureRegressionUpper-limb function

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Area of Science:

  • Neuroscience
  • Clinical Assessment
  • Biomedical Engineering

Background:

  • Huntington's disease (HD) is a progressive neurodegenerative disorder.
  • Objective and reliable assessment tools are needed to track HD progression.
  • The Clinch Token Transfer Test (C3t) is a novel bi-manual task designed to assess motor and cognitive function.

Purpose of the Study:

  • To evaluate the concurrent and convergent validity of the C3t.
  • To determine if the C3t can objectively reflect Huntington's disease severity.
  • To assess the C3t's utility as a standalone measure without requiring clinical expertise.

Main Methods:

  • 105 participants with pre-manifest or manifest HD completed the C3t and Unified Huntington's Disease Rating Scale (UHDRS).
  • Regression analyses estimated clinical scores from C3t scores.
  • Spearman correlations and scatterplots assessed C3t's association with disease severity.
  • Longitudinal changes were analyzed over 12 months in 33 participants.

Main Results:

  • Baseline C3t scores accurately predicted baseline clinical scores (9-13% accuracy).
  • C3t scores demonstrated significant associations with individual and composite UHDRS scores.
  • Changes in C3t scores over 12 months mirrored changes in clinical measures.

Conclusions:

  • The C3t is a promising, simple, and objective outcome measure for Huntington's disease.
  • It accurately reflects disease severity and supports remote clinical monitoring.
  • The C3t may also serve as a screening tool for clinical trial recruitment.