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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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Minimal Clinically Important Difference on Cognitive Measures for Huntington's Disease Using Function as an Anchor.

Cali M Roiboit1, Douglas R Langbehn2, Julie C Stout1

  • 1School of Psychological Sciences, Monash University, Clayton, Victoria, Australia.

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Minimally clinically important differences (MCID) for cognitive measures in Huntington's disease (HD) vary by disease stage and time. Using functional capacity anchors helps interpret cognitive changes in daily life for HD patients.

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Area of Science:

  • Neuroscience
  • Clinical Research
  • Biostatistics

Background:

  • Cognitive decline is a key feature of Huntington's disease (HD), impacting daily function.
  • Interpreting the clinical significance of cognitive changes in HD trials is challenging.
  • Functional capacity measures can serve as anchors to define minimally clinically important differences (MCID) in cognition.

Purpose of the Study:

  • To estimate MCID for common cognitive measures in HD using functional capacity anchors.
  • To compare MCID estimates derived from functional capacity versus health impairment (SF-12).

Main Methods:

  • Analysis of Enroll-HD study data from participants with Stage 2 and 3 HD.
  • Utilized linear mixed-effects regression to estimate MCID at 12, 24, and 36 months.
  • Employed the smallest measurable change in functional capacity as anchors.

Main Results:

  • Cognitive changes associated with functional decline were more pronounced with disease progression and longer timeframes (p < 0.05).
  • MCID estimates using functional capacity anchors were comparable to those using health impairment.
  • Health impairment anchors yielded larger MCID estimates at 36 months (p < 0.05).

Conclusions:

  • MCID estimates for cognitive measures in HD are dependent on disease stage and observation period.
  • Anchoring MCID on everyday functional measures enhances the interpretation of cognitive changes in HD.
  • This approach aids in understanding the real-world impact of cognitive alterations in Huntington's disease.