Baseline Variables Associated with Functional Decline in 2CARE, A Randomized Clinical Trial in Huntington's Disease

J Huntingtons Dis. 2020;9(1):47-58. doi: 10.3233/JHD-190391.


Background: Despite the clearly recognized progressive functional decline of Huntington's disease (HD), detailed investigations of factors associated with the rate of functional progression are limited.

Objective: Understanding factors associated with functional decline through examination of existing HD clinical databases may improve efforts to mitigate it.

Methods: We analyzed data from 2CARE, a randomized clinical trial with up to 5 years of follow-up, to assess potential risk factors for more rapid functional decline in HD.

Results: Variables associated with faster functional decline included worse motor performance, worse cognitive test scores, female sex, lower weight and body mass index, and a higher CAG repeat length, especially in younger people.

Conclusion: While our data are limited to the structured environment and homogeneity of a clinical trial, attention to several of the identified risk factors may be useful towards managing functional decline over time. The observation that women progress faster than men, while potentially confounded by an association between sex and weight, deserves further study.

Keywords: Huntington’s disease; clinical trial; functional decline; total functional capacity.

Publication types

  • Randomized Controlled Trial
  • Research Support, N.I.H., Extramural

MeSH terms

  • Adult
  • Age Factors
  • Body Mass Index
  • Body Weight / physiology
  • Cognitive Dysfunction / etiology
  • Cognitive Dysfunction / physiopathology
  • Disease Progression*
  • Female
  • Follow-Up Studies
  • Humans
  • Huntington Disease / epidemiology
  • Huntington Disease / physiopathology*
  • Male
  • Middle Aged
  • Motor Activity / physiology
  • Risk Factors
  • Severity of Illness Index*
  • Sex Factors
  • Trinucleotide Repeats / genetics