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Related Experiment Videos

Functional decline in Huntington's disease

A Feigin1, K Kieburtz, K Bordwell

  • 1Department of Neurology, University of Rochester Medical Center, NY 14642-8673, USA.

Movement Disorders : Official Journal of the Movement Disorder Society
|March 1, 1995
PubMed
Summary
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Huntington's disease (HD) causes functional decline at a rate of 0.63 units per year. Motor symptoms like chorea decrease as dystonia increases during illness progression.

Area of Science:

  • Neurology
  • Neuroscience
  • Genetics

Background:

  • Huntington's disease (HD) is a progressive neurodegenerative disorder.
  • Understanding the rate and clinical correlates of functional decline is crucial for patient management.

Purpose of the Study:

  • To determine the annual rate of functional decline in patients with manifest Huntington's disease.
  • To identify clinical features associated with the progression of functional impairment.

Main Methods:

  • Prospective evaluation of 129 patients with manifest Huntington's disease.
  • Assessment of functional capacity using the HD Functional Capacity Scale.
  • Evaluation of motor performance with the HD Rating Scale in a subset of patients.

Main Results:

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  • Total Functional Capacity declined at an average rate of 0.63 units per year.
  • Worsening functional capacity correlated with decreased chorea and increased dystonia.
  • No correlation was found between functional decline rate and age at onset, body weight, parental gender, or neuroleptic history.

Conclusions:

  • Functional capacity in Huntington's disease declines steadily over time.
  • Specific motor symptoms change in relation to functional decline.
  • Several patient-specific factors do not predict the rate of functional deterioration.