Research Article

Motor abnormalities in premanifest persons with Huntington's disease: The PREDICT-HD study†

  1. Kevin M. Biglan MD, MPH1,
  2. Christopher A. Ross MD, PhD2,
  3. Douglas R. Langbehn MD, PhD3,
  4. Elizabeth H. Aylward PhD4,
  5. Julie C. Stout PhD5,
  6. Sarah Queller PhD6,
  7. Noelle E. Carlozzi PhD7,
  8. Kevin Duff PhD3,
  9. Leigh J. Beglinger PhD3,
  10. Jane S. Paulsen PhD3,*

Article first published online: 26 JUN 2009

DOI: 10.1002/mds.22601

Issue

Movement Disorders

Movement Disorders

Volume 24, Issue 12, pages 1763–1772, 15 September 2009

Additional Information

How to Cite

Biglan, K. M., Ross, C. A., Langbehn, D. R., Aylward, E. H., Stout, J. C., Queller, S., Carlozzi, N. E., Duff, K., Beglinger, L. J. and Paulsen, J. S. (2009), Motor abnormalities in premanifest persons with Huntington's disease: The PREDICT-HD study. Movement Disorders, 24: 1763–1772. doi: 10.1002/mds.22601

Author Information

  1. 1

    University of Rochester, Rochester, New York, USA

  2. 2

    Johns Hopkins University, Baltimore, Maryland, USA

  3. 3

    University of Iowa, Iowa City, Iowa, USA

  4. 4

    University of Washington, Seattle, Washington, USA

  5. 5

    Monash University, Victoria, Australia

  6. 6

    Indiana University, Bloomington, Indiana, USA

  7. 7

    Kessler Medical Rehabilitation Research and Education Center, West Orange, New Jersey, USA

*University of Iowa, College of Medicine, 1-305 MEB, Iowa City, IA 52242

  1. †

    Potential conflict of interest: Nothing to report.

Publication History

  1. Issue published online: 11 SEP 2009
  2. Article first published online: 26 JUN 2009
  3. Manuscript Accepted: 17 MAR 2009
  4. Manuscript Revised: 9 FEB 2009
  5. Manuscript Received: 9 DEC 2008

Funded by

  • National Institutes of Health. Grant Number: NS40068
  • CHDI, Inc
  • Douglas Langbehn at The University of Iowa

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Keywords:

  • Huntington's disease;
  • at-risk;
  • UHDRS

Abstract

The PREDICT-HD study seeks to identify clinical and biological markers of Huntington's disease in premanifest individuals who have undergone predictive genetic testing. We compared baseline motor data between gene-expansion carriers (cases) and nongene-expansion carriers (controls) using t-tests and Chi-square. Cases were categorized as near, mid, or far from diagnosis using a CAG-based formula. Striatal volumes were calculated using volumetric magnetic resonance imaging measurements. Multiple linear regression associated total motor score, motor domains, and individual motor items with estimated diagnosis and striatal volumes. Elevated total motor scores at baseline were associated with higher genetic probability of disease diagnosis in the near future (partial R2 0.14, P < 0.0001) and smaller striatal volumes (partial R2 0.15, P < 0.0001). Nearly all motor domain scores showed greater abnormality with increasing proximity to diagnosis, although bradykinesia and chorea were most highly associated with diagnostic immediacy. Among individual motor items, worse scores on finger tapping, tandem gait, Luria, saccade initiation, and chorea show unique association with diagnosis probability. Even in this premanifest population, subtle motor abnormalities were associated with a higher probability of disease diagnosis and smaller striatal volumes. Longitudinal assessment will help inform whether motor items will be useful measures in preventive clinical trials. © 2009 Movement Disorder Society

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