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

Kevin M. Biglan, Christopher A. Ross, Douglas R. Langbehn, Elizabeth H. Aylward, Julie C. Stout, Sarah Queller, Noelle E. Carlozzi, Kevin Duff, Leigh J. Beglinger, Jane S. Paulsen, Hans Johnson, Karl Kieburtz, David Oakes, Ira Shoulson, Mark Guttman, Michael Hayden, Bernhard G. Landwehrmeyer, Martha Nance

Research output: Contribution to journalArticlepeer-review

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 geneexpansion 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 R 2 0.14, P < 0.0001) and smaller striatal volumes (partial R 2 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.

Original languageEnglish (US)
Pages (from-to)1763-1772
Number of pages10
JournalMovement Disorders
Volume24
Issue number12
DOIs
StatePublished - Sep 15 2009

Keywords

  • At-risk
  • Huntington's disease
  • UHDRS

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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