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Motor abnormalities in premanifest persons with Huntington's disease: The PREDICT-HD study
Movement Disorders, 07/01/09
Biglan KM et al. – Even in a premanifest population, subtle motor abnormalities were associated with a higher probability of disease diagnosis and smaller striatal volumes. Longitudinal assessment must determine whether motor items can serve as useful measures in preventive clinical trials.
Methods- PREDICT-HD study: identify clinical and biological markers of Huntington's disease in premanifest pts who had predictive genetic testing
- Comparison of baseline motor data between gene-expansion carriers (cases) and nongene-expansion carriers (controls) using t-tests and Chi-square
- Cases categorized as near, mid, or far from diagnosis using a CAG-based formula
- Striatal volumes 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 associated with higher genetic probability of disease diagnosis in near future and smaller striatal volumes
- Nearly all motor domain scores showed greater abnormality with increasing proximity to diagnosis; bradykinesia and chorea 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
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