Neurology Medical News about premanifest persons;motor abnormalities;Huntingtons disease

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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

Results

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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