Authors
Jane S Paulsen, Jeffrey D Long, Christopher A Ross, Deborah L Harrington, Cheryl J Erwin, Janet K Williams, Holly James Westervelt, Hans J Johnson, Elizabeth H Aylward, Ying Zhang, H Jeremy Bockholt, Roger A Barker
Publication date
2014/12/1
Journal
The Lancet Neurology
Volume
13
Issue
12
Pages
1193-1201
Publisher
Elsevier
Description
Background
Although the association between cytosine-adenine-guanine (CAG) repeat length and age at onset of Huntington's disease is well known, improved prediction of onset would be advantageous for clinical trial design and prognostic counselling. We compared various measures for tracking progression and predicting conversion to manifest Huntington's disease.
Methods
In this prospective observational study, we assessed the ability of 40 measures in five domains (motor, cognitive, psychiatric, functional, and imaging) to predict time to motor diagnosis of Huntington's disease, accounting for CAG repeat length, age, and the interaction of CAG repeat length and age. Eligible participants were individuals from the PREDICT-HD study (from 33 centres in six countries [USA, Canada, Germany, Australia, Spain, UK]) with the gene mutation for Huntington's disease but without a motor diagnosis (a rating below 4 …
Total citations
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