Journal article
Detection of Huntington’s disease decades before diagnosis: the Predict-HD study
Journal of neurology, neurosurgery and psychiatry, Vol.79(8), pp.874-880
08/2008
DOI: 10.1136/jnnp.2007.128728
PMCID: PMC2569211
PMID: 18096682
Abstract
Objective: The objective of the Predict-HD study is to use genetic, neurobiological and refined clinical markers to understand the early progression of Huntington’s disease (HD), prior to the point of traditional diagnosis, in persons with a known gene mutation. Here we estimate the approximate onset and initial course of various measurable aspects of HD relative to the time of eventual diagnosis. Methods: We studied 438 participants who were positive for the HD gene mutation, but did not yet meet the diagnostic criteria for HD and had no functional decline. Predictability of baseline cognitive, motor, psychiatric and imaging measures was modelled non-linearly using estimated time until diagnosis (based on CAG repeat length and current age) as the predictor. Results: Estimated time to diagnosis was related to most clinical and neuroimaging markers. The patterns of association suggested the commencement of detectable changes one to two decades prior to the predicted time of clinical diagnosis. The patterns were highly robust and consistent, despite the varied types of markers and diverse measurement methodologies. Conclusions: These findings from the Predict-HD study suggest the approximate time scale of measurable disease development, and suggest candidate disease markers for use in preventive HD trials.
Details
- Title: Subtitle
- Detection of Huntington’s disease decades before diagnosis: the Predict-HD study
- Creators
- J S Paulsen - University of Iowa, The Roy J and Lucille A Carver College of Medicine, Iowa City, Iowa, USAD R Langbehn - University of Iowa, The Roy J and Lucille A Carver College of Medicine, Iowa City, Iowa, USAJ C Stout - Indiana University, Department of Psychology, Bloomington, Indiana, USAE Aylward - University of Washington, Department of Radiology, Seattle, Washington, USAC A Ross - Johns Hopkins University School of Medicine, Department of Psychiatry, Division of Neurobiology, Baltimore, Maryland, USAM Nance - Park Nicollet Clinic, Department of Neurosciences, St Louis Park, Minnesota, USAM Guttman - Center for Movement Disorders, Markham, Ontario, CanadaS Johnson - Indiana University, Department of Psychology, Bloomington, Indiana, USAM MacDonald - Massachusetts General Hospital, Neuroscience Center, Boston, Massachusetts, USAL J Beglinger - University of Iowa, The Roy J and Lucille A Carver College of Medicine, Iowa City, Iowa, USAK Duff - University of Iowa, The Roy J and Lucille A Carver College of Medicine, Iowa City, Iowa, USAE Kayson - University of Rochester, Clinical Trials Coordination Center, Rochester, New York, USAK Biglan - University of Rochester, Clinical Trials Coordination Center, Rochester, New York, USAI Shoulson - University of Rochester, Clinical Trials Coordination Center, Rochester, New York, USAD Oakes - University of Rochester Medical Center, Department of Biostatistics, Rochester, New York, USAM Hayden - University of British Columbia, Medical Genetics, Vancouver, British Columbia, CanadaPredict-HD Investigators and Coordinators of the Huntington Study Group
- Resource Type
- Journal article
- Publication Details
- Journal of neurology, neurosurgery and psychiatry, Vol.79(8), pp.874-880
- DOI
- 10.1136/jnnp.2007.128728
- PMID
- 18096682
- PMCID
- PMC2569211
- NLM abbreviation
- J Neurol Neurosurg Psychiatry
- ISSN
- 0022-3050
- eISSN
- 1468-330X
- Language
- English
- Date published
- 08/2008
- Academic Unit
- Psychiatry; Psychological and Brain Sciences; Iowa Neuroscience Institute
- Record Identifier
- 9984003959602771
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