作者
Sarah J Tabrizi, Douglas R Langbehn, Blair R Leavitt, Raymund AC Roos, Alexandra Durr, David Craufurd, Christopher Kennard, Stephen L Hicks, Nick C Fox, Rachael I Scahill, Beth Borowsky, Allan J Tobin, H Diana Rosas, Hans Johnson, Ralf Reilmann, Bernhard Landwehrmeyer, Julie C Stout
发表日期
2009/9/1
期刊
The Lancet Neurology
卷号
8
期号
9
页码范围
791-801
出版商
Elsevier
简介
Background
Huntington's disease (HD) is an autosomal dominant, fully penetrant, neurodegenerative disease that most commonly affects adults in mid-life. Our aim was to identify sensitive and reliable biomarkers in premanifest carriers of mutated HTT and in individuals with early HD that could provide essential methodology for the assessment of therapeutic interventions.
Methods
This multicentre study uses an extensive battery of novel assessments, including multi-site 3T MRI, clinical, cognitive, quantitative motor, oculomotor, and neuropsychiatric measures. Blinded analyses were done on the baseline cross-sectional data from 366 individuals: 123 controls, 120 premanifest (pre-HD) individuals, and 123 patients with early HD.
Findings
The first participant was enrolled in January, 2008, and all assessments were completed by August, 2008. Cross-sectional analyses identified significant changes in whole-brain …
引用总数
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