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John L. Dart Library
Closed for Maintenance
Phone: (843) 722-7550
West Ashley Library
9 a.m. - 5 p.m.
Phone: (843) 766-6635
Folly Beach Library
9 a.m. - 2 p.m.
*open the 2nd and 4th Saturday
*open the 2nd and 4th Saturday
Phone: (843) 588-2001
Edgar Allan Poe/Sullivan's Island Library
Closed for renovations
Phone: (843) 883-3914
Wando Mount Pleasant Library
9 a.m. - 5 p.m.
Phone: (843) 805-6888
Village Library
9 a.m. - 1 p.m.
Phone: (843) 884-9741
St. Paul's/Hollywood Library
9 a.m. - 5 p.m.
Phone: (843) 889-3300
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9 a.m. - 5 p.m.
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Biological and clinical manifestations of Huntington's disease in the longitudinal TRACK-HD study: cross-sectional analysis of baseline data
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- Author(s): Tabrizi, Sarah J1 ; Langbehn, Douglas R2; Leavitt, Blair R3; Roos, Raymund AC4; Durr, Alexandra5; Craufurd, David6; Kennard, Christopher7; Hicks, Stephen L7; Fox, Nick C1; Scahill, Rachael I1; Borowsky, Beth8; Tobin, Allan J8; Rosas, H Diana9; Johnson, Hans10; Reilmann, Ralf11; Landwehrmeyer, Bernhard12; Stout, Julie C13,14; Paulsen, Jane S (AUTHOR)
- Source:
Lancet Neurology. Sep2009, Vol. 8 Issue 9, p791-801. 11p.- Subject Terms:
*HUNTINGTON disease; *NEURODEGENERATION; *ADULTS; *LONGITUDINAL method; *MIDDLE age; *CROSS-sectional method; *BIOMARKERS; *GENETIC mutation; *HUNTINGTON'S chorea diagnosis; *ACADEMIC medical centers; *BRAIN; *HEALTH care teams; *MEDICAL cooperation; *QUESTIONNAIRES; *RESEARCH; *RESEARCH funding; *PREDICTIVE tests; *BLIND experiment; *DISEASE progression; *GENETIC carriers; *PROGNOSIS - Source:
- Additional Information
- Subject Terms:
- Abstract: Summary: 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 volume, regional grey and white matter differences, impairment in a range of voluntary neurophysiological motor, and oculomotor tasks, and cognitive and neuropsychiatric dysfunction in premanifest HD gene carriers with normal motor scores through to early clinical stage 2 disease. Interpretation: We show the feasibility of rapid data acquisition and the use of multi-site 3T MRI and neurophysiological motor measures in a large multicentre study. Our results provide evidence for quantifiable biological and clinical alterations in HTT expansion carriers compared with age-matched controls. Many parameters differ from age-matched controls in a graded fashion and show changes of increasing magnitude across our cohort, who range from about 16 years from predicted disease diagnosis to early HD. These findings might help to define novel quantifiable endpoints and methods for rapid and reliable data acquisition, which could aid the design of therapeutic trials. Funding: CHDI/High Q Foundation. [Copyright &y& Elsevier]
- Abstract: Copyright of Lancet Neurology is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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