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Regional atrophy associated with cognitive and motor function in prodromal Huntington disease
Journal article   Peer reviewed

Regional atrophy associated with cognitive and motor function in prodromal Huntington disease

Elizabeth H Aylward, Deborah L Harrington, James A Mills, Peggy C Nopoulos, Christopher A Ross, Jeffrey D Long, Dawei Liu, Holly K Westervelt, Jane S Paulsen and PREDICT-HD Investigators and Coordinators of the Huntington Study Group
Journal of Huntington's disease, Vol.2(4), pp.477-489
2013
DOI: 10.3233/JHD-130076
PMCID: PMC4412155
PMID: 25062732
url
http://doi.org/10.3233/JHD-130076View
Open Access

Abstract

Neuroimaging studies suggest that volumetric MRI measures of specific brain structures may serve as excellent biomarkers in future clinical trials of Huntington disease (HD). Demonstration of the clinical significance of these measures is an important step in determining their appropriateness as potential outcome measures. Measures of gray- and white-matter lobular volumes and subcortical volumes (caudate, putamen, globus pallidus, thalamus, nucleus accumbens, hippocampus) were obtained from MRI scans of 516 individuals who tested positive for the HD gene expansion, but were not yet exhibiting signs or symptoms severe enough to warrant diagnosis ("pre-HD"). MRI volumes (corrected for intracranial volume) were correlated with cognitive, motor, psychiatric, and functional measures known to be sensitive to subtle changes in pre-HD. Caudate, putamen, and globus pallidus volumes consistently correlated with cognitive and motor, but not psychiatric or functional measures in pre-HD. Volumes of white matter, nucleus accumbens, and thalamus, but not cortical gray matter, also correlated with some of the motor and cognitive measures. Results of regression analyses suggest that volumes of basal ganglia structures contributed more highly to the prediction of most motor and cognitive variables than volumes of other brain regions. These results support the use of volumetric measures, especially of the basal ganglia, as outcome measures in future clinical trials in pre-HD. Results may also assist investigators in selecting the most appropriate measures for treatment trials that target specific clinical features or regions of neuropathology.
Magnetic Resonance Imaging Psychomotor Performance Humans Huntington Disease - pathology Atrophy - pathology Cognition Disorders - pathology Image Interpretation, Computer-Assisted Male Cognition Prodromal Symptoms Huntington Disease - complications Neuropsychological Tests Cognition Disorders - etiology Brain - pathology Adult Female

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