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

Elizabeth H Aylward, Deborah L Harrington, James A Mills, Peggy C Nopoulos, Jeffrey D Long, Dawei Liu, Holly K Westervelt, Jane S Paulsen, Stephen Cross (Collaborator), Patricia Ryan (Collaborator), Eric A Epping (Collaborator), Edmund Chiu (Collaborator), Joy Preston (Collaborator), Anita Goh (Collaborator), Stephanie Antonopoulos (Collaborator), Samantha Loi (Collaborator), Lynn Raymond (Collaborator), Joji Decolongon (Collaborator), Mannie Fan (Collaborator), Allison Coleman (Collaborator)William M Mallonee (Collaborator), Greg Suter (Collaborator), Christopher A Ross (Collaborator), Mark Varvaris (Collaborator), Nadine Yoritomo (Collaborator), Elizabeth McCusker (Collaborator), Jane Griffith (Collaborator), Clement Loy (Collaborator), David Gunn (Collaborator), Mark Guttman (Collaborator), Alanna Sheinberg (Collaborator), Albie Law (Collaborator), Kimberly Quaid (Collaborator), Melissa Wesson (Collaborator), Joanne Wojcieszek (Collaborator), Joel Perlmutter (Collaborator), Stacey Barton (Collaborator), Shineeka Smith (Collaborator), Roger A Barker (Collaborator), Sarah Mason (Collaborator), Natalie Valle Guzman (Collaborator), Susan Perlman (Collaborator), Brian Clemente (Collaborator), Randi Jones (Collaborator), Cathy Wood-Siverio (Collaborator), Stewart A Factor (Collaborator), Ali Samii (Collaborator), Alma Macaraeg (Collaborator), Peter Panegyres (Collaborator), Joseph Lee (Collaborator), Maria Tedesco (Collaborator), Brenton Maxwell (Collaborator), Rajeev Kumar (Collaborator), Diane Erickson (Collaborator), Breanna Nickels (Collaborator), Frederick Marshall (Collaborator), Amy Chesire (Collaborator), Mary Wodarski (Collaborator), Charlyne Hickey (Collaborator), Michael D Geschwind (Collaborator), Sharon Sha (Collaborator), Gabriela Satris (Collaborator), Anwar Ahmed (Collaborator), Christine Reece (Collaborator), Alex Bura (Collaborator), Lyla Mourany (Collaborator), Jagan Pallai (Collaborator), Pietro Mazzoni (Collaborator), Karen Marder (Collaborator), Paula Wasserman (Collaborator), David Craufurd (Collaborator), Judith Bek (Collaborator), Elizabeth Howard (Collaborator), Tom Warner (Collaborator), Maggie Burrows (Collaborator), Michael Orth (Collaborator), Sigurd Süssmuth (Collaborator), Katrin Barth (Collaborator), Sonja Trautmann (Collaborator), Daniela Schwenk (Collaborator), Carolin Eschenbach (Collaborator), Vicki Wheelock (Collaborator), Lisa Kjer (Collaborator), Amanda Martin (Collaborator), Sarah Farias (Collaborator), Zosia Miedzybrodzka (Collaborator), Daniela Rae (Collaborator), Mariella D'Alessandro (Collaborator), Oksana Suchowersky (Collaborator), Phyllis Chua (Collaborator), Angela Komiti (Collaborator), Diana Rosas (Collaborator), Anne Rosser (Collaborator), Kathy Price (Collaborator), Sarah Hunt (Collaborator), Joseph Jankovic (Collaborator), William Ondo (Collaborator), Wayne Martin (Collaborator), Pamela King (Collaborator), Marguerite Wieler (Collaborator), Satwinder Sran (Collaborator), Martha Nance (Collaborator), Justo Garcia De Yebenes (Collaborator), Richard Dubinsky (Collaborator)

    Research output: Contribution to journalArticlepeer-review


    BACKGROUND: Neuroimaging studies suggest that volumetric MRI measures of specific brain structures may serve as excellent biomarkers in future clinical trials of Huntington disease (HD). OBJECTIVE: Demonstration of the clinical significance of these measures is an important step in determining their appropriateness as potential outcome measures. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.
    Original languageEnglish
    JournalJournal of Huntington's disease
    Issue number4
    Publication statusPublished - 2013


    • Huntington disease
    • cognitive
    • magnetic resonance imaging
    • motor
    • psychiatric


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