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Abstract Details

New Sensitive Measures of Executive Functioning in Premotor Huntington's Disease
Movement Disorders
S20 - (-)
002
Huntington disease is an inherited neurodegenerative disease characterized by progressive motor, cognitive, and psychiatric symptoms. Although clinical diagnosis typically is dependent on the presence of motor abnormalities, cognitive changes and caudate volume loss are often observed prior to motor diagnosis. Sensitive neuropsychological assessments are needed to identify patients in early stages of the disease and as outcome measures for neuroprotective treatment trials. Identifying problems with executive functions is particularly compelling given their association with functional decline (e.g., early unemployment). The NIH EXAMINER (Executive Abilities: Measures and Instruments for Neurobehavioral Evaluation and Research) is a new neuropsychological battery assessing executive function and designed with clinical trials in mind. It has alternate forms and produces composite scores using item response theory with excellent psychometric properties.
Fifteen patients with pdHD and 14 matched healthy controls were compared using independent samples t-tests on the NIH EXAMINER's Total Executive Composite, as well as on composites that represent subcomponents of executive functions: the Working Memory, Fluency, and Cognitive Control Composites. The Composites were correlated with 3T caudate volumes in pmHD.
The pmHD patients scored lower on the Executive Composite and on the Working Memory Composite, both ps < .001, but did not differ significantly from controls on the Fluency or Cognitive Control Composites. The Executive and Working Memory Composites correlated robustly with caudate volumes, r = .53 and r = .65 respectively.
The NIH EXAMINER Executive and Working Memory Composites are sensitive markers of cognitive changes and caudate volume size (presumed atrophy) in pmHD. Results support the idea that executive dysfunction, and particularly working memory deficits, might precede motor symptoms and are viable early disease markers.
Authors/Disclosures
Shou-Chin C. You
PRESENTER 		No disclosure on file
No disclosure on file
No disclosure on file
No disclosure on file
Sharon Sha, MD (Stanford Univeristy) Dr. Sha has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Guidepoint Global. Dr. Sha has received personal compensation in the range of $500-$4,999 for serving as a Consultant for ExpertConnect. Dr. Sha has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Sha has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Cognition Therapeutics. Dr. Sha has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Forefront Collaborative. Dr. Sha has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for ReachMD. Dr. Sha has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for PeerView. Dr. Sha has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Medscape. The institution of Dr. Sha has received research support from Biogen. The institution of Dr. Sha has received research support from Jannssen. The institution of Dr. Sha has received research support from UCB Pharma. The institution of Dr. Sha has received research support from Eisai. The institution of Dr. Sha has received research support from Eli Lilly. The institution of Dr. Sha has received research support from Aribio. Dr. Sha has received publishing royalties from a publication relating to health care.
No disclosure on file
Michael D. Geschwind, MD, PhD, FAAN (UCSF) Dr. Geschwind has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Brainstorm Cell Therapeutics, Inc.. Dr. Geschwind has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Walter Grubb. Dr. Geschwind has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Gerson Lehrman Group. Dr. Geschwind has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Reata Pharmaceuticals, Inc..
Michael S. Okun, MD, FAAN (University of Florida) Dr. Okun has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for NIH. Dr. Okun has received personal compensation in the range of $10,000-$49,999 for serving as an officer or member of the Board of Directors for Parkinson's Foundation. Dr. Okun has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for JAMA Neurology. Dr. Okun has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for NEJM Journal Watch. The institution of Dr. Okun has received research support from NIH. The institution of Dr. Okun has received research support from Parkinson's Foundation. The institution of Dr. Okun has received research support from Tourette Association of America. The institution of Dr. Okun has received research support from Michael J Fox. Dr. Okun has received publishing royalties from a publication relating to health care.
Joel Kramer, PhD (UCSF Medical Center) The institution of Dr. Kramer has received research support from tau consortium. Dr. Kramer has received publishing royalties from a publication relating to health care.
Katherine L. Possin, PhD (U of CA San Francisco, Neurology) Dr. Possin has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for ClearView Healthcare Partners. The institution of Dr. Possin has received research support from Quest Diagnostics. The institution of Dr. Possin has received research support from NIH. The institution of Dr. Possin has received research support from The Global Brain Health Institute. The institution of Dr. Possin has received research support from The Rainwater Foundation.