Session Information
Date: Tuesday, June 6, 2017
Session Title: Huntington's Disease
Session Time: 1:45pm-3:15pm
Location: Exhibit Hall C
Objective: To utilize the UCSD Huntington’s Disease Behavioral Questionnaire (HD-BQ) to assess behavioral changes that distinguish HD gene carriers transitioning to manifest HD.
Background: Behavioral changes, including apathy, depression, irritability, anxiety, and difficulty prioritizing and initiating activities, are characteristic features of HD and may serve as an early marker of disease onset. The UCSD HD-BQ consists of 30 items with a 3-point rating scale (0-3), and takes about 5 minutes to complete. The maximum score is 90 with higher scores indicating greater severity.
Methods: Gene positive subjects (n=118) were stratified using the UHDRS Total Functional Capacity and Penny Burden of Pathology score as early pre-manifest (EPM); transitional (i.e. individuals close to disease onset or with very mild HD); or mild-moderate manifest HD. The UCSD HD-BQ was administered to 22 normal control (NC); 29 EPM; 27 transitional, and 62 HD subjects. We used one-way ANOVA/Tukey Posthoc to compare groups on dependent variables and effect sizes to quantify the mean difference between each group.
Results: Mean MMSE, TFC, and TMS scores were 28.9, 12.8, and 1.7 respectively for NC; 28.2, 13.0, and 1.6 respectively for EPM; 27.3, 12.4, and 7.6 respectively for transitional; and 24.1, 8.2, and 35.6 respectively for manifest HD subjects. Although statistically significant differences were not seen between transitional or EPM subjects and NC with regard to these cognitive, functional and motoric measures, statistically significant differences were seen with regard to the HD-BQ between transitional (mean HD-BQ=32.3; p=0.000) and, more surprisingly, EPM (mean HD-BQ=22.8, p=0.011) subjects and NC (mean HD-BQ=5.7). HD showed the largest effect size (0.620) compared to transitional (0.611) and EPM (0.481) subjects. Highest scoring symptoms among transitional and EPM subjects comprised anxiety, difficulty concentrating, and memory.
Conclusions: The HD-BQ is a brief, reliable instrument for screening behavioral changes in individuals at risk for, and transitioning to, HD. Behavioral disturbances are important in HD and may serve as an early marker of disease onset.
To cite this abstract in AMA style:
S. Park, S. Nam, A. Nathan, A. Haque, A. Haque, P. Gilbert, J. Corey-Bloom. Behavioral Changes on the UCSD Huntington’s Disease Behavioral Questionnaire (HD-BQ) Distinguish Patients Transitioning to Manifest Huntington’s disease (HD) [abstract]. Mov Disord. 2017; 32 (suppl 2). https://www.mdsabstracts.org/abstract/behavioral-changes-on-the-ucsd-huntingtons-disease-behavioral-questionnaire-hd-bq-distinguish-patients-transitioning-to-manifest-huntingtons-disease-hd/. Accessed May 9, 2025.« Back to 2017 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/behavioral-changes-on-the-ucsd-huntingtons-disease-behavioral-questionnaire-hd-bq-distinguish-patients-transitioning-to-manifest-huntingtons-disease-hd/