Screening for Behavioral Disturbances in Huntington's Disease (HD) with the Huntington's Disease-Behavioral Questionnaire (HD-BQ) (P1.056)

Objective: To examine the usefulness of a new behavioral questionnaire for patients with, and at risk for, Huntingon’s disease (HD). Background: Behavioral changes, including apathy, depression, anxiety, and difficulty prioritizing and initiating activities, are characteristic features of HD. For families and caregivers, they are often the most distressing aspect of the disease. There is evidence that behavioral manifestations may predate motor signs by a decade in HD. The assessment of these behavioral disturbances is therefore important. Methods: The UCSD HD-BQ was administered to 65 HD, 35 at-risk gene positive (AR+), and 22 normal control (NC) subjects at our Center. The UCSD HD-BQ was also administered to 37 HD caregivers. We used Cronbach9s alpha analysis to examine the internal consistency of the HD-BQ components; One-Way ANOVA/Tukey Posthoc to compare groups on dependent variables; Pearson product-moment correlations to compare HD-BQ scores with other variables. Results: The HD-BQ showed substantial internal consistency (Cronbach α = 0.94). It showed construct validity with high-very high correlations with other behavioral measures (PBA r=0.65; HADS r=0.76), and poor correlations with measures of cognitive functioning and motor impairment (r=-0.01 to -0.10 and 0.01, respectively). The HD-BQ discriminated between HD (mean 30.8), preHD (mean 27.6), and control subjects (mean 8.1) (p=0.000). When questionnaires were completed independently by 37 HD subject/caregiver pairs, there was a discrepancy in the perception of impairment between patients and their caregivers, with caregivers identifying more behavioral changes (mean difference=16.22 points). Items with the largest discrepancies included less insight with regard to symptoms, difficulty concentrating, loss of interest in previously enjoyed activities, and difficulty initiating activities. Conclusions: The HD-BQ is a quick, reliable instrument for screening behavioral changes in patients with, and at risk for, HD. When possible, information regarding behavior should be corroborated by an informant since HD patients tend to underestimate their impairment. Disclosure: Dr. Corey-Bloom has received research support from PRANA, Omeros, Teva, HSG, and CHDI. Dr. Nam has nothing to disclose. Dr. Herndon has nothing to disclose. Dr. Park has nothing to disclose. Dr. Haque has nothing to disclose. Dr. Gilbert has nothing to disclose.