Using the Huntington's Disease-Behavioral Questionnaire (HD-BQ) as a Screening Tool for Behavioral Disturbances in HD

Objective: To examine the usefulness of a new behavioral questionnaire for patients with, and at risk for, Huntington’s disease (HD). Background: Behavioral changes are characteristic features of HD and often the most distressing aspect of the disease for families and caregivers. Design/Methods: The HD-BQ, Mini-Mental State Examination (MMSE), Montreal Cognitive Assessment (MoCA), Unified Huntington’s Disease Rating Scale (UHDRS) Total Functional Capacity (TFC) and Total Motor Score (TMS), Problem Behaviors Assessment-Short (PBA-s), and Hospital Anxiety and Depression Scale (HADS) were administered to 98 HD, 66 pre-manifest HD (PM), and 106 control (NC) subjects. The HD-BQ was also administered to 61 HD caregivers. Results: The HD-BQ discriminated between HD (mean 31.4), PM (mean 22.7) and NC (mean 15.3) subjects (p Conclusions: The HD-BQ is a rapidly administered instrument for screening behavioral changes in individuals with, and at risk for, HD. It can be used to interrogate patients and caregivers with regard to behavior. It discriminates well between NC, PM, and HD subjects, and compares favorably to the current gold standards for behavioral assessment on ROC analysis. Behavioral changes are a possible early marker for HD onset. 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 personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Genentech, Teva, Serono, and Alexion. Dr. Corey-Bloom has received research support from Vaccinex, Wave Life Sciences, and CHDI. Dr. Wong has nothing to disclose. Dr. Aboufadel has nothing to disclose. Dr. Snell has nothing to disclose. Dr. Castleton has nothing to disclose. Dr. Smith has nothing to disclose. Dr. Wright has nothing to disclose. Dr. Gilbert has nothing to disclose.