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Abstract Details

Using the Huntington's Disease-Behavioral Questionnaire (HD-BQ) as a Screening Tool for Behavioral Disturbances in HD
Movement Disorders
P5 - Poster Session 5 (8:00 AM-9:00 AM)
3-008
To examine the usefulness of a new behavioral questionnaire for patients with, and at risk for, Huntington's disease (HD).

Behavioral changes are characteristic features of HD and often the most distressing aspect of the disease for families and caregivers.  
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.
The HD-BQ discriminated between HD (mean 31.4), PM (mean 22.7) and NC (mean 15.3) subjects (p < .001); importantly, it identified significant differences between PM and NC (p = .048) subjects that traditional cognitive, functional, and motoric measures did not. Effect sizes were 60% greater for the HD-BQ (d = 0.93) as compared to the HADS (d = 0.58) and PBA-s (d = 0.58) for NC vs HD. Caregiver assessments suggested that HD patients underestimate their impairment (mean difference=13.3 points; p < .001). ROC analysis showed highest area under the curve for HD-BQ (0.789) followed by PBA-s (0.703) and HADS (0.687). Principal component analysis identified 3 components (cognitive, psychiatric, functional) underlying the HD-BQ, explaining 52.8% of the variance.  

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.
Authors/Disclosures
Jody Corey-Bloom, MD, PhD, FÂ鶹´«Ã½Ó³»­ (UCSD Neurosciences)
PRESENTER 		Dr. Corey-Bloom has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UniQure. Dr. Corey-Bloom has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Teva Pharmaceuticaks. Dr. Corey-Bloom has received personal compensation in the range of $10,000-$49,999 for serving as a Co-Director, HD-Net with Huntington Study Group.
No disclosure on file
No disclosure on file
No disclosure on file
No disclosure on file
No disclosure on file
Brenton A. Wright, MD Dr. Wright has nothing to disclose.
Paul Gilbert, PhD (SDSU-UCSD) Dr. Gilbert has nothing to disclose.