Objective: To examine the usefulness of a new behavioral questionnaire for patients with, and at risk for, Huntington’s disease (HD).

Background: Behavioral changes, including apathy, depression, irritability, anxiety, disinhibition, and difficulty prioritizing and initiating activities, are characteristic features of HD. For families and caregivers, these changes are often the most distressing aspect of the disease. The assessment of these behavioral disturbances is therefore important. The HD-BQ consists of 30 items with a 3-point rating scale and takes about 5 minutes to complete. The maximum score is 90, with higher scores indicating more behavioral changes.

Method: 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, 68 pre-manifest HD (PM), and 106 control (NC) subjects. The HD-BQ was also administered to 55 HD caregivers. Analyses were performed controlling for age. The False Discovery Rate (FDR) was used to adjust for multiple comparisons.

Results: The HD-BQ discriminated between HD (mean 31.4, range 0-79), PM (mean 22.7, range 0-73) and NC (mean 15.3, range 0-65) subjects (p < .001). Importantly, the HD-BQ identified significant differences between PM and NCs (p = .048) that traditional cognitive (SDM p=0.33; MoCA p=0.43; MMSE p=0.27), functional (TFC p=0.83), and motoric (TMS p=0.09) measures did not. Effect sizes, measured by Cohen’s D, were also 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 subjects. Questionnaires completed independently by 55 HD subject/caregiver pairs indicated that there was a discrepancy in the perception of impairment between patients and their caregivers, with caregivers identifying significantly more behavioral changes (mean difference=14.9 points; p < .001).

Conclusion: The HD-BQ is a quick, reliable instrument for screening behavioral changes in individuals with, and transitioning to, HD. 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.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/the-huntingtons-disease-behavioral-questionnaire-hd-bq-a-new-screening-tool-for-behavioral-disturbances-in-hd/