Objective: To examine the utility of a new behavioral measure, the Huntington’s Disease–Behavioral Questionnaire (HD-BQ) (Hughes et al, 2023), in individuals with Huntington’s disease (HD).

Background: Individuals with HD often experience behavioral disturbances that cause distress to caregivers. Clinical trials and clinical practice often rely on self-report from individuals with HD; however, it is common for HD individuals to experience anosognosia – a lack of awareness of deficits. Utilizing collateral sources such as caregivers may achieve more reliable information about the patient’s challenges.

Method: The HD-BQ was administered to 81 individuals with manifest HD and their caregivers. The HD-BQ is a 30 item self-report scale that surveys patients and caregivers on the affected individual’s behavior over the past year using a 4-point Likert scale from 0 (completely disagree) to 3 (completely agree). Independent student T tests compared total and item scores between HD individuals and caregivers. Frequency distributions of ratings 0-1 (disagreement) and 2-3 (agreement) for both self-report and caregiver ratings were calculated. Lastly, linear regression analyses were performed to ascertain whether Total Functional Capacity (TFC), Total Motor Score (TMS), or the Symbol Digit Modality Test (SDMT) were more predictive of caregiver or patient HD-BQ total score.

Results: Overall, caregivers rated individuals with HD ~11 points higher than HD individuals rated themselves on the HD-BQ [t(160)=-3.345, p<.001]. Caregivers consistently rated individuals with HD higher than patients on each one of the individual items of the HD-BQ. Questions reflective of executive dysfunction and psychiatric disturbance demonstrated the most significant difference between caregiver and self-report ratings (p<.01). Importantly, TFC [F(1,80)=24.612, p<.001, R²=.486], TMS [F(1,80)=8.430, p=.005, R²=.309], and SDMT [F(1,79)=5.908, p=.017, R²=.265] were significant predictors of caregiver, but not patient, total HD-BQ scores.

Conclusion: We conclude that the HD-BQ shows utility for identifying problematic behaviors in HD, particularly when co-administered to collateral sources of information. Patient functional capacity, motor severity, and cognition were highly predictive of caregiver, but not patient, BQ scores indicating that caregivers may be more reliable informants than patients regarding their behavioral difficulties.

References: Hughes, S., Aboufadel, S., Smirnova, A., Snell, C., Churchill, E., Hall, A., Malcarne, V., Gilbert, P.E. and Corey-Bloom, J. (2023), Development and Psychometric Properties of a New Brief, Yet Comprehensive, Behavioral Questionnaire for Huntington’s Disease. Mov Disord Clin Pract. https://doi.org/10.1002/mdc3.13661

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MDS Abstracts - https://www.mdsabstracts.org/abstract/self-report-vs-informant-report-of-problematic-behaviors-using-the-huntingtons-disease-behavioral-questionnaire/