Affiliations: [a] Department of Neurology, Vanderbilt University Medical Center, Nashville, TN, USA
| [b] Department of Psychology and Human Development, Vanderbilt University, Nashville, TN, USA
| [c] Department of Pediatrics, Division of Child Neurology, Vanderbilt Children’s Hospital, United States
Correspondence:
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Correspondence to: Katherine McDonell, MD, MSCI, 1500 21st Ave South, Suite 1532, Nashville, TN 37212, USA. Tel.: +1 615 875 7160; Fax: +1 615 343 3946; E-mail: katherine.mcdonell@vumc.org.
Abstract: Background:Risky behaviors are common in Huntington’s disease (HD) and can lead to significant adverse consequences. However, the prevalence and scope of these symptoms have not been studied systematically, and no empirically validated measures are available to screen for them. Objective:To test a novel screening tool designed to assess risk-taking behaviors in HD. Methods:We administered the Risk Behavior Questionnaire (RBQ-HD) to HD patients and caregivers at Vanderbilt University Medical Center between 2018-2019. Patients completed the questionnaire based on self-report; caregivers provided collateral reports. Clinical and demographic information were obtained from the electronic medical record. Results:60 patients and 60 caregivers completed the RBQ-HD. 80% of patients (n = 48) and 91.7% of caregivers (n = 60) reported at least one risky behavior. Adverse social behaviors, impulsive/compulsive behaviors, and reckless driving were the most common behavioral domains reported. Male patients were more likely to report risky behaviors than females (92.3% vs. 70.6%, p = 0.04). The number of risky behaviors reported by patients and caregivers was negatively correlated with patient age (r = –0.32, p = 0.01; r = –0.47, p = 0.0001, respectively). Patient and caregiver reports were highly correlated in matched pairs (n = 30; r = 0.63, p = 0.0002). Conclusion:These findings emphasize that risky behaviors are highly prevalent in HD and can be effectively identified through the use of a novel screening measure. We hypothesize that early pathological involvement of frontostriatal and mesolimbic networks may be important factors in the development of these behaviors.
Keywords: Huntington’s disease, risk-taking, impulsive behavior, surveys and questionnaires
