Impact of cognitive and behavioural changes on quality of life in Huntington's disease

Huntington's disease (HD) is associated with both cognitive and behavioural changes in addition to movement disorder. We aimed to investigate relationships between self-reported Quality of Life (QoL), motor symptoms, cognitive deficits and behavioural problems in HD. Twenty outpatients with HD (mean age 53.85 years, SD = 7.05; mean education 13.30 years, SD = 2.49; mean years since motor onset 6.87 years, SD = 4.93) were recruited from the Queen Elizabeth Psychiatric Hospital, Birmingham, UK. QoL was assessed using the SF-36 questionnaire. Executive measures were used to assess attention, verbal fluency, working memory and inhibition. Behavioural measures included the Problem Behaviours Assessment-Short Form (PBA-S) to assess depressive symptoms, and the frontal systems behaviour scale (FrsBe) to assess apathy, disinhibition and executive dysfunction. Unified Huntington's Disease Rating Scale motor scores (UHDRS-MS) were also recorded. QoL scores were not related to UHDRS-MS, PBA-S depression scores, or performance on specific executive tasks. However, QoL was lower for patients who exhibited greater apathy and showed more evidence of executive dysfunction in their everyday behaviour as assessed by the FrsBe. Our findings imply that behavioural evidence of apathy and executive problems are closely related to self-reported QoL in HD, and that these factors can impact patients’ wellbeing more than movement disorder.