Relationships Among Apathy, Health-Related Quality of Life, and Function in Huntington’s Disease

Abstract

Up to 90% of individuals with Huntington's disease (HD)-a progressive, inherited neurodegenerative disorder-experience apathy. Apathy is particularly debilitating because it is marked by a reduction in goal-directed behaviors, including self-care, social interactions, and mobility. The objective of this study was to examine relationships between variables of apathy, functional status, physical function, cognitive function, behavioral status/emotional function, and health-related quality of life. Clinician-rated measures of physical, cognitive, and behavioral function, including one clinician-rated item on apathy, and self-reported measures of physical function, health-related quality of life, and emotional, cognitive, and social function were collected in a single session from 487 persons with the HD mutation (prodromal, N=193; early-stage manifest, N=186; late-stage manifest, N=108). Multiple linear regression models were used to examine which outcomes best predicted clinician-rated apathy after controlling for disease stage. Greater apathy related to less independence, increased motor impairment, and more clinician-rated behavioral problems (i.e., anger, irritability, depression). Similarly, poorer self-reported health-related quality of life; greater chorea; greater upper- and lower-extremity dysfunction; greater speech and swallowing dysfunction; worse anxiety, depression, and behavioral dyscontrol; worse cognitive function; and less satisfaction with social roles related to greater apathy. In conclusion, apathy related to physical, cognitive, and behavioral dysfunction across disease stages. Future work should explore whether clinical interventions targeting different functional domains may have the potential to reduce apathy in this patient population.

Validity of irritability in Huntington's disease: A scoping review

“…Nine of these studies reported significantly positive correlations with other psychological difficulties including apathy (Bouwens et al, 2015;Pflanz et al, 1991), anxiety (Litvan et al, 1998Nimmagadda et al, 2011;Paulsen et al, 2001), depression (Litvan et al, 1998Nimmagadda et al, 2011;van Duijn et al, 2014), bad temper (Burns et al, 1990) and suicidal ideation and/or behaviour (Anderson et al, 2016;van Duijn et al, 2018). Fritz et al (2018) additionally identified that poorer behavioural scores (a composite score from the PBA-HD short form, covering ten behavioural problems including irritability, although not a specific measure of irritability in itself) were associated with higher apathy scores (large effect size: R 2 = 0.30), in line with research specifically examining irritability as a stand-alone variable.…”

“…IRRITABILITY IN HUNTINGTON'S DISEASE 9 (PBA;Craufurd et al, 2001;Gregory et al, 2015;Kingma et al, 2008;Reedeker et al, 2012;Thompson et al, 2002;Thompson et al, 2012;van Duijn et al, 2013;van den Stock et al, 2015), one its Dutch equivalent (NL-PBA; Bouwens et al, 2016), and four the PBA shortform (PBA-s;Fritz et al, 2018;Honrath et al, 2018;Martinez-Horta et al, 2016;Ruiz-Idiago et al, 2017). Three studies used the Irritability Scale (IS; Bouwens et al, 2015;Diago et al, 2018;Reedeker et al, 2012), four used the Snaith Irritability Scale (SIS; Berrios et al, 2001;Klöppel et al, 2010;Maltby et al, 2016) and two the Hospital Anxiety and Depression Scale combined with the SIS (HADS-SIS; Underwood et al, 2016;Vassos et al, 2007).…”

“…2015; Bouwens et al, 2016;Burns et al, 1990;Diago et al, 2018;Fritz et al, 2018;Honrath et al, 2018;Hubers et al, 2013;Litvan et al, 1998;Nimmagadda et al, 2011;Paulsen et al, 2001;Pflanz et al, 1991;Thompson et al, 2002;Underwood et al, 2016;van Duijn et al, 2014;van Duijn et al, 2018), nine reported correlations between irritability and other psychological difficulties, and one reported association via multiple regression. Nine of these studies reported significantly positive correlations with other psychological difficulties including apathy (Bouwens et al, 2015;Pflanz et al, 1991), anxiety (Litvan et al, 1998Nimmagadda et al, 2011;Paulsen et al, 2001), depression (Litvan et al, 1998Nimmagadda et al, 2011;van Duijn et al, 2014), bad temper (Burns et al, 1990) and suicidal ideation and/or behaviour (Anderson et al, 2016;van Duijn et al, 2018).…”

“…No significant correlation between sleep quality (rho = .368; p = .023), sleep latency (rho = .224; p = .177), sleep disturbance (rho = .321, p = .0050), sleep dysfunction (rho = .193, p = .247) or sleepiness (rho = .277, p = .092). Fritz et al (2018) Examine relationships between apathy and behaviour, cognition, physical function and health-related quality of life in people with HD.…”

Apathy Associated With Impaired Recognition of Happy Facial Expressions in Huntington’s Disease

“…Apathy is a prevalent neuropsychiatric symptom in patients with HD (Camacho, Barker, & Mason, 2018) and has important implications for the patients’ functional capacity of the patient (Hamilton et al, 2003; van Duijn et al, 2014), quality of life (Eddy & Rickards, 2013), employment (Jacobs, Hart & Roos, 2018), and social functioning (Fritz et al, 2018). Indeed, both patients and caregivers rate apathy in the top three most impactful features of the disease (Simpson, Lovecky, Kogan, Vetter, & Yohrling, 2016).…”