Apathy is one of the most common behavioural symptoms of amyotrophic lateral sclerosis (ALS), yet there are few studies that have investigated the relationship between apathy and quality of life (QOL) as they are experienced by the patient. A cohort of 60 ALS patients were evaluated using the Apathy Evaluation Scale which measured cognitive, behavioural, emotional and non-specific symptoms of apathy combined with the Personal Wellbeing Index, a multidimensional measure of QOL. The relationship between patient-rated apathy and QOL scores, controlling for potential clinical and psychological confounders were analysed using univariate and multivariate methods. Apathy was identified in 30% of ALS patients. Patients with apathy reported higher levels of depression (p = 0.0001). Compared to non-apathetic patients, patients with apathy had lower overall QOL (p = 0.001), most pronounced in the domains related to achievements in life (p = 0.001) and community-connectedness (p = 0.0001). Of the cognitive, behavioural, emotional and non-specific manifestations of apathy, only the emotional symptoms explained a significant amount of variance in achievements in life (p = 0.003) and community-connectedness (p = 0.001). As such, emotional manifestations of apathy may underlie worse QOL in ALS patients presenting with behavioural impairment. Patient-reported outcomes, particularly those assessing psychosocial functioning may be important for demonstrating the efficacy of interventions designed to improve QOL in ALS patients with behavioural impairment.
Amyotrophic lateral sclerosis Motor neuron disease Quality of life Patient-reported outcome measures Apathy Depression
This is a preview of subscription content, log in to check access.
Compliance with ethical standards
Conflicts of interest
On behalf of all authors, the corresponding author states that there is no conflict of interest.
This work was supported by a National Health and Medical Research Council of Australia Postgraduate Research Scholarship (APP1092891) (J. Caga) and a National Health and Medical Research Council of Australia Program Grant (1037746) (M.C. Kiernan).
All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.
Caga J et al (2016) Apathy is associated with poor prognosis in amyotrophic lateral sclerosis. Eur J Neurol 23:891–897CrossRefPubMedGoogle Scholar
Chio A et al (2010) Neurobehavioral symptoms in ALS are negatively related to caregivers’ burden and quality of life. Eur J Neurol 17:1298–1303CrossRefPubMedGoogle Scholar
Andrews SC et al (2017) Which behaviours? Identifying the most common and burdensome behaviour changes in amyotrophic lateral sclerosis Psychol Health Med 22:483–492PubMedGoogle Scholar
Cui B et al (2015) Behavioral symptoms in motor neuron disease and their negative impact on caregiver burden. Chin Med J (Engl) 128:2295–2300CrossRefGoogle Scholar
Burke T et al (2015) Caregiver burden in amyotrophic lateral sclerosis: a cross-sectional investigation of predictors. J Neurol 262:1526–1532CrossRefPubMedGoogle Scholar
Watermeyer TJ et al (2015) Impact of disease, cognitive and behavioural factors on caregiver outcome in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener 16:316–323CrossRefPubMedGoogle Scholar
Bock M et al (2016) Cognitive-behavioral changes in amyotrophic lateral sclerosis: screening prevalence and impact on patients and caregivers. Amyotroph Lateral Scler Frontotemporal Degener 17:366–373CrossRefPubMedGoogle Scholar
Swash M (1997) Health outcome and quality-of-life measurements in amyotrophic lateral sclerosis. J Neurol 244(Suppl 2):S26–S29CrossRefPubMedGoogle Scholar
Miller RG et al (2009) Practice parameter update: the care of the patient with amyotrophic lateral sclerosis: multidisciplinary care, symptom management, and cognitive/behavioral impairment (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 73:1227–1233CrossRefPubMedPubMedCentralGoogle Scholar
Brooks BR et al (2000) El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord 1:293–299CrossRefPubMedGoogle Scholar
Strong MJ et al (2009) Consensus criteria for the diagnosis of frontotemporal cognitive and behavioural syndromes in amyotrophic lateral sclerosis. Amyotroph Lateral Scler 10:131–146CrossRefPubMedGoogle Scholar
Cedarbaum JM et al (1999) The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS Study Group (Phase III). J Neurol Sci 169:13–21CrossRefPubMedGoogle Scholar