Which behaviours? Identifying the most common and burdensome behaviour changes in amyotrophic lateral sclerosis

Abstract

Behaviour change is increasingly recognised as a common feature of amyotrophic lateral sclerosis (ALS), and may be similar to that seen in frontotemporal dementia (FTD). The behaviours most disturbed in ALS, and those that relate most significantly to caregiver burden, however, have not been well established. Forty ALS participants and their caregivers, and 27 age- and gender-matched healthy controls and their relatives, participated in this study. ALS participants were assessed on a disease rating scale, and caregivers and control informants completed the revised version of the Cambridge Behaviour Inventory and a measure of burden. ALS caregivers reported significantly more disturbance than healthy control informants on the functional domains of everyday skills, self-care, and sleep, and in the behavioural domains of mood and motivation. There were no differences between groups in frequency of memory and orientation difficulties, or behaviours characteristic of FTD, such as changes to eating habits or stereotypic and motor behaviour, indicating that the behavioural profile in ALS may differ from FTD. In the ALS group, the domains with the strongest relationship to caregiver burden were everyday skills, motivation and memory, likely because poor motivation, memory dysfunction and difficulties completing activities of daily living require more carer support via direct supervision, prompting or hands on care. Services to support ALS patients and caregivers need to provide targeted interventions for those functional and behavioural changes which are most burdensome in the disease.

Alterations in the stomatognathic system due to amyotrophic lateral sclerosis

“…Higher activity was also observed for masseter and temporal muscles in subject with Duchenne muscular dystrophy 13 . Individuals with ALS present with continual stress due to emotional problems related to the neurodegenerative disease 4 . Our research did not evaluate the levels of stress in individuals with ALS.…”

Burden of caregivers of patients with frontotemporal lobar degeneration – a scoping review

“…Zarit Burden Interview 10Andrews et al (2017),,,Brodaty et al (2014),Davis and Tremont (2007),Kaiser and Panegyres (2006),Knutson et al (2008),Liu et al (2017),Nicolaou et al (2010), Uflacker et al (2015) Short Zarit Burden Interview 11 Hsieh et al (2013), Hsieh et al (2016), Kaizik et al (2017), Kumfor et al (2014), Kumfor et al (2016), Lillo et al (2012), Lima-Silva et al (2015), Mekala et al (2013), Miller et al (2013), Mioshi et al (2013a), Uflacker et al (2016) Neuropsychiatric Inventory 9 Lima-Silva et al (2015), Mourik et al (2004), Ng et al (2012), Riedijk et al (2006), Riedijk et al (2008), Riedijk et al (2009), Uflacker et al (2015), Vugt et al (2009), Wong and Wallhagen (2012) Caregiver Burden Scale 2 Bock et al (2016), Bristow et al (2008) Caregiver Burden Index 3 Chio et al (2010), Cosseddu et al (2013), Cui et al (2015) Caregiver Strain Index 3 Diehl-Schmid et al (2013), Merrilees et al (2010), Roche et al (2015) Screen for Caregiver Burden 1 Ng et al (2012) Visual Analogue Scale 4 Riedijk et al (2006, 2008, 2009), de Vugt et al (2006) FTLD diagnoses in quantitative studies Not specified 18 Ascher et al (2010), Brodaty et al (2014), Davis and Tremont (2007), Kaizik et al (2017), Mekala et al (2013), Merrilees et al (2010), Mioshi et al (2009), Mourik et al (2004), Nicolaou et al (2010), Ng et al (2012), Riedijk et al (2006), Riedijk et al (2008), Riedijk et al (2009), Rosness et al (2008), Uflacker et al (2015), de Vugt et al (2006), Wong and Wallhagen (2012), Wong and Wallhagen (2014) bvFTD/fvFTD 16 Armstrong et al (2013), Boutoleau-Bretonniere et al (2008), Brioschi Guevara et al (2015), Bristow et al (2008), Chow et al (2011), Cosseddu et al (2013), Diehl-Schmid et al (2013), Hsieh et al (2013), Hsieh et al (2016), Knutson et al (2008), Kumfor et al (2016), Lima-Silva et al (2015), Liu et al (2017), Miller et al (2013), Mioshi et al (2013a), Uflacker et al (2016) Aphasia not specified 4 Armstrong et al (2013), Cosseddu et al (2013), Diehl-Schmid et al (2013), Kaiser and Panegyres (2006) SD 10 Armstrong et al (2013), Bristow et al (2008), Chow et al (2011), Hsieh et al (2013), Hsieh et al (2016), Kumfor et al (2016), Liu et al (2017), Miller et al (2013), Mioshi et al (2013a), Riedl et al (2014) PNFA 6 Chow et al (2011), Kumfor et al (2014), Liu et al (2017), Miller et al (2013), Mioshi et al (2013a), Riedl et al (2014) Overlap syndromes in quantitative studies CBS 4 Armstrong et al (2013), Chow et al (2011), Knutson et al (2008), Kaizik et al (2017) PSP 3 Chow et al (2011), Davis and Tremont (2007), Kaizik et al (2017) MND 6 Andrews et al (2017), Bock et al (2016), Chio et al (2010), Cui et al (2015), Hsieh et al (2016), Lillo et al (2012)…”

Rehabilitation bei Amyotropher Lateralsklerose

“…Das zusätzliche Auftreten einer frontotemporalen Demenz der Pa tienten stellt eine besondere Herausfordferung für die Caregiver dar [92]. Als besonders belastend werden die Symptome empfun den, die den Caregivern im Alltag besonders viel Supervision, An leitung und direkte Hilfestellung abverlangten wie Gedächtnisstö rungen, Antriebsstörungen und Schwierigkeiten bei Aktivitäten des täglichen Lebens [93]. Caregiver stellen ihre Bedürfnisse oft hinter denen des Patienten zurück und benötigen daher einen proaktiven Unterstützungsansatz, bei dem auch EHealth eine Rolle spielen könnte [94].…”