Beyond the motor account of amyotrophic lateral sclerosis: Verbal humour and its relationship with the cognitive and pragmatic profile

Abstract

Background Amyotrophic Lateral Sclerosis (ALS) was traditionally described as a disease restricted to the motor system. However, recent findings suggested that it also affects cognition, especially executive functions, social cognition, language and pragmatics. A relevant issue in current research is thus the description of the cognitive phenotype of ALS and the identification of the most vulnerable aspects. Aims The focus was on a communicative phenomenon placed at the crossroads of pragmatic and other cognitive domains, namely humour, which till now has been poorly explored in ALS. The first aim was to investigate whether ALS is associated with impairments in understanding and appreciating jokes. The second aim was to explore the predictors of humour comprehension and appreciation in patients, to confirm the involvement of pragmatic skills and to explore the role of other cognitive and clinical aspects. Methods & Procedures A total of 30 non‐demented patients with ALS and 27 controls were assessed with a task of verbal humour comprehension and appreciation, including two types of jokes: phonological and mental. We also administered a battery of pragmatic and other language tasks, and cognitive and socio‐cognitive tasks. Mixed‐effects models were used to test differences in the humour task between the two groups. Multiple regressions determined the best predictors of humour comprehension and appreciation in patients. Outcomes & Results Patients obtained lower comprehension accuracy scores than controls in the humour task, independently of the type of joke. Conversely, patients and controls did not differ in joke appreciation and both rated mental jokes as funnier than the phonological ones. Patients’ comprehension accuracy was predicted by pragmatic skills and ALS severity, whereas appreciation was predicted by several clinical variables and, to a smaller extent, by language skills. Conclusions & Implications The findings suggest that humour is a very vulnerable aspect in ALS, and that impairment in humour comprehension might be part of the larger cognitive impairment, being linked to pragmatic impairment. Clinical variables were also important, especially in relation to humour appreciation. More generally, these data speak in favour of pragmatics as a relevant aspect to sketch the cognitive phenotype of ALS. On the practical level, these findings point to the need of supporting communication at large, not only motor‐related aspects such as dysarthria but also social–pragmatic aspects such as understanding jokes, to increase well‐being in ALS.

Cognitive dysfunction in amyotrophic lateral sclerosis: can we predict it?

“…As highlighted in a recent review [ 26 ], SC deficits in ALS range from ToM abnormalities, to lack of empathy and changes in social behavior. With respect to diagnostic tools, Reading the Mind in the Eyes [ 27 ] and the Faux-pas Test are indeed the most well-known tests assessing ToM [ 7 ], and the Animated Shapes task [ 28 ], which provides moving stimuli, has also been used in a recent study [ 29 ]. Additionally, performance on understanding thoughts and feelings of cartoon characters (Happé cartoons) is also compromised in ALS.…”

“…On the other hand, self-reported and partner-reported interviews can provide information on empathy and social behavior changes. Intriguingly, a recent study by Bambini et al [ 29 ] addressed another underestimated aspect of ALS, which straddles between SC and pragmatic fields: the impairment of verbal humor comprehension at the Phonological and Mental Jokes [ 31 ]. Furthermore, a prominent role for pragmatic impairment was observed, while scores at tests assessing executive and ToM abilities did not show a significant predictive role, in opposition to previous studies.…”

When No Laughing Matter Is No Laughing Matter: The Challenges in Developing a Cognitive Theory of Humor

“…And so various experiential states will be classified as "anger" not because they share any sort of deep phenomenological similarity or mechanistic underpinning, but because the way in which we've been taught to apply the "anger" concept directly influences how we make sense of our own experiences. The illusion that we can all  E. g., Adamczyk et al 2017;Bambini et al 2020;Bischetti et al 2019;Mobbs et al 2003;Rozengurt 2011;Samson 2013;Samson and Hegenloh 2010;Chen et al 2017. easily recognize anger when we experience it comes from the fact that our experiences are structured by, and filtered through, our emotion concepts. Thus, we force our experiences to fit into the categories we provide, instead of recognizing some fundamental similarity between different experiential states.…”

“…Or consider a number of studies that claim to show a deficit in humor for those with conditions like autism spectrum disorder (Samson and Hegenloh 2010;Samson 2013), schizophrenia (Vrticka, Black and Reiss 2013;Adamczyk et al 2017), amyotrophic lateral sclerosis (Bambini et al 2020), alcohol use disorder (Uekermann et al 2007; Le Berre, Fama, and Sullivan 2017), and major depression (Uekermann et al 2008). To understand these cases, one must understand the role that Theory of Mind is thought to play in many kinds of jokes.…”

Case report: Pragmatic impairment in Multiple Sclerosis after worsening of clinical symptoms

“…Several theoretical frameworks have been proposed to account for pragmatic mechanisms in the field of linguistics (e.g., Goodman & Frank, 2016;Sperber & Wilson, 2002;Grice 1975). Moreover, in the last decades, pragmatics has become a topic also for neuropsychological assessment, and pragmatic impairments have been found in several neurological diseases (Cummings, 2021;Bambini et al, 2016a;Bambini et al, 2020b;Bambini & Ceroni, 2021;Gabbatore et al, 2015;Montemurro et al, 2019).…”