Abstract
Amyotrophic lateral sclerosis (ALS) is a rapidly progressive condition involving degeneration of both upper and lower motor neurons. Recent research suggests that a proportion of persons with ALS show a profile similar to that of frontotemporal dementia (FTD), with this group of ALS patients exhibiting social cognitive deficits. Although social cognitive deficits have been partially explored in ALS, research has yet to investigate such changes using ecologically valid measures. Therefore, this study aimed to further characterize the scope of social cognitive and emotion recognition deficits in non-demented ALS patients using an ecologically valid measure of social cognition. A sample of 35 ALS patients and 30 age-and-education matched controls were assessed using the Addenbrooke's Cognitive Examination, the Brixton Spatial Anticipation Test, and The Awareness of Social Inference Test, where participants were required to discriminate between various emotions and decipher socially challenging scenarios enacted in video vignettes. Participants with ALS showed significant difficulties in recognizing both sarcastic and paradoxical sarcastic statements, but not sincere statements, when compared to controls. After controlling for executive difficulties, ALS patients still displayed significant difficulties on tasks that assessed their comprehension of both sarcastic and paradoxical sarcastic statements. The inability to read social cues and make social inferences has the potential to place significant strain on familial/interpersonal relationships in ALS. The findings of this study highlight the importance of employing a broader range of neuropsychological assessment tools to aid in early detection of frontal lobe impairment in non-demented ALS patients.
Highlights
Amyotrophic lateral sclerosis (ALS), the most common variant of motor neuron disease, is a rapidly progressive condition involving degeneration of both upper and lower motor neurons (Mitsumotot et al, 2005)
Research consistently reports functional and structural changes in the frontotemporal region, manifesting as executive dysfunction, including behavioral dysinhibition; a pattern of deficits similar to that observed in frontotemporal dementia (FTD; Lomen-Hoerth et al, 2000; Ringholz et al, 2005; Murphy et al, 2007; Meier et al, 2010; Girardi et al, 2011)
Given that Theory of Mind (ToM) tasks place strong demands on frontal networks, the authors hypothesized that executive dysfunction may contribute or be solely responsible for the poor social inferential and reasoning skills displayed within this ALS sample
Summary
Amyotrophic lateral sclerosis (ALS), the most common variant of motor neuron disease, is a rapidly progressive condition involving degeneration of both upper and lower motor neurons (Mitsumotot et al, 2005). In contrast a relatively new measure of executive functioning, the Brixton Spatial Anticipation Test (Burgess and Shallice, 1997), measures a person’s ability to detect a rule, to follow it, and to switch to a new rule This executive task does not require a verbal or complex motor response by the patient, and circumvents administrative issues faced by researchers attempting to measure executive decline in ALS. Meier et al (2010) recently investigated social cognitive deficits in a small non-demented sample of ALS patients using the Faux Pas test. This ToM task required ALS patients to determine whether a character in a scenario had said something socially inappropriate. Error analysis revealed that a subset of patients displayed a lack of inhibition and an egocentric tendency to select their own favored object while disregarding the one preferred by the face in the task
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