Alessandra Girardi

Deficits in Emotional and Social Cognition in Amyotrophic Lateral Sclerosis

OBJECTIVE These studies investigated whether non-demented ALS patients display impairments on tests of emotional decision making and social and emotional cognition, sensitive to frontal variant Frontotemporal Dementia (fvFTD). Previous studies have shown predominant executive dysfunction and dorsolateral prefrontal involvement in ALS, but evidence of other prefrontal dysfunction implicated in fvFTD is sparse. METHOD In Study A, 19 ALS patients and 20 healthy controls undertook a test of affective decision making, modified Iowa Gambling Task (IGT). Behavioral measures included the Frontal Systems Behavior Scale. In Study B, 14 ALS patients and 20 controls undertook tests of social and emotional cognition (Judgment of Preference based on eye gaze, the Mind in the Eyes, recognition of Facial Expressions of Emotion). RESULTS In Study A, ALS patients demonstrated a significantly different performance profile from healthy controls on the IGT and did not learn to avoid the disadvantageous stimuli (Block 3, d = 0.60, Block 4 days = 0.68). Behavior ratings showed increased apathy from premorbid levels. In Study B, ALS patients were impaired on attentionally demanding (d = 3.12) and undemanding (d = 7.52) conditions of the Judgment of Preference task, despite many showing intact executive functions. A smaller subset showed impaired emotion recognition. Behavior change was also evident. CONCLUSIONS The findings reveal a Theory of Mind deficit on a simple test that was dissociated from the presence of executive dysfunction and suggests a profile of cognitive and behavioral dysfunction indicative of a subclinical fvFTD syndrome. The relative contribution of prefrontal pathways to the cognitive profile in ALS is considered.

Theory of mind and the Ultimatum Game in healthy adult aging

ABSTRACT Background: The Ultimatum Game assesses decision-making involved in cooperative interactions with others. However, little is known about the role that the ability to understand other people’s intentions plays in these interactions. Methods: This study examined performance on the Ultimatum Game and theory of mind (ToM) tasks in younger and older adults. Results: Age differences were not found on the ToM tasks, and a lack of variability in performance prevented analyses of the relationships between performance on the Ultimatum Game and ToM. However, age differences were found on the Ultimatum Game, with older adults accepting more unfair offers. Yet, the two age groups did not differ in their appreciation of fairness, as assessed using subjective fairness ratings. Conclusions: These findings suggest that older adults are more rational in their behavior, accepting unfair offers even when they know they are unfair, as it is in their self-interest to accept small monetary values rather than nothing at all.

Social and Emotional Cognition and Behaviour: Evidence of Subclinical frontotemporal Dementia in ALS

Background: The prevalence of frontotemporal dementia (FTD) in the ALS population is estimated to be between 30 to 50 percent. FTD is recognized to present as three distinct subtypes, including isolated behavioural change, a frontal cortical associated aphasia of a progressive non-fluent type and a temporal cortical associated aphasia of a fluent type characterized by loss of semantic knowledge. Given the aggressive course of the disease process, we wanted to identify ALS patients whose emerging FTD was rendering them less capable of independent decision-making. We developed an evaluation approach that included a behavioural inventory as well as screening measures of language change characteristic of either frontal dysfunction (FD) or temporal dysfunction (TD).