Measurement of Social Cognition in Amyotrophic Lateral Sclerosis: A Population Based Study

Abstract

Background: Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disease. Executive dysfunction is common in patients with ALS, with up to 50% of patients performing within an impaired range. There is evidence that social cognitive deficits associated with ALS are a function of deficits in executive function. The ‘Reading the Mind in the Eyes’ Test is a recognized test of social cognitive function, although the reliability of this instrument remains to be established. Methodology: Patients with ALS (n = 106), and age and IQ matched controls (n = 50) were recruited and asked to perform the Reading the Mind in the Eyes Test as part of an on-going population-based study of cognitive function. ALS patients were sub-stratified based on the presence, and/or extent of executive dysfunction. Results: Cronbach’s Alpha of .73 was observed, indicating good reliability on this measure. Split-half reliability analysis further confirms these findings (p = 0.826). The Reading the Mind in the Eyes test had excellent psychometric properties when discriminating between ALS patients who are cognitively intact, and those who have executive impairment, with an overall medium difficulty. There was a large magnitude significant difference between patients and controls (p< 0.001; η2 = .19). Post-hoc analysis revealed that controls performed significantly higher than patients with executive impairment (p< 0.001), and patients with single executive deficits (p = 0.002). Conclusion: Executive dysfunction impacts on social cognitive performance. This study contributes not only to the psychometric knowledge of this measure, but also to the usability, efficacy, and reliability of social cognitive assessment in ALS. Using population-specific normative data, we confirm the Reading the Mind in the Eyes Test is a reliable measure of social cognitive processes in ALS.

Theory of Mind and Its Neuropsychological and Quality of Life Correlates in the Early Stages of Amyotrophic Lateral Sclerosis

“…Some evidence supports a stronger association between executive functions and “fluid intelligence,” both involving mainly dorsolateral prefrontal networks (Roca et al, 2012), in contrast to ToM processes, hypothesized to be probably more dependent from medial prefrontal networks, especially with regard to the affective subcomponent (Shamay-Tsoory et al, 2005, 2006; Xi et al, 2011; Bertoux et al, 2016). However, more recently, Burke et al (2016b) demonstrated a significant impairment of ET, as a measure of affective social cognition, in ALS patients with executive dysfunctions compared to healthy subjects. Probably, these inconsistent results may be due by the fact that the anatomical and biological bases of the ToM performances are still debated.…”

“…Impairment of social cognition has also been described in several cohorts of patients in heterogeneous stages of ALS (Girardi et al, 2011; Van der Hulst et al, 2015; Burke et al, 2016a,b). Moreover, abnormalities of both cognitive and affective ToM, related to social behavior dysfunctions, such as loss of empathy and apathy (Gregory et al, 2002; Girardi et al, 2011), have been recently confirmed as a prominent feature of behavioral profile in advanced ALS (Van der Hulst et al, 2015).…”

“…Approximately 5–15% of ALS patients meet the diagnostic criteria for FTLD (Neary et al, 1998; Rascovsky et al, 2011), predominantly developing clinical features typical of the behavioral variant frontotemporal dementia (bvFTD; Lomen-Hoerth, 2004) and a larger proportion of non-demented ALS patients shows cognitive (mainly involving the executive domain) and/or behavioral deficits, belonging to a spectrum of symptoms frequently recognized within FTLD syndromes (Lomen-Hoerth, 2004; Abrahams et al, 2005; Burrell et al, 2016). With regard to social cognitive deficit in ALS, as well as in overall the disease spectrum of FTLD (Elamin et al, 2012), Burke et al (2016b) demonstrated that ET is a valid measure of the cognitive decline in this domain in ALS patients, also in terms of severity, showing, in a previous study (Burke et al, 2016a), that patients with ALS with bulbar onset (ALS-B) may perform worse in this task, thus supporting the view that ALS-B may represent a biologically more aggressive phenotype of the disease (Cardenas-Blanco et al, 2014). Moreover, Van der Hulst et al (2015) revealed that in advanced ALS impairment of both subcomponents of ToM may be associated with apathy and impairment of frontotemporal-related abilities, such as verbal fluency and naming abilities, suggesting that evidence of both deficits may reflect a widespread degeneration of frontal lobes as the disease progresses.…”

Alexithymia in Amyotrophic Lateral Sclerosis and Its Neural Correlates

“…Patients also have impaired emotional facial expression recognition, impaired emotional responses, impaired excitability in response to emotional stimuli, and impaired judgment of emotional valence ( 4 , 5 ). Affective and cognitive Theory of Mind (ToM) is also affected by the disease ( 6 , 7 ), this finding having been shown to be discrepant when comparing within ALS patients, stratified by bulbar and spinal onset in some studies ( 8 , 9 ), but not in others ( 6 ).…”

Editorial: Biomarkers and Clinical Indicators in Motor Neuron Disease

“…Christidi et al reviews the evidence for memory deficits in MND with a painstaking analysis of the available clinical(52), radiological (1) and post mortem literature(53). Professor Mioshi's group eloquently reviews the impact of cognitive and behavioral deficits in ALS on patients and caregivers drawing attention to an important aspect of ALS care which is relatively understudied(54,55).The novelty of their analysis lies in the identification of viable non-pharmaceutical strategies to improve patient and caregiver well-being (Caga et al). Grollemund et al perform an in-depth analysis of the ever expanding literature of machine-learning in MND, and discuss the advantages and drawbacks of specific mathematical models.…”