Social Cognition deficits in Amyotrophic Lateral Sclerosis: a pilot cross-sectional population-based study

Background: Social Cognition (SC) deficits are included in Amyotrophic Lateral Sclerosis-Frontotemporal spectrum disorder (ALS-FTDS) revised diagnostic criteria. However, SC performances among ALS patients are heterogeneous due to the phenotypic variability of the disease and the wide range of neuropsychological tools employed. The aim of the present study was to assess Facial Emotion Recognition (FER) and Theory of Mind (ToM) in ALS patients compared to controls and to evaluate the correlations with the other cognitive domains and degree of motor impairment. Methods: 83 ALS patients and 42 controls underwent a cognitive evaluation and SC assessment through the Ekman-60 faces test (EK-60F), the Reading the Mind in the Eyes-Test (RMET-36) and the Story-Based Empathy Task (SET). Results: ALS patients showed significantly worse performances compared to controls in EK-60F test global score (p<0.001), recognition of disgust (p 0.032), anger (p 0.038), fear, sadness, RMET-36 and SET-GS (p < 0.001). Also Cognitively normal patients (ALS-CN) showed significantly worse performances compared to controls in EK-60F test global score (p <0.001), recognition of fear (p 0.002), sadness and SET (p < 0.001). RMET-36 showed a significant correlation with the Category Fluency test (p 0.041). SC tests showed no correlation with motor impairment expressed by ALSFRS-R. Conclusions: ALS patients, also when categorized as ALS-CN, may show impairment in SC performances. The quite frequent identification of early SC impairment in ALS patients, supports the need to routinely assess SC for its impact on end-of-life decisions and its potential influence on patients' quality of life.