Social cognition deficits in amyotrophic lateral sclerosis: A pilot cross-sectional population-based study

Abstract

Background and purpose: Social cognition (SC) deficits are included in amyotrophic lateral sclerosis (ALS)-frontotemporal spectrum disorder revised diagnostic criteria. However, SC performance among ALS patients is 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 and theory of mind in ALS patients compared to controls and to evaluate correlations with the other cognitive domains and degree of motor impairment.

Methods: Eighty-three 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-36 Faces (RMET-36), and the Story-Based Empathy Task (SET).

Results: ALS patients showed significantly worse performance compared to controls in EK-60F global score (p < 0.001), recognition of disgust (p = 0.032), anger (p = 0.038), fear (p < 0.001), and sadness (p < 0.001); RMET-36 (p < 0.001), and SET global score (p < 0.001). Also, cognitively normal patients (ALS-CN) showed significantly worse performance compared to controls in EK-60F global score (p < 0.001), recognition of fear (p = 0.002), sadness (p < 0.001), 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 Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised.

Conclusions: ALS patients, also when categorized as ALS-CN, may show impairment in SC performance. The 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.

Keywords: cognitive disorders and dementia; motor neuron disease; social cognition.