Discordant performance on the 'Reading the Mind in the Eyes' Test, based on disease onset in amyotrophic lateral...

Executive dysfunction is a core feature of amyotrophic lateral sclerosis (ALS) and is associated with brain atrophy in cortical and subcortical regions. Social cognitive deficits may also be a prominent feature of ALS. This study investigated executive, and social cognitive performance, in a population based cohort of patients with ALS, stratified by disease onset. Participants were recruited as part of a population based study investigating cognitive decline in ALS. Patients carrying pathogenic C9orf72 hexanucleotide repeat were excluded. Participants were stratified based on bulbar (n = 20) or spinal (n = 39) disease onset (n = 59). Matched healthy controls were used to generate culturally specific comparative data for within-patient analyses (n = 59). Results showed that ALS patients performed significantly worse than controls on a number of measures of executive function. When sub-stratified by disease onset, there was a significant difference between bulbar- and spinal-onset patients with respect to the 'Reading the Mind in the Eyes' Test scores (p < 0.001). Conversely, standardized scores of executive function did not differ between the patient groups. In conclusion, patients performed significantly worse than matched controls on measures of executive function. Bulbar-onset ALS patients evidenced more social-affective deficits compared to spinal-onset patients, with matched performance on measures of executive function.