title: Discordant performance on the 'Reading the Mind in the Eyes' Test, based on disease onset in amyotrophic lateral sclerosis.
abstract: 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.
authors.affiliation: a Departments of Psychology , Trinity College , Dublin.|b Academic Unit of Neurology , Trinity Biomedical Sciences Institute, Trinity College Dublin.
authors.forename: Tom
authors.initials: T
authors.lastname: Burke
authors.affiliation: b Academic Unit of Neurology , Trinity Biomedical Sciences Institute, Trinity College Dublin.
authors.forename: Marwa
authors.initials: M
authors.lastname: Elamin
authors.affiliation: b Academic Unit of Neurology , Trinity Biomedical Sciences Institute, Trinity College Dublin.
authors.forename: Peter
authors.initials: P
authors.lastname: Bede
authors.affiliation: a Departments of Psychology , Trinity College , Dublin.|b Academic Unit of Neurology , Trinity Biomedical Sciences Institute, Trinity College Dublin.
authors.forename: Marta
authors.initials: M
authors.lastname: Pinto-Grau
authors.affiliation: a Departments of Psychology , Trinity College , Dublin.|b Academic Unit of Neurology , Trinity Biomedical Sciences Institute, Trinity College Dublin.
authors.forename: Katie
authors.initials: K
authors.lastname: Lonergan
authors.affiliation: b Academic Unit of Neurology , Trinity Biomedical Sciences Institute, Trinity College Dublin.|c Neurology , Beaumont Hospital , Dublin.
authors.forename: Orla
authors.initials: O
authors.lastname: Hardiman
authors.affiliation: a Departments of Psychology , Trinity College , Dublin.|b Academic Unit of Neurology , Trinity Biomedical Sciences Institute, Trinity College Dublin.|d Department of Psychology , Royal College of Surgeons , Dublin , Ireland.
authors.forename: Niall
authors.initials: N
authors.lastname: Pender
mesh_terms: D000328:Adult; D000339:Affect / Q000502:physiology; D000368:Aged; D000369:Aged, 80 and over; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications* / Q000235:genetics; D000073885:C9orf72 Protein; D003072:Cognition Disorders / Q000175:diagnosis* / Q000209:etiology*; D015331:Cohort Studies; D056344:Executive Function / Q000502:physiology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009154:Mutation / Q000235:genetics; D009483:Neuropsychological Tests; D011506:Proteins / Q000235:genetics; D011569:Psychiatric Status Rating Scales; D011932:Reading; D012919:Social Behavior
chemical_list: D000073885:C9orf72 Protein; C568651:C9orf72 protein, human; D011506:Proteins
keywords: ALS; executive function; neuropsychology; social cognition