Journal
AMYOTROPHIC LATERAL SCLEROSIS AND FRONTOTEMPORAL DEGENERATION
Volume 17, Issue 7-8, Pages 467-472Publisher
TAYLOR & FRANCIS LTD
DOI: 10.1080/21678421.2016.1177088
Keywords
ALS; neuropsychology; social cognition; executive function
Categories
Funding
- European Community's Seventh Framework Programme [259867]
- JPND SOPHIA project
- Irish Health Research Board
- Irish Institute of Clinical Neuroscience/Novartis
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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.
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