Abstract
SUMMARY There is an overlap between amyotrophic lateral sclerosis and frontotemporal dementia. Approximately 15% of amyotrophic lateral sclerosis patients suffer from frontotemporal dementia characterized by behavioral change while a further third experience subtle executive dysfunction (typically letter fluency deficits) and corresponding prefrontal changes. Behavior change appears prevalent with apathy being the most prominent feature. Reports of social and emotional cognition deficits are increasing. Deficits have been described on theory of mind tasks including interpretation of stories and cartoons, faux pas detection and in the judgment of preference based on direction of eye-gaze. Impairments in emotional face and prosody perception and emotional enhancement of memory have been reported, and decision making (with and without risk) appears affected. The role of executive dysfunction in this social cognition deficit remains unresolved and more direct evidence of oribitofrontal involvement has yet to be found. Implications for healthcare provision are discussed with deterioration of social interaction with carers predicted.
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