Emotion processing deficits distinguish pure amyotrophic lateral sclerosis from frontotemporal dementia

Abstract Amyotrophic lateral sclerosis (ALS) is a multisystem disease that overlaps with frontotemporal dementia (FTD). Although FTD patients exhibit prominent deficits in emotion perception and social cognition, these domains have received relatively little attention in ALS. Moreover, direct comparisons between ALS and FTD on emotion processing tasks remain lacking. Twenty-nine patients with ALS (16 with coexisting FTD (FTD-ALS) and 13 without dementia), 25 behavioural variant FTD patients and 30 healthy controls completed neuropsychological and emotion tasks (Ekman Caricatures and the TASIT). Both ALS and FTD patient groups showed significant deficits on the emotion tasks compared to controls. After dividing ALS patients into those with and without FTD, only the patients with coexisting FTD (FTD-ALS) were impaired. FTD-ALS and FTD patient groups displayed similar levels of impairment, even after controlling for measures of general cognition, and demonstrated similar profiles across different types of emotions. We conclude that patients with FTD-ALS and FTD show similar, significant impairments in emotional processing. By contrast, ALS patients without dementia exhibit preserved emotion processing. Performance on emotion processing tasks may provide a useful clinical tool in identifying those with early FTD-ALS.