Deficits in Emotion Recognition as Markers of Frontal Behavioral Dysfunction in Amyotrophic Lateral Sclerosis

Abstract

Background: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease with prominent motor symptoms. ALS patients may also manifest frontal behavior and cognitive decline, including impairment in facial emotion recognition. We aimed to investigate whether deficits in emotion recognition are associated with frontal behavior symptoms in ALS. Methods: We enrolled two groups of participants: 1) patients with probable or definite sporadic ALS (n= 21, 11 male/10 female; median age 62 years-old; median disease duration = 3 years) and 2) age and education matched controls (n = 25, 18 male/11 female; median age 61 years-old). The Facial Emotion Recognition Test (FERT) was applied to all participants. Patients underwent the Cambridge Behavior Inventory-Revised (CBI-R) and were classified according to the presence of frontal behavioral symptoms into two groups: ALS with no behavioral symptom (ALSns, n = 9) and ALS with at least one behavioral symptom (ALSbs, n = 12). Results: Apathy and mood symptoms were the most frequent neuropsychiatric symptoms in ALS. ALS patients performed worse than controls in the recognition of sadness (p<0.004). There was no difference between controls and ALSns for all FERT scores, but ALSbs had lower performance than controls in sadness (p<0.003). Conclusion: Emotion recognition deficit may be a marker of frontal behavior in ALS.

Emotional Processing and Experience in Amyotrophic Lateral Sclerosis: A Systematic and Critical Review

“…ALS sample sizes are relatively small in most studies: only one study has more than one-hundred patients [25]; four studies have 50-100 included patients [2,16,26,27]; nineteen studies have 20-50 patients [9,18,19,24,[28][29][30][31][32][33][34][35][36][37][38][39][40][41]; thirteen studies recruited 10-20 ALS patients [21][22][23][42][43][44][45][46][47][48][49][50][51]; two studies had 2-10 patients [52,53]. Two were single case studies [17,20].…”

“…Some articles showed in ALS with cognitive or behavioral impairments subgroups lower performances than control participants in emotion recognition tasks, while the ALS total group was not impaired [35,36]. Differently, other studies found a similar proportion of patients with and without cognitive/behavioral deficits among those who presented impaired recognition of emotions according to facial expressions or story-based emotion recognition tests [23,31].…”

“…Some study did not reported the most impaired type of emotion [2,16,25,34,40,46]. Other Authors specifically highlighted an impairment in sadness, anger, disgust or surprise recognition [19,23,27,28,31,34,36,37,44,54]; in few cases, also the recognition of happiness was involved [33].…”

“…The majority of reviewed studies investigated emotion recognition in ALS by means of static facial expressions of emotions [2,9,16,19,20,23,24,27,28,[32][33][34][35][36][37][38]40,43,44,46,47,[49][50][51]54]. Other studies evaluated emotion recognition abilities through static scenes [2,21,32,44], dynamic social vignettes [17][18][19][20]27,38], written stories [40,41], and vocal prosody [20,23,48,54].…”

Structural and functional underpinnings of precentral abnormalities in amyotrophic lateral sclerosis

“…This finding is consistent with previous studies, which reported decreased GM volume and thickness in several brain regions, including the SFG and STG [25,26] Functionally, the SFG, which is located in the dorsolateral prefrontal cortex, has been shown to play a crucial role in working memory [27] and attention [28] The STG has been involved in speech perception [29] and emotion recognition [30] In ALS, a number of studies have reported varying degrees of impairments in the language, cognitive, and behavioural domains, such as frontal executive deficits that involve verbal fluency, attention, and working memory [31,32] and facial or prosodic emotion recognition deficits, especially for disgust and surprise [33,34] The finding of significant cortical thinning in the SFG and STG might be the anatomical substrate underlying such nonmotor impairments in patients with ALS and adds further evidence for a continuum between ALS and frontotemporal dementia.…”