Beyond disgust: impaired recognition of negative emotions prior to diagnosis in Huntington's disease

Abstract

Previous studies of emotion recognition suggest that detection of disgust relies on processing within the basal ganglia and insula. Research involving individuals with symptomatic and pre-diagnostic Huntington's disease (HD), a disease with known basal ganglia atrophy, has generally indicated a relative impairment in recognizing disgust. However, some data have suggested that recognition of other emotions (particularly fear and anger) may also be affected in HD, and a recent study found fear recognition deficits in the absence of other emotion-recognition impairments, including disgust. To further examine emotion recognition in HD, we administered a computerized facial emotion recognition task to 475 individuals with the HD CAG expansion and 57 individuals without. Logistic regression was used to examine associations of emotion recognition performance with estimated proximity to clinical diagnosis (based on CAG repeat length and current age) and striatal volumes. Recognition of anger, disgust, fear, sadness and surprise (but not happiness) was associated with estimated years to clinical diagnosis; performance was unrelated to striatal volumes. Compared to a CAG-normal control group, the CAG-expanded group demonstrated significantly less accurate recognition of all negative emotions (anger, disgust, fear, sadness). Additionally, participants with more pronounced motor signs of HD were significantly less accurate at recognizing negative emotions than were individuals with fewer motor signs. Findings indicate that recognition of all negative emotions declines early in the disease process, and poorer performance is associated with closer proximity to clinical diagnosis. In contrast to previous results, we found no evidence of relative impairments in recognizing disgust or fear, and no evidence to support a link between the striatum and disgust recognition.

Emotion perception and electrophysiological correlates in Huntington’s disease

“…Snowden et al, (2008) failed to find impairments in neutral facial discrimination in HD. Moreover, impairments were not found in premanifest HD (Johnson et al, 2007) or in symptomatic HD using neutral video clips (Dogan et al, 2013). It is unclear what may be driving the difference in results between this study and that by Snowden et al, (2008) since there is limited information on the neutral face performance results provided by Snowden et al, (2008).…”

“…However, as impairment to angry faces was also observed, the present study does not support the notion that disgust (or the neural processes subserving its recognition) is qualitatively different to other negative emotions, but accords well with recent studies reporting deficits in other negative emotion processing (specifically anger). This suggests a more universal deficit in negative emotion processing (Calder et al, 2010, Dogan et al, 2013, Henley et al, 2008, Johnson et al, 2007, Snowden et al, 2008. It should be noted however that as the present study did not use sad or fearful faces, it was not able to determine the degree of generalizability.…”

“…Behavioural: Accuracy was defined for each emotion type as correct, incorrect or no response, and in line with previous literature (Johnson et al, 2007) calculated as a percentage of the total number of stimuli for each emotion type. Intensity was defined as the average Likert response per emotion type.…”

“…This study aimed to investigate for the first time the relationship between emotion perception deficits and underlying neurophysiological indices in symptomatic HD participants. We adopted a similar behavioural paradigm to that used previously (see Johnson et al, 2007), with negative (disgust and anger), neutral and positive (happy) emotion types to further substantiate whether deficits in emotion perception are disgustspecific or generalise to another negative emotion (anger). Moreover, by measuring ERPs, this study also characterised the nature of the reported neurophysiological modulation in HD in response to emotional faces.…”

The impairment of emotion recognition in Huntington’s disease extends to positive emotions

“…However, several studies have documented a more general impairment encompassing several negative emotions in clinical patients or virtually all negative emotions across both pre-clinical and clinical individuals (e.g., De Gelder et al, 2008;Johnson et al, 2007;Snowden, et al, 2008). Some authors have argued that the selective impairment for disgust stimuli found in some studies may have been a consequence of item difficulty (Milders et al, 2003).…”