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

University of Toronto, Toronto, Ontario, Canada
Brain (Impact Factor: 9.2). 08/2007; 130(Pt 7):1732-44. DOI: 10.1093/brain/awm107
Source: PubMed


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.

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    • "Further studies are needed to determine the precise cerebral correlates of this deficit, but it might be related to the progressive neurodegeneration of brain structures involved in cognitive empathy (particularly orbitofrontal-ventromedial areas). Although our results appear sound in view of the large effect sizes observed, the present data should be replicated and extended on larger samples as the current study was based on small groups in comparison with several earlier ones focusing on emotional processes (e.g.Johnson et al., 2007). Centrally, the self-report nature of the questionnaire might have influenced the results, as clinical HD patients might present reduced insight or self-evaluation abilities (Callaghan et al., 2010). "
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    ABSTRACT: Huntington’s disease (HD) is centrally characterized by motor, neurocognitive and psychiatric symptoms, but impaired emotional decoding abilities have also been reported. However, more complex affective abilities are still to be explored, and particularly empathy which is essential for social relations and is impaired in various psychiatric conditions. This study evaluates empathic abilities and social skills in pre-clinical and clinical HD, and explores the distinction between two empathy sub-components (emotional-cognitive). 36 HD participants (17 pre-clinical) and 36 matched controls filled in the Empathy Quotient Scale, while controlling for psychopathological comorbidities. At the clinical stage of HD, no global empathy impairment was observed but rather a specific deficit for the cognitive sub-component, while emotional empathy was preserved. A deficit was also observed for social skills. Pre-clinical HD was not associated with any empathy deficit. Emotional deficits in clinical HD are thus not limited to basic emotion decoding but extend towards complex interpersonal abilities. The dissociation between impaired cognitive and preserved emotional empathy in clinical HD reinforces the proposal that empathy subtypes are sustained by distinct processes. Finally, these results underline the extent of distinct affective and social impairments in HD and the need to grasp them in clinical contexts.
    Full-text · Article · Jan 2016 · Psychiatry Research
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    • "Social cognition refers to the cognitive processes associated with social interaction, which encompass the ability to recognize, process, and express emotions (Ekman 1992; Ekman 1993). These have been shown to be problematic in neurodegenerative diseases (Müller & Bekkelund 2013), including HD (Johnson et al. 2007). "
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    ABSTRACT: Background. We report the case of a 52-year-old male with pre-motor Huntington’s disease (HD) who has undergone detailed clinical and neuropsychological examination. This patient’s negative symptomatology and behavioural change are having a detrimental impact on his social, occupational and interpersonal life, in the absence of motor symptoms. Methods. The patient has undergone repeat neuropsychological testing (T1 aged 50; T2 aged 52) with particular focus on executive function and social cognition on repeat testing. Results. This case details a specific manifestation of HD relating to behavioural, psychiatric and social affective deficits. Conclusions. This case illustrates how social cognitive changes can occur in HD, months and even years prior to the onset of motor features and how such unrecognized deficits can have a deleterious impact on an individual’s functional ability and lifestyle, before the disease is traditionally considered to have become manifest.
    Full-text · Article · Oct 2015 · Irish journal of psychological medicine
    • "In patients with HD, deficits in emotion recognition (for review see Henley et al., 2012) are already present in the premanifest stage, i.e., prior to the onset of motor signs. Whereas some studies point toward a specific deficit in recognizing facial disgust (Gray et al., 1997; Hennenlotter et al., 2004), others reveal a general impairment in recognizing negative emotions (Johnson et al., 2007; Labuschagne et al., 2013; Rees et al., 2014). However, intact emotion recognition competencies in premanifest HD have also been reported (Novak et al., 2012; Van Asselen et al., 2012), which might be due to variability in disease severity or gene dosage. "
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    ABSTRACT: Neurodegenerative diseases are primarily characterized by motor signs, but are also accompanied by emotional disturbances. Due to the limited knowledge about these dysfunctions, this review provides an overview of emotional competencies in Huntington's disease (HD), Parkinson's disease (PD), and multiple sclerosis (MS), with a focus on emotion recognition, emotion regulation and depression. Most studies indicate facial emotion recognition deficits in HD and PD, whereas data in MS are inconsistent. On a neural level, dysfunctions of amygdala and striatum, amongst others, have been linked to the impairments. These dysfunctions also tap brain regions that are part of the emotion regulation network, suggesting problems in this competency, too. Research points to dysfunctional emotion regulation in MS, whereas findings in PD and HD are missing. The high prevalence of depression in all three disorders emphasizes the need for effective therapies. Research on emotional disturbances might improve treatment, thereby increasing patients' and caregivers' well-being. This article is protected by copyright. All rights reserved. © 2015 Wiley Periodicals, Inc.
    No preview · Article · May 2015 · The Journal of Comparative Neurology
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