Accurate processing of emotional information is a critical component of appropriate social interactions and interpersonal relationships. Disturbance of emotion processing is present in frontotemporal dementia (FTD) and is a clinical feature in two of the three subtypes: behavioural-variant FTD and semantic dementia. Emotion processing in progressive nonfluent aphasia, the third FTD subtype, is thought to be mostly preserved, although current evidence is scant. This paper reviews the literature on emotion recognition, reactivity and expression in FTD subtypes, although most studies focus on emotion recognition. The relationship between patterns of emotion processing deficits and patterns of neural atrophy are considered, by integrating evidence from recent neuroimaging studies. The review findings are discussed in the context of three contemporary theories of emotion processing: the limbic system model, the right hemisphere model and a multimodal system of emotion. Results across subtypes of FTD are most consistent with the multimodal system model, and support the presence of somewhat dissociable neural correlates for basic emotions, with strongest evidence for the emotions anger and sadness. Poor emotion processing is evident in all three subtypes, although deficits are more widespread than what would be predicted based on studies in healthy cohorts. Studies that include behavioural and imaging data are limited. Future investigations combining these approaches will help improve the understanding of the neural network underlying emotion processing. Presently, longitudinal investigations of emotion processing in FTD are lacking, and studies investigating emotion processing over time are critical to understand the clinical manifestations of disease progression in FTD.
"Dementias are generally defined by progressive deterioration in cognitive function but often produce less wellcharacterized alterations in emotional, motivational, and social functions. These alterations are particularly early and significant in behavioral variant frontotemporal dementia (bvFTD) and semantic dementia (SD) within the frontotemporal lobar degeneration (FTLD) spectrum        , and are probably underrecognized in progressive nonfluentaphasia (PNFA)  and Alzheimer's disease (AD) [4,10– 12]. However, although emotional disturbances are hallmarks of many dementias and potentially relevant to disease detection, tracking and therapy, the pathophysiology of disturbed emotion in dementia is poorly understood and challenging to measure objectively. "
[Show abstract][Hide abstract] ABSTRACT: Introduction: Emotional behavioral disturbances are hallmarks of many dementias but their pathophysiology is poorly understood. Here we addressed this issue using the paradigm of emotionally salient sounds. Methods: Pupil responses and affective valence ratings for nonverbal sounds of varying emotional salience were assessed in patients with behavioral variant frontotemporal dementia (bvFTD) (n=14), semantic dementia (SD) (n=10), progressive nonfluent aphasia (PNFA) (n=12), and AD (n=10) versus healthy age-matched individuals (n=26). Results: Referenced to healthy individuals, overall autonomic reactivity to sound was normal in Alzheimer's disease (AD) but reduced in other syndromes. Patients with bvFTD, SD, and AD showed altered coupling between pupillary and affective behavioral responses to emotionally salient sounds. Discussion: Emotional sounds are a useful model system for analyzing how dementias affect the processing of salient environmental signals, with implications for defining pathophysiological mechanisms and novel biomarker development.
"SD is typically characterised as a language disorder, with prominent anomia and loss of general conceptual knowledge. Increasingly , however, it is recognised that deficits in social cognition and emotional functioning are also present in this syndrome (Hsieh et al., 2013a; Irish et al., 2014a; Kumfor et al., 2013, 2011; Kumfor and Piguet 2012; Rankin et al., 2009; Rosen et al., 2002). "
"These are subjective ratings of emotional reactions and experience , and though some responses may be conventionally more appropriate , none are strictly incorrect . These deficits in emotion recognition and emotional mirroring for negative stimuli may be the result of greater atrophy in regions involved in the processing and re - cognition of negative emotions ( Kumfor and Piguet , 2012 ) . More specifically , negative emotions such as fear and disgust appear to have more focal representation in the brain , such that lesions to specific regions can result in relatively selective recognition defi - cits for these expressions ( Adolphs et al . "
[Show abstract][Hide abstract] ABSTRACT: Objectives:
Behavioural variant frontotemporal dementia (bvFTD) is a debilitating neurodegenerative disorder characterized by frontal and temporal lobe atrophy primarily affecting social cognition and emotion, including loss of empathy. Many consider empathy to be a multidimensional construct, including cognitive empathy (the ability to adopt and understand another's perspective) and emotional empathy (the capacity to share another's emotional experience). Cognitive and emotional empathy deficits have been associated with bvFTD; however, little is known regarding the performance of patients with bvFTD on behavioural measures of emotional empathy, and whether empathic responses differ for negative versus positive stimuli.
24 patients with bvFTD and 24 healthy controls completed the Multifaceted Empathy Test (MET; Dziobek et al., 2008), a performance-based task that taps both cognitive and emotional facets of empathy, and allows for the discrimination of responses to negative versus positive realistic images. MET scores were also compared with caregiver ratings of patient behaviour on the Interpersonal Reactivity Index, which assesses patients' everyday demonstrations of perspective taking and empathic concern.
Patients with bvFTD were less accurate than controls at inferring mental states for negative and positive stimuli. They also demonstrated lower levels of shared emotional experience, more positive emotional reactions, and diminished arousal to negative social stimuli relative to controls. Patients showed reduced emotional reactions to negative non-social stimuli as well. Lastly, the MET and IRI measures of emotional empathy were found to be significantly correlated within the bvFTD group.
The results suggest that patients with bvFTD show a global deficit in cognitive empathy, and deficient emotional empathy for negative, but not positive, experiences. Further, a generalized emotional processing impairment for negative stimuli was observed, which could contribute to the emotional empathy deficit. This work highlights potential treatment targets and a means to assess the impact of novel therapies on socioemotional impairment in bvFTD.
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