The Importance of Being Familiar: The Role of Semantic Knowledge in the Activation of Emotions and Factual Knowledge from Music in the Semantic Variant of Primary Progressive Aphasia

2021 ◽  
pp. 1-14
Author(s):  
Joël Macoir ◽  
Marie-Pier Tremblay ◽  
Maximiliano A. Wilson ◽  
Robert Laforce ◽  
Carol Hudon
Keyword(s):  
Emotion Recognition ◽  
Primary Progressive Aphasia ◽  
Theoretical Models ◽  
Semantic Knowledge ◽  
Factual Knowledge ◽  
Basic Emotions ◽  
Musical Excerpts ◽  
Semantic Concepts ◽  
Semantic Variant

Background: The role of semantic knowledge in emotion recognition remains poorly understood. The semantic variant of primary progressive aphasia (svPPA) is a degenerative disorder characterized by progressive loss of semantic knowledge, while other cognitive abilities remain spared, at least in the early stages of the disease. The syndrome is therefore a reliable clinical model of semantic impairment allowing for testing the propositions made in theoretical models of emotion recognition. Objective: The main goal of this study was to investigate the role of semantic memory in the recognition of basic emotions conveyed by music in individuals with svPPA. Methods: The performance of 9 individuals with svPPA was compared to that of 32 control participants in tasks designed to investigate the ability: a) to differentiate between familiar and non-familiar musical excerpts, b) to associate semantic concepts to musical excerpts, and c) to recognize basic emotions conveyed by music. Results: Results revealed that individuals with svPPA showed preserved abilities to recognize familiar musical excerpts but impaired performance on the two other tasks. Moreover, recognition of basic emotions and association of musical excerpts with semantic concepts was significantly better for familiar than non-familiar musical excerpts in participants with svPPA. Conclusion: Results of this study have important implications for theoretical models of emotion recognition and music processing. They suggest that impairment of semantic memory in svPPA affects both the activation of emotions and factual knowledge from music and that this impairment is modulated by familiarity with musical tunes.

scholarly journals When affect overlaps with concept: emotion recognition in semantic variant of primary progressive aphasia

Brain ◽  
2020 ◽  
Author(s):  
Maxime Bertoux ◽  
Harmony Duclos ◽  
Marie Caillaud ◽  
Shailendra Segobin ◽  
Catherine Merck ◽  
...  
Keyword(s):  
Emotion Recognition ◽  
Conceptual Knowledge ◽  
Grey Matter ◽  
Primary Progressive Aphasia ◽  
Semantic Knowledge ◽  
Facial Emotion Recognition ◽  
Facial Emotion ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Semantic Variant

Abstract The most recent theories of emotions have postulated that their expression and recognition depend on acquired conceptual knowledge. In other words, the conceptual knowledge derived from prior experiences guide our ability to make sense of such emotions. However, clear evidence is still lacking to contradict more traditional theories, considering emotions as innate, distinct and universal physiological states. In addition, whether valence processing (i.e. recognition of the pleasant/unpleasant character of emotions) also relies on semantic knowledge is yet to be determined. To investigate the contribution of semantic knowledge to facial emotion recognition and valence processing, we conducted a behavioural and neuroimaging study in 20 controls and 16 patients with the semantic variant of primary progressive aphasia, a neurodegenerative disease that is prototypical of semantic memory impairment, and in which an emotion recognition deficit has already been described. We assessed participants’ knowledge of emotion concepts and recognition of 10 basic (e.g. anger) or self-conscious (e.g. embarrassment) facial emotional expressions presented both statically (images) and dynamically (videos). All participants also underwent a brain MRI. Group comparisons revealed deficits in both emotion concept knowledge and emotion recognition in patients, independently of type of emotion and presentation. These measures were significantly correlated with each other in patients and with semantic fluency in patients and controls. Neuroimaging analyses showed that both emotion recognition and emotion conceptual knowledge were correlated with reduced grey matter density in similar areas within frontal ventral, temporal, insular and striatal regions, together with white fibre degeneration in tracts connecting frontal regions with each other as well as with temporal regions. We then performed a qualitative analysis of responses made during the facial emotion recognition task, by delineating valence errors (when one emotion was mistaken for another of a different valence), from other errors made during the emotion recognition test. We found that patients made more valence errors. The number of valence errors correlated with emotion conceptual knowledge as well as with reduced grey matter volume in brain regions already retrieved to correlate with this score. Specificity analyses allowed us to conclude that this cognitive relationship and anatomical overlap were not mediated by a general effect of disease severity. Our findings suggest that semantic knowledge guides the recognition of emotions and is also involved in valence processing. Our study supports a constructionist view of emotion recognition and valence processing, and could help to refine current theories on the interweaving of semantic knowledge and emotion processing.

scholarly journals The role of the hippocampus in the semantic variant of primary progressive aphasia: A resting‐state fcMRI study

Hippocampus ◽  
2019 ◽  
Vol 29 (11) ◽  
pp. 1127-1132
Author(s):  
Marianne Chapleau ◽  
Maxime Montembeault ◽  
Mariem Boukadi ◽  
Christophe Bedetti ◽  
Robert Laforce ◽  
...  
Keyword(s):  
Resting State ◽  
Primary Progressive Aphasia ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Semantic Variant

scholarly journals Right Anterior Temporal Degeneration, Emotions, and Loss of Nonverbal Semantics: Guidelines for Diagnosis of the Emotional Semantic Variant Frontotemporal Dementia

2021 ◽  
Author(s):  
Kyan Younes ◽  
Valentina Borghesani ◽  
Maxime Montembeault ◽  
Salvatore Spina ◽  
Ariane E Welch ◽  
...  
Keyword(s):  
Frontotemporal Dementia ◽  
Right Hemisphere ◽  
Clinical Symptoms ◽  
Neuropsychological Testing ◽  
Primary Progressive Aphasia ◽  
Structural Mri ◽  
Underlying Mechanism ◽  
Semantic Knowledge ◽  
Clinical Syndrome ◽  
Semantic Variant

Anterior temporal lobe (ATL) degeneration is caused by a pathological process that has a focal onset in the left or right hemisphere. Patients with left-lateralized ATL atrophy typically meet criteria for semantic variant primary progressive aphasia (PPA), a clinical syndrome characterized by loss of verbal semantic knowledge. There is less consensus regarding the symptoms that emerge when atrophy targets the right ATL (rATL), but previous studies have emphasized prosopagnosia as well as alterations in emotion, memory, behavior, and semantic knowledge, symptoms that often lead to a diagnosis of behavioral variant frontotemporal dementia (bvFTD). The goal of the present study was to characterize the cognitive and socioemotional deficits of patients with rATL degeneration in order to refine current conceptualizations of the rATL clinical syndrome. We identified individuals clinically diagnosed as bvFTD or PPA in our cohort of patients prospectively evaluated for FTD-spectrum disorders. We selected patients who also underwent structural MRI and a comprehensive, multidisciplinary evaluation (n = 478). Based on structural MRI atrophy index, individuals with predominant, rATL atrophy (n = 46) were identified and patients with co-occurrence of significant frontal atrophy were excluded. Nineteen patients with rATL degeneration had undergone autopsy. We used the clinical histories to identify early symptoms and examined the cognitive, socioemotional, genetic, and pathological profiles of patients with rATL degeneration. In patients with rATL degeneration, the most common early clinical symptoms were loss of empathy (27%), person-specific semantic knowledge (23%), and complex compulsions (18%). On socioemotional testing and informant-reported measures, patients exhibited diminished interpersonal warmth, empathy, and emotional theory of mind. Neuropsychological testing revealed deficits in identifying famous people and discriminating facial affect despite preserved face perception. FTLD-TDP was the most frequent pathological correlate of rATL degeneration (84%), followed by Pick type (10%), a subtype of FTLD-tau. Our results indicate that patients with early, rATL-predominant degeneration present with a behavioral syndrome that results from loss of empathy for others. The underlying mechanism is a progressive loss of semantic knowledge for concepts of social-emotional relevance. We herein refer to this syndrome as emotional semantic variant frontotemporal dementia. We propose novel diagnostic criteria for this rATL syndrome in order to facilitate early identification in clinical and research settings. This classification is relevant because, if appropriately diagnosed, these patients most often have FTLD-TDP Type-C pathology.

scholarly journals Profile of linguistic and cognitive deficits in persons with a semantic variant of a primary progressive aphasia

2020 ◽  
Vol 54 (3) ◽  
pp. 113-119
Author(s):  
Lana Jerkić ◽  
Dragan Pavlović ◽  
Mile Vuković ◽  
Jelena Todorović ◽  
Mirna Zelić
Keyword(s):  
Specific Treatment ◽  
Primary Progressive Aphasia ◽  
Semantic Knowledge ◽  
Cognitive Changes ◽  
Progressive Aphasia ◽  
Progressive Deterioration ◽  
Primary Progressive ◽  
Language Functions ◽  
Semantic Variant ◽  
Speech And Language Disorders

Primary progressive aphasia (PPA) includes a group of neurodegenerative disorders that are characterized by progressive deterioration of language functions, while other cognitive functions, at least at the onset of the disease, are relatively spared. There are three basic subtypes of PPA: the nonfluent progressive aphasia (nvPPA), the semantic variant of a primary progressive aphasia (svPPA), and the logopenic progressive aphasia (lvPPA). The semantic variant of a PPA can also be found in the literature under the term of semantic dementia. It is clinically manifested by progressive deterioration of semantic knowledge, fluent aphasia, impaired naming and comprehension, prosopagnosia and surface dyslexia and dysgraphia (in languages with irregular orthography). As the disease progresses, other cognitive changes can be observed. The main cause of the disorder is progressive bilateral atrophy of the anterior temporal lobes, which is more manifested in the left hemisphere. The literature is modest in terms of the use of specific treatment methods in the rehabilitation of these patients. Since speech and language disorders are the most conspicuous symptom, at least at the beginning of the disease, the role of speech therapists in the assessment and restitution of speechlanguage and communication skills is also indisputable.

Errorless Learning Therapy in Semantic Variant of Primary Progressive Aphasia

2021 ◽  
Vol 79 (1) ◽  
pp. 415-422
Author(s):  
Núria Montagut ◽  
Sergi Borrego-Écija ◽  
Magdalena Castellví ◽  
Immaculada Rico ◽  
Ramón Reñé ◽  
...  
Keyword(s):  
Speech Therapy ◽  
Primary Progressive Aphasia ◽  
Semantic Knowledge ◽  
Errorless Learning ◽  
Short Term ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Semantic Variant ◽  
Naming Tasks

Background: The semantic variant of primary progressive aphasia (svPPA) is characterized by a progressive loss of semantic knowledge impairing the ability to name and to recognize the meaning of words. Objective: We aimed to evaluate the immediate and short-term effect of errorless learning speech therapy on the naming and recognition of commonly used words in patients with svPPA. Methods: Eight participants diagnosed with svPPA received 16 sessions of intensive errorless learning speech therapy. Naming and word comprehension tasks were evaluated at baseline, immediately postintervention, and at follow-up after 1, 3, and 6 months. These evaluations were performed using two item sets (a trained list and an untrained list). Results: In the naming tasks, patients showed a significant improvement in trained items immediately after the intervention, but that improvement decayed progressively when therapy ended. No improvements were found either in trained comprehension or in untrained tasks. Conclusion: Errorless learning therapy could improve naming ability in patients with svPPA. This effect may be due to the relative preservation of episodic memory, but the benefit is not maintained over time, presumably because there is no consolidation.

scholarly journals Semantic Variant Primary Progressive Aphasia: Practical Recommendations for Treatment from 20 Years of Behavioural Research

2021 ◽  
Vol 11 (12) ◽  
pp. 1552
Author(s):  
Aida Suárez-González ◽  
Sharon A. Savage ◽  
Nathalie Bier ◽  
Maya L. Henry ◽  
Regina Jokel ◽  
...  
Keyword(s):  
Best Practice ◽  
Current Knowledge ◽  
Primary Progressive Aphasia ◽  
Memory Systems ◽  
Semantic Knowledge ◽  
Future Research ◽  
Long Term Memory ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Semantic Variant

People with semantic variant primary progressive aphasia (svPPA) present with a characteristic progressive breakdown of semantic knowledge. There are currently no pharmacological interventions to cure or slow svPPA, but promising behavioural approaches are increasingly reported. This article offers an overview of the last two decades of research into interventions to support language in people with svPPA including recommendations for clinical practice and future research based on the best available evidence. We offer a lay summary in English, Spanish and French for education and dissemination purposes. This paper discusses the implications of right- versus left-predominant atrophy in svPPA, which naming therapies offer the best outcomes and how to capitalise on preserved long-term memory systems. Current knowledge regarding the maintenance and generalisation of language therapy gains is described in detail along with the development of compensatory approaches and educational and support group programmes. It is concluded that there is evidence to support an integrative framework of treatment and care as best practice for svPPA. Such an approach should combine rehabilitation interventions addressing the language impairment, compensatory approaches to support activities of daily living and provision of education and support within the context of dementia.

scholarly journals Neural dynamics of semantic categorization in semantic variant of Primary Progressive Aphasia

2020 ◽  
Author(s):  
V. Borghesani ◽  
C. L. Dale ◽  
S. Lukic ◽  
L. B. N. Hinkley ◽  
M. Lauricella ◽  
...  
Keyword(s):  
Primary Progressive Aphasia ◽  
Superior Temporal Gyrus ◽  
Semantic Knowledge ◽  
Clinical Syndrome ◽  
Perceptual Processing ◽  
Semantic Representations ◽  
Semantic Categorization ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Semantic Variant

AbstractAwake humans constantly extract conceptual information from a flow of perceptual inputs. Category membership (e.g., is it an animate or inanimate thing?) is a critical semantic feature used to determine the appropriate response to a stimulus. Semantic representations are thought to be processed along a posterior-to-anterior gradient reflecting a shift from perceptual (e.g., it has eight legs) to conceptual (e.g., venomous spiders are rare) information. One critical region is the anterior temporal lobe (ATL): patients with semantic variant primary progressive aphasia (svPPA), a clinical syndrome associated with ATL neurodegeneration, manifest a deep loss of semantic knowledge.Here, we test the hypothesis that svPPA patients, in the absence of an intact ATL, perform semantic tasks by over-recruiting areas implicated in perceptual processing. We acquired MEG recordings of 18 svPPA patients and 18 healthy controls during a semantic categorization task. While behavioral performance did not differ, svPPA patients showed greater activation over bilateral occipital cortices and superior temporal gyrus, and inconsistent engagement of frontal regions.These findings indicate a pervasive reorganization of brain networks in response to ATL neurodegeneration: the loss of this critical hub leads to a dysregulated (semantic) control system, and defective semantic representations are compensated via enhanced perceptual processing.

scholarly journals Differential chemokine alteration in the variants of primary progressive aphasia—a role for neuroinflammation

2021 ◽  
Vol 18 (1) ◽  
Author(s):  
Aitana Sogorb-Esteve ◽  
Imogen J. Swift ◽  
Ione O. C. Woollacott ◽  
Jason D. Warren ◽  
Henrik Zetterberg ◽  
...  
Keyword(s):  
Neurodegenerative Disorders ◽  
Primary Progressive Aphasia ◽  
The Other ◽  
Primary Progressive ◽  
Total Tau ◽  
Semantic Variant ◽  
Primary Focus ◽  
Underlying Pathology ◽  
Proximity Extension Assay

Abstract Background The primary progressive aphasias (PPA) represent a group of usually sporadic neurodegenerative disorders with three main variants: the nonfluent or agrammatic variant (nfvPPA), the semantic variant (svPPA), and the logopenic variant (lvPPA). They are usually associated with a specific underlying pathology: nfvPPA with a primary tauopathy, svPPA with a TDP-43 proteinopathy, and lvPPA with underlying Alzheimer’s disease (AD). Little is known about their cause or pathophysiology, but prior studies in both AD and svPPA have suggested a role for neuroinflammation. In this study, we set out to investigate the role of chemokines across the PPA spectrum, with a primary focus on central changes in cerebrospinal fluid (CSF) Methods Thirty-six participants with sporadic PPA (11 svPPA, 13 nfvPPA, and 12 lvPPA) as well as 19 healthy controls were recruited to the study and donated CSF and plasma samples. All patients with lvPPA had a tau/Aβ42 biomarker profile consistent with AD, whilst this was normal in the other PPA groups and controls. We assessed twenty chemokines in CSF and plasma using Proximity Extension Assay technology: CCL2 (MCP-1), CCL3 (MIP-1a), CCL4 (MIP-1β), CCL7 (MCP-3), CCL8 (MCP-2), CCL11 (eotaxin), CCL13 (MCP-4), CCL19, CCL20, CCL23, CCL25, CCL28, CX3CL1 (fractalkine), CXCL1, CXCL5, CXCL6, CXCL8 (IL-8), CXCL9, CXCL10, and CXCL11. Results In CSF, CCL19 and CXCL6 were decreased in both svPPA and nfvPPA compared with controls whilst CXCL5 was decreased in the nfvPPA group with a borderline significant decrease in the svPPA group. In contrast, CCL2, CCL3 and CX3CL1 were increased in lvPPA compared with controls and nfvPPA (and greater than svPPA for CX3CL1). CXCL1 was also increased in lvPPA compared with nfvPPA but not the other groups. CX3CL1 was significantly correlated with CSF total tau concentrations in the controls and each of the PPA groups. Fewer significant differences were seen between groups in plasma, although in general, results were in the opposite direction to CSF, i.e. decreased in lvPPA compared with controls (CCL3 and CCL19), and increased in svPPA (CCL8) and nfvPPA (CCL13). Conclusion Differential alteration of chemokines across the PPA variants is seen in both CSF and plasma. Importantly, these results suggest a role for neuroinflammation in these poorly understood sporadic disorders, and therefore also a potential future therapeutic target.

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