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 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.

scholarly journals Speech Metrics and Samples That Differentiate Between Nonfluent/Agrammatic and Logopenic Variants of Primary Progressive Aphasia

2021 ◽  
pp. 1-22
Author(s):  
Katarina L. Haley ◽  
Adam Jacks ◽  
Jordan Jarrett ◽  
Taylor Ray ◽  
Kevin T. Cunningham ◽  
...  
Keyword(s):  
Large Scale ◽  
Primary Progressive Aphasia ◽  
Diagnostic Tools ◽  
Future Research ◽  
Apraxia Of Speech ◽  
Connected Speech ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Syllable Duration ◽  
Semantic Variant

Purpose Of the three currently recognized variants of primary progressive aphasia, behavioral differentiation between the nonfluent/agrammatic (nfvPPA) and logopenic (lvPPA) variants is particularly difficult. The challenge includes uncertainty regarding diagnosis of apraxia of speech, which is subsumed within criteria for variant classification. The purpose of this study was to determine the extent to which a variety of speech articulation and prosody metrics for apraxia of speech differentiate between nfvPPA and lvPPA across diverse speech samples. Method The study involved 25 participants with progressive aphasia (10 with nfvPPA, 10 with lvPPA, and five with the semantic variant). Speech samples included a word repetition task, a picture description task, and a story narrative task. We completed acoustic analyses of temporal prosody and quantitative perceptual analyses based on narrow phonetic transcription and then evaluated the degree of differentiation between nfvPPA and lvPPA participants (with the semantic variant serving as a reference point for minimal speech production impairment). Results Most, but not all, articulatory and prosodic metrics differentiated statistically between the nfvPPA and lvPPA groups. Measures of distortion frequency, syllable duration, syllable scanning, and—to a limited extent—syllable stress and phonemic accuracy showed greater impairment in the nfvPPA group. Contrary to expectations, classification was most accurate in connected speech samples. A customized connected speech metric—the narrative syllable duration—yielded excellent to perfect classification accuracy. Discussion Measures of average syllable duration in multisyllabic utterances are useful diagnostic tools for differentiating between nfvPPA and lvPPA, particularly when based on connected speech samples. As such, they are suitable candidates for automatization, large-scale study, and application to clinical practice. The observation that both speech rate and distortion frequency differentiated more effectively in connected speech than on a motor speech examination suggests that it will be important to evaluate interactions between speech and discourse production in future research.

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

eLife ◽  
2021 ◽  
Vol 10 ◽  
Author(s):  
Valentina Borghesani ◽  
Corby L Dale ◽  
Sladjana Lukic ◽  
Leighton BN Hinkley ◽  
Michael Lauricella ◽  
...  
Keyword(s):  
Primary Progressive Aphasia ◽  
Superior Temporal Gyrus ◽  
Semantic Knowledge ◽  
Clinical Syndrome ◽  
Perceptual Processing ◽  
Behavioral Performance ◽  
Semantic Representations ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Semantic Variant

Semantic representations are 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. We test the hypothesis that svPPA patients perform semantic tasks by over-recruiting areas implicated in perceptual processing. We compared MEG recordings of svPPA patients and healthy controls during a categorization task. While behavioral performance did not differ, svPPA patients showed indications of greater activation over bilateral occipital cortices and superior temporal gyrus, and inconsistent engagement of frontal regions. These findings suggest 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 seemingly compensated via enhanced perceptual processing.

scholarly journals Primary Progressive Aphasia: Toward a Pathophysiological Synthesis

2021 ◽  
Vol 21 (3) ◽  
Author(s):  
Justina Ruksenaite ◽  
Anna Volkmer ◽  
Jessica Jiang ◽  
Jeremy CS Johnson ◽  
Charles R Marshall ◽  
...  
Keyword(s):  
Care Management ◽  
Primary Progressive Aphasia ◽  
Disease Stage ◽  
Major Step ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
New Findings ◽  
Critical Issues ◽  
Semantic Variant ◽  
Auditory Impairments

Abstract Purpose of Review The term primary progressive aphasia (PPA) refers to a diverse group of dementias that present with prominent and early problems with speech and language. They present considerable challenges to clinicians and researchers. Recent Findings Here, we review critical issues around diagnosis of the three major PPA variants (semantic variant PPA, nonfluent/agrammatic variant PPA, logopenic variant PPA), as well as considering ‘fragmentary’ syndromes. We next consider issues around assessing disease stage, before discussing physiological phenotyping of proteinopathies across the PPA spectrum. We also review evidence for core central auditory impairments in PPA, outline critical challenges associated with treatment, discuss pathophysiological features of each major PPA variant, and conclude with thoughts on key challenges that remain to be addressed. Summary New findings elucidating the pathophysiology of PPA represent a major step forward in our understanding of these diseases, with implications for diagnosis, care, management, and therapies.

scholarly journals Breakdowns in Informativeness of Naturalistic Speech Production in Primary Progressive Aphasia

2021 ◽  
Vol 11 (2) ◽  
pp. 130
Author(s):  
Jeanne Gallée ◽  
Claire Cordella ◽  
Evelina Fedorenko ◽  
Daisy Hochberg ◽  
Alexandra Touroutoglou ◽  
...  
Keyword(s):  
Primary Progressive Aphasia ◽  
Speech Analysis ◽  
Clinical Settings ◽  
Functional Communication ◽  
Or Efficiency ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Communication Impairment ◽  
Semantic Variant ◽  
Lower Production

“Functional communication” refers to an individual’s ability to communicate effectively in his or her everyday environment, and thus is a paramount skill to monitor and target therapeutically in people with aphasia. However, traditional controlled-paradigm assessments commonly used in both research and clinical settings often fail to adequately capture this ability. In the current study, facets of functional communication were measured from picture-elicited speech samples from 70 individuals with mild primary progressive aphasia (PPA), including the three variants, and 31 age-matched controls. Building upon methods recently used by Berube et al. (2019), we measured the informativeness of speech by quantifying the content of each patient’s description that was relevant to a picture relative to the total amount of speech they produced. Importantly, form-based errors, such as mispronunciations of words, unusual word choices, or grammatical mistakes are not penalized in this approach. We found that the relative informativeness, or efficiency, of speech was preserved in non-fluent variant PPA patients as compared with controls, whereas the logopenic and semantic variant PPA patients produced significantly less informative output. Furthermore, reduced informativeness in the semantic variant is attributable to a lower production of content units and a propensity for self-referential tangents, whereas for the logopenic variant, a lower production of content units and relatively ”empty” speech and false starts contribute to this reduction. These findings demonstrate that functional communication impairment does not uniformly affect all the PPA variants and highlight the utility of naturalistic speech analysis for measuring the breakdown of functional communication in PPA.

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