Advances in Primary Progressive Aphasia

A special issue of Brain Sciences (ISSN 2076-3425). This special issue belongs to the section "Neurolinguistics".

Deadline for manuscript submissions: closed (10 August 2021) | Viewed by 37852

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Special Issue Editors

Department of Neurology, Institute of Neurosciences, Hospital Clinico San Carlos, San Carlos Institute for Health Research (IdISSC), Universidad Complutense de Madrid, 28223 Madrid, Spain
Interests: Alzheimer disease; neurodegenerative diseases; cognition; cognitive post-COVID-19 disease; neuroimaging
Special Issues, Collections and Topics in MDPI journals
Clinique Interdisciplinaire de Mémoire (CIME) du CHU de Québec, Département des Sciences Neurologiques, Québec, QC G3N 0A4 , Canada
Interests: atypical dementia; frontotemporal dementia; primary progressive aphasia; molecular neuroimaging; biomarkers; cognitive screening tests
Special Issues, Collections and Topics in MDPI journals
Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA
Interests: frontotemporal dementia; aphasia; apraxia of speech; neuroimaging; electroencephalography
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Primary progressive aphasia is a clinical syndrome that includes a group of neurodegenerative disorders characterized by progressive language impairment. Our knowledge about this disorder has evolved significantly in recent years. Notably, correlations between clinical findings and pathology have improved, and main clinical, neuroimaging, and genetic features have been described. Furthermore, primary progressive aphasia is a good model for the study of brain–behavior relationships, and has contributed to the knowledge of the neural basis of language functioning. However, there are many open questions remaining. For instance, classification into three variants (non-fluent, semantic, and logopenic) is under debate; further data about epidemiology and natural history of the diseases are needed; and, as in other neurodegenerative disorders, successful therapies are lacking. 

For this Special Issue, we invite scholars to submit original research studies and reviews regarding all possible aspects of primary progressive aphasia, from basic science, epidemiology, clinical findings, longitudinal course, language and speech features, validation of neuropsychological tools for diagnosis, neuroimaging, blood and cerebrospinal fluid biomarkers, pathology, and treatments. A special focus should be placed on topics that can advance our assessment, differential diagnosis, and treatment of PPAs. 

Dr. Jordi A. Matias-Guiu
Dr. Robert Jr Laforce
Dr. Rene L. Utianski
Guest Editors

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Keywords

  • Primary progressive aphasia (PPA)
  • Frontotemporal dementia
  • Apraxia of speech
  • Alzheimer’s disease
  • Neuropsychology
  • Neuroimaging

Published Papers (12 papers)

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Editorial

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4 pages, 193 KiB  
Editorial
Advances in Primary Progressive Aphasia
by Jordi A. Matias-Guiu, Robert Laforce, Jr., Monica Lavoie and Rene L. Utianski
Brain Sci. 2022, 12(5), 636; https://doi.org/10.3390/brainsci12050636 - 12 May 2022
Viewed by 1753
Abstract
Primary progressive aphasia (PPA) is a neurodegenerative syndrome characterized by progressive and predominant language impairment [...] Full article
(This article belongs to the Special Issue Advances in Primary Progressive Aphasia)

Research

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14 pages, 1679 KiB  
Article
A Preliminary Report of Network Electroencephalographic Measures in Primary Progressive Apraxia of Speech and Aphasia
by Rene L. Utianski, Hugo Botha, John N. Caviness, Gregory A. Worrell, Joseph R. Duffy, Heather M. Clark, Jennifer L. Whitwell and Keith A. Josephs
Brain Sci. 2022, 12(3), 378; https://doi.org/10.3390/brainsci12030378 - 12 Mar 2022
Cited by 1 | Viewed by 1821
Abstract
The objective of this study was to characterize network-level changes in nonfluent/agrammatic Primary Progressive Aphasia (agPPA) and Primary Progressive Apraxia of Speech (PPAOS) with graph theory (GT) measures derived from scalp electroencephalography (EEG) recordings. EEGs of 15 agPPA and 7 PPAOS patients were [...] Read more.
The objective of this study was to characterize network-level changes in nonfluent/agrammatic Primary Progressive Aphasia (agPPA) and Primary Progressive Apraxia of Speech (PPAOS) with graph theory (GT) measures derived from scalp electroencephalography (EEG) recordings. EEGs of 15 agPPA and 7 PPAOS patients were collected during relaxed wakefulness with eyes closed (21 electrodes, 10–20 positions, 256 Hz sampling rate, 1–200 Hz bandpass filter). Eight artifact-free, non-overlapping 1024-point epochs were selected. Via Brainwave software, GT weighted connectivity and minimum spanning tree (MST) measures were calculated for theta and upper and lower alpha frequency bands. Differences in GT and MST measures between agPPA and PPAOS were assessed with Wilcoxon rank-sum tests. Of greatest interest, Spearman correlations were computed between behavioral and network measures in all frequency bands across all patients. There were no statistically significant differences in GT or MST measures between agPPA and PPAOS. There were significant correlations between several network and behavioral variables. The correlations demonstrate a relationship between reduced global efficiency and clinical symptom severity (e.g., parkinsonism, AOS). This preliminary, exploratory study demonstrates potential for EEG GT measures to quantify network changes associated with degenerative speech–language disorders. Full article
(This article belongs to the Special Issue Advances in Primary Progressive Aphasia)
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23 pages, 2526 KiB  
Article
Treatment for Anomia in Bilingual Speakers with Progressive Aphasia
by Stephanie M. Grasso, Elizabeth D. Peña, Nina Kazemi, Haideh Mirzapour, Rozen Neupane, Borna Bonakdarpour, Maria Luisa Gorno-Tempini and Maya L. Henry
Brain Sci. 2021, 11(11), 1371; https://doi.org/10.3390/brainsci11111371 - 20 Oct 2021
Cited by 9 | Viewed by 3776
Abstract
Anomia is an early and prominent feature of primary progressive aphasia (PPA) and other neurodegenerative disorders. Research investigating treatment for lexical retrieval impairment in individuals with progressive anomia has focused primarily on monolingual speakers, and treatment in bilingual speakers is relatively unexplored. In [...] Read more.
Anomia is an early and prominent feature of primary progressive aphasia (PPA) and other neurodegenerative disorders. Research investigating treatment for lexical retrieval impairment in individuals with progressive anomia has focused primarily on monolingual speakers, and treatment in bilingual speakers is relatively unexplored. In this series of single-case experiments, 10 bilingual speakers with progressive anomia received lexical retrieval treatment designed to engage relatively spared cognitive-linguistic abilities and promote word retrieval. Treatment was administered in two phases, with one language targeted per phase. Cross-linguistic cognates (e.g., rose and rosa) were included as treatment targets to investigate their potential to facilitate cross-linguistic transfer. Performance on trained and untrained stimuli was evaluated before, during, and after each phase of treatment, and at 3, 6, and 12 months post-treatment. Participants demonstrated a significant treatment effect in each of their treated languages, with maintenance up to one year post-treatment for the majority of participants. Most participants showed a significant cross-linguistic transfer effect for trained cognates in both the dominant and nondominant language, with fewer than half of participants showing a significant translation effect for noncognates. A gradual diminution of translation and generalization effects was observed during the follow-up period. Findings support the implementation of dual-language intervention approaches for bilingual speakers with progressive anomia, irrespective of language dominance. Full article
(This article belongs to the Special Issue Advances in Primary Progressive Aphasia)
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2 pages, 202 KiB  
Article
Cognitive Intervention Strategies Directed to Speech and Language Deficits in Primary Progressive Aphasia: Practice-Based Evidence from 18 Cases
by Thais Helena Machado, Maria Teresa Carthery-Goulart, Aline Carvalho Campanha and Paulo Caramelli
Brain Sci. 2021, 11(10), 1268; https://doi.org/10.3390/brainsci11101268 - 25 Sep 2021
Cited by 11 | Viewed by 2290 | Correction
Abstract
Background: Practice-based evidence can inform and support clinical decision making. Case-report series about the implementation of programs in real-world clinical settings may contribute to verifying the effectiveness of interventions for treating PPA in specific contexts, as well as illustrating challenges that need to [...] Read more.
Background: Practice-based evidence can inform and support clinical decision making. Case-report series about the implementation of programs in real-world clinical settings may contribute to verifying the effectiveness of interventions for treating PPA in specific contexts, as well as illustrating challenges that need to be overcome. Objective: To describe and provide practice-based evidence on the effectiveness of four cognitive rehabilitation programs designed for individuals with PPA and directed to speech and language impairments, which were implemented in a specialized outpatient clinic. Methods: Multiple single-case study. Eighteen individuals with different subtypes of PPA were each assigned to one out of four training programs based on comprehensive speech and language assessments. The treatments targeted naming deficits, sentence production, speech apraxia, and phonological deficits. Pre- and post-treatment assessments were undertaken to compare trained and untrained items. Gains were generalized to a different task in the first two types of intervention (naming and sentence production). A follow-up assessment was conducted 1–8 months after treatment among 7 participants. Results: All individuals presented better performance in the trained items at the post-test for each rehabilitation program accomplished, demonstrating that learning of the trained strategies was achieved during the active phase of treatment. For 13 individuals, statistical significance was reached; while for five, the results were maintained. Results about untrained items, generalization to other tasks, and follow-up assessments are presented. Conclusions: The positive results found in our sample bring some practice-based evidence for the benefits of speech and language treatment strategies for clinical management of individuals with PPA. Full article
(This article belongs to the Special Issue Advances in Primary Progressive Aphasia)
18 pages, 5144 KiB  
Article
Application of Machine Learning to Electroencephalography for the Diagnosis of Primary Progressive Aphasia: A Pilot Study
by Carlos Moral-Rubio, Paloma Balugo, Adela Fraile-Pereda, Vanesa Pytel, Lucía Fernández-Romero, Cristina Delgado-Alonso, Alfonso Delgado-Álvarez, Jorge Matias-Guiu, Jordi A. Matias-Guiu and José Luis Ayala
Brain Sci. 2021, 11(10), 1262; https://doi.org/10.3390/brainsci11101262 - 24 Sep 2021
Cited by 10 | Viewed by 2985
Abstract
Background. Primary progressive aphasia (PPA) is a neurodegenerative syndrome in which diagnosis is usually challenging. Biomarkers are needed for diagnosis and monitoring. In this study, we aimed to evaluate Electroencephalography (EEG) as a biomarker for the diagnosis of PPA. Methods. We [...] Read more.
Background. Primary progressive aphasia (PPA) is a neurodegenerative syndrome in which diagnosis is usually challenging. Biomarkers are needed for diagnosis and monitoring. In this study, we aimed to evaluate Electroencephalography (EEG) as a biomarker for the diagnosis of PPA. Methods. We conducted a cross-sectional study with 40 PPA patients categorized as non-fluent, semantic, and logopenic variants, and 20 controls. Resting-state EEG with 32 channels was acquired and preprocessed using several procedures (quantitative EEG, wavelet transformation, autoencoders, and graph theory analysis). Seven machine learning algorithms were evaluated (Decision Tree, Elastic Net, Support Vector Machines, Random Forest, K-Nearest Neighbors, Gaussian Naive Bayes, and Multinomial Naive Bayes). Results. Diagnostic capacity to distinguish between PPA and controls was high (accuracy 75%, F1-score 83% for kNN algorithm). The most important features in the classification were derived from network analysis based on graph theory. Conversely, discrimination between PPA variants was lower (Accuracy 58% and F1-score 60% for kNN). Conclusions. The application of ML to resting-state EEG may have a role in the diagnosis of PPA, especially in the differentiation from controls. Future studies with high-density EEG should explore the capacity to distinguish between PPA variants. Full article
(This article belongs to the Special Issue Advances in Primary Progressive Aphasia)
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15 pages, 1335 KiB  
Article
Primary Progressive Aphasia: Use of Graphical Markers for an Early and Differential Diagnosis
by Alexandra Plonka, Aurélie Mouton, Joël Macoir, Thi-Mai Tran, Alexandre Derremaux, Philippe Robert, Valeria Manera and Auriane Gros
Brain Sci. 2021, 11(9), 1198; https://doi.org/10.3390/brainsci11091198 - 11 Sep 2021
Cited by 7 | Viewed by 2287
Abstract
Primary progressive aphasia (PPA) brings together neurodegenerative pathologies whose main characteristic is to start with a progressive language disorder. PPA diagnosis is often delayed in non-specialised clinical settings. With the technologies’ development, new writing parameters can be extracted, such as the writing pressure [...] Read more.
Primary progressive aphasia (PPA) brings together neurodegenerative pathologies whose main characteristic is to start with a progressive language disorder. PPA diagnosis is often delayed in non-specialised clinical settings. With the technologies’ development, new writing parameters can be extracted, such as the writing pressure on a touch pad. Despite some studies having highlighted differences between patients with typical Alzheimer’s disease (AD) and healthy controls, writing parameters in PPAs are understudied. The objective was to verify if the writing pressure in different linguistic and non-linguistic tasks can differentiate patients with PPA from patients with AD and healthy subjects. Patients with PPA (n = 32), patients with AD (n = 22) and healthy controls (n = 26) were included in this study. They performed a set of handwriting tasks on an iPad® digital tablet, including linguistic, cognitive non-linguistic, and non-cognitive non-linguistic tasks. Average and maximum writing pressures were extracted for each task. We found significant differences in writing pressure, between healthy controls and patients with PPA, and between patients with PPA and AD. However, the classification of performances was dependent on the nature of the tasks. These results suggest that measuring writing pressure in graphical tasks may improve the early diagnosis of PPA, and the differential diagnosis between PPA and AD. Full article
(This article belongs to the Special Issue Advances in Primary Progressive Aphasia)
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10 pages, 1568 KiB  
Article
Survival in the Three Common Variants of Primary Progressive Aphasia: A Retrospective Study in a Tertiary Memory Clinic
by Maud Tastevin, Monica Lavoie, Justine de la Sablonnière, Julie Carrier-Auclair and Robert Laforce, Jr.
Brain Sci. 2021, 11(9), 1113; https://doi.org/10.3390/brainsci11091113 - 24 Aug 2021
Cited by 9 | Viewed by 2357
Abstract
Knowledge on the natural history of the three main variants of primary progressive aphasia (PPA) is lacking, particularly regarding mortality. Moreover, advanced stages and end of life issues are rarely discussed with caregivers and families at diagnosis, which can cause more psychological distress. [...] Read more.
Knowledge on the natural history of the three main variants of primary progressive aphasia (PPA) is lacking, particularly regarding mortality. Moreover, advanced stages and end of life issues are rarely discussed with caregivers and families at diagnosis, which can cause more psychological distress. We analyzed data from 83 deceased patients with a diagnosis of PPA. We studied survival in patients with a diagnosis of logopenic variant (lvPPA), semantic variant (svPPA), or non-fluent variant (nfvPPA) and examined causes of death. From medical records, we retrospectively collected data for each patient at several time points spanning five years before the first visit to death. When possible, interviews were performed with proxies of patients to complete missing data. Results showed that survival from symptom onset and diagnosis was significantly longer in svPPA than in lvPPA (p = 0.002) and nfvPPA (p < 0.001). No relevant confounders were associated with survival. Mean survival from symptom onset was 7.6 years for lvPPA, 7.1 years for nfvPPA, and 12 years for svPPA. The most common causes of death were natural cardio-pulmonary arrest and pneumonia. Aspiration pneumonia represented 23% of deaths in nfvPPA. In conclusion, this pilot study found significant differences in survival between the three variants of PPA with svPPA showing the longest and nfvPPA showing more neurologically-related causes of death. Full article
(This article belongs to the Special Issue Advances in Primary Progressive Aphasia)
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23 pages, 3717 KiB  
Article
Verbal Short-Term Memory Disturbance in the Primary Progressive Aphasias: Challenges and Distinctions in a Clinical Setting
by David Foxe, Sau Chi Cheung, Nicholas J. Cordato, James R. Burrell, Rebekah M. Ahmed, Cathleen Taylor-Rubin, Muireann Irish and Olivier Piguet
Brain Sci. 2021, 11(8), 1060; https://doi.org/10.3390/brainsci11081060 - 12 Aug 2021
Cited by 10 | Viewed by 2779
Abstract
Impaired verbal ‘phonological’ short-term memory is considered a cardinal feature of the logopenic variant of primary progressive aphasia (lv-PPA) and is assumed to underpin most of the language deficits in this syndrome. Clinically, examination of verbal short-term memory in individuals presenting with PPA [...] Read more.
Impaired verbal ‘phonological’ short-term memory is considered a cardinal feature of the logopenic variant of primary progressive aphasia (lv-PPA) and is assumed to underpin most of the language deficits in this syndrome. Clinically, examination of verbal short-term memory in individuals presenting with PPA is common practice and serves two objectives: (i) to help understand the possible mechanisms underlying the patient’s language profile and (ii) to help differentiate lv-PPA from other PPA variants or from other dementia syndromes. Distinction between lv-PPA and the non-fluent variant of PPA (nfv-PPA), however, can be especially challenging due to overlapping language profiles and comparable psychometric performances on verbal short-term memory tests. Here, we present case vignettes of the three PPA variants (lv-PPA, nfv-PPA, and the semantic variant (sv-PPA)) and typical Alzheimer’s disease (AD). These vignettes provide a detailed description of the short-term and working memory profiles typically found in these patients and highlight how speech output and language comprehension deficits across the PPA variants differentially interfere with verbal memory performance. We demonstrate that a combination of verbal short-term and working memory measures provides crucial information regarding the cognitive mechanisms underlying language disturbances in PPA. In addition, we propose that analogous visuospatial span tasks are essential for the assessment of PPA as they measure memory capacity without language contamination. Full article
(This article belongs to the Special Issue Advances in Primary Progressive Aphasia)
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18 pages, 1878 KiB  
Article
Breakdowns in Informativeness of Naturalistic Speech Production in Primary Progressive Aphasia
by Jeanne Gallée, Claire Cordella, Evelina Fedorenko, Daisy Hochberg, Alexandra Touroutoglou, Megan Quimby and Bradford C. Dickerson
Brain Sci. 2021, 11(2), 130; https://doi.org/10.3390/brainsci11020130 - 20 Jan 2021
Cited by 8 | Viewed by 3193
Abstract
“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 [...] Read more.
“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. Full article
(This article belongs to the Special Issue Advances in Primary Progressive Aphasia)
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Review

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18 pages, 1154 KiB  
Review
Semantic Variant Primary Progressive Aphasia: Practical Recommendations for Treatment from 20 Years of Behavioural Research
by Aida Suárez-González, Sharon A. Savage, Nathalie Bier, Maya L. Henry, Regina Jokel, Lyndsey Nickels and Cathleen Taylor-Rubin
Brain Sci. 2021, 11(12), 1552; https://doi.org/10.3390/brainsci11121552 - 23 Nov 2021
Cited by 8 | Viewed by 6260
Abstract
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 [...] Read more.
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. Full article
(This article belongs to the Special Issue Advances in Primary Progressive Aphasia)
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19 pages, 1185 KiB  
Review
Longitudinal Changes in Cognition, Behaviours, and Functional Abilities in the Three Main Variants of Primary Progressive Aphasia: A Literature Review
by Justine de la Sablonnière, Maud Tastevin, Monica Lavoie and Robert Laforce, Jr.
Brain Sci. 2021, 11(9), 1209; https://doi.org/10.3390/brainsci11091209 - 14 Sep 2021
Cited by 11 | Viewed by 2788
Abstract
Primary progressive aphasias (PPAs) are a group of neurodegenerative diseases presenting with insidious and relentless language impairment. Three main PPA variants have been described: the non-fluent/agrammatic variant (nfvPPA), the semantic variant (svPPA), and the logopenic variant (lvPPA). At the time of diagnosis, patients [...] Read more.
Primary progressive aphasias (PPAs) are a group of neurodegenerative diseases presenting with insidious and relentless language impairment. Three main PPA variants have been described: the non-fluent/agrammatic variant (nfvPPA), the semantic variant (svPPA), and the logopenic variant (lvPPA). At the time of diagnosis, patients and their families’ main question pertains to prognosis and evolution, but very few data exist to support clinicians’ claims. The objective of this study was to review the current literature on the longitudinal changes in cognition, behaviours, and functional abilities in the three main PPA variants. A comprehensive review was undertaken via a search on PUBMED and EMBASE. Two authors independently reviewed a total of 65 full-text records for eligibility. A total of 14 group studies and one meta-analysis were included. Among these, eight studies included all three PPA variants. Eight studies were prospective, and the follow-up duration was between one and five years. Overall, svPPA patients showed more behavioural disturbances both at baseline and over the course of the disease. Patients with lvPPA showed a worse cognitive decline, especially in episodic memory, and faster progression to dementia. Finally, patients with nfvPPA showed the most significant losses in language production and functional abilities. Data regarding the prodromal and last stages of PPA are still missing and studies with a longer follow-up observation period are needed. Full article
(This article belongs to the Special Issue Advances in Primary Progressive Aphasia)
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12 pages, 298 KiB  
Review
Contribution of the Cognitive Approach to Language Assessment to the Differential Diagnosis of Primary Progressive Aphasia
by Joël Macoir, Annie Légaré and Monica Lavoie
Brain Sci. 2021, 11(6), 815; https://doi.org/10.3390/brainsci11060815 - 19 Jun 2021
Cited by 8 | Viewed by 2933
Abstract
Diagnosis of primary progressive aphasia (PPA) is essentially based on the identification of progressive impairment of language abilities while other cognitive functions are preserved. The three variants of PPA are characterized by core and supportive clinical features related to the presence or absence [...] Read more.
Diagnosis of primary progressive aphasia (PPA) is essentially based on the identification of progressive impairment of language abilities while other cognitive functions are preserved. The three variants of PPA are characterized by core and supportive clinical features related to the presence or absence of language impairment in different linguistic domains. In this article, we review the cognitive neuropsychological approach to the assessment of PPA and its contribution to the differential diagnosis of the three variants. The main advantage of this assessment approach is that it goes beyond the mere description and classification of clinical syndromes and identifies impaired and preserved cognitive and linguistic components and processes. The article is structured according to the main language domains: spoken production, language comprehension, and written language. Each section includes a brief description of the cognitive processes involved in the assessment tasks, followed by a discussion of typical characteristics for each PPA variant and common pitfalls in the interpretation of the results. In addition, the clinical benefit of the cognitive neuropsychological approach for the behavioral management of PPA is briefly sketched out in the conclusion. Full article
(This article belongs to the Special Issue Advances in Primary Progressive Aphasia)
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