Primary Progressive Aphasia: What Happens to Speech and Language? What Can We Do to Help?

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

Deadline for manuscript submissions: 15 July 2026 | Viewed by 9186

Special Issue Editors


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Fondation de la Famille Lemaire, CHU de Québec—Université Laval, Québec, QC G1V 0A6, Canada
Interests: primary progressive aphasia; dementia; speech and language therapy

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Guest Editor
Division of Psychology and Language Sciences, University College London (UCL), London WC1H 0AP, UK
Interests: primary progressive aphasia; dementia; speech and language therapy

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Guest Editor
Dementia Research Centre, Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, University College London, London WC1E 6BT, UK
Interests: primary progressive aphasia; dementia; neurology

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Guest Editor
Istituti Clinici Scientifici Maugeri IRCCS, Laboratory of Neuropsychology, Institute of Bari, 70010 Bari, Italy
Interests: primary progressive aphasia; speech & language therapy; cognitive impairment; dementia; artificial intelligence
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Special Issue Information

Dear Colleagues,

Primary progressive aphasia is a neurocognitive disorder characterized by a progressive and predominant decline of language. Our knowledge of PPA has evolved rapidly in recent years, both in terms of our understanding of the condition and treatment as well as support options.

For this Special Issue, we invite researchers to submit manuscripts describing original research and reviews of cutting-edge research on primary progressive aphasia. The aim and scope of this Special Issue will include research on the underlying mechanisms of core symptoms of PPA and research on promising approaches for the management and treatment of PPA, including behavioral, neurostimulation, and pharmacological treatments. We also wish to highlight the importance of interdisciplinary work and the involvement of people with lived experience throughout this Special Issue.

Dr. Monica Lavoie
Dr. Anna Volkmer
Prof. Dr. Jason D. Warren
Dr. Petronilla Battista
Guest Editors

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Keywords

  • primary progressive aphasia
  • language
  • brain
  • communication
  • cognition

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Published Papers (8 papers)

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Research

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19 pages, 1669 KB  
Article
‘I Am No Longer Anxious When I Speak’: Experiences of People with Primary Progressive Aphasia Taking Part in a Biographic-Narrative Therapy (Cope PPA)
by Mirjam Gauch, Anna-Lena Köb, Julia Tanase, Julia Feldmann, Johanna Jochmann, Katharina Geschke, Helen Klaus, Oliver Tüscher, Isabel Heinrich and Sabine Corsten
Brain Sci. 2026, 16(2), 233; https://doi.org/10.3390/brainsci16020233 - 16 Feb 2026
Cited by 1 | Viewed by 892
Abstract
Background: Due to communication problems, people with primary progressive aphasia (PwPPA) are often affected in their self-image and experience a reduced quality of life (QoL). Biographic-narrative therapy is an effective approach to improve QoL in post-stroke aphasia. This study describes how PwPPA experienced [...] Read more.
Background: Due to communication problems, people with primary progressive aphasia (PwPPA) are often affected in their self-image and experience a reduced quality of life (QoL). Biographic-narrative therapy is an effective approach to improve QoL in post-stroke aphasia. This study describes how PwPPA experienced their participation in the biographic-narrative intervention called Cope PPA. Methods: The intervention comprised a combination of five individual and seven group therapy sessions as well as the use of music and art therapy elements. Inclusion criteria were a capacity to give consent and sufficient visual/auditory abilities of PwPPA. Exclusion criteria were the presence of severe depression (MADRS > 35) or severe cognitive deficits (MMST < 10). After the therapy, PwPPA and their family members took part in half-hour semi-structured interviews. Interviews were analysed according to the reflexive thematic analysis by Braun and Clarke. Results: The qualitative analysis was based on a data set of 34 interviews. A total of six themes were identified: (1) Participation required adherence; (2) Materials were considered remarkable; (3) Storytelling was conducted in an aphasia-free area; (4) Group participation created a sense of belonging; (5) Experiences encouraged self-reflection and (6) Coping is lengthy and ongoing. Conclusions: The findings of our reflexive thematic analysis suggest that PwPPA experienced the intervention as meaningful. Some PwPPA described the effects of our intervention on their self-image. Others emphasised that coping with their condition was an ongoing process requiring continuous support. Full article
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13 pages, 857 KB  
Article
Neurostimulation with Naming Therapy for Primary Progressive Aphasia: A Pilot Study Targeting Transcranial Direct Current (tDCS) Stimulation for the Individual
by Christopher Bernard Leahy, Jennifer C. Thompson, Matthew Jones and Anna Woollams
Brain Sci. 2026, 16(2), 128; https://doi.org/10.3390/brainsci16020128 - 25 Jan 2026
Viewed by 688
Abstract
Background: Transcranial Direct Current Stimulation (tDCS) in conjunction with behavioural language therapy in PPA has previously been modified for variation at the group level, but not at the individual level. This pilot study used individualised tDCS targeting by identifying regions of peak [...] Read more.
Background: Transcranial Direct Current Stimulation (tDCS) in conjunction with behavioural language therapy in PPA has previously been modified for variation at the group level, but not at the individual level. This pilot study used individualised tDCS targeting by identifying regions of peak atrophy in the language system. Methods: Six PPA participants (four semantic and two non-fluent variant) were randomly allocated to receive tDCS or sham stimulation. The target electrode was selected for each based on their region of peak atrophy. Participants received naming therapy, individually calibrated according to baseline naming performance. Three sets of therapy were delivered in conjunction with tDCS (1 mA) or sham stimulation within participants’ homes. The study was not powered to demonstrate efficacy but to show proof-of-concept for an individualised, home-based tDCS targeting method. Results: All participants successfully completed the protocol. In one participant the region of peak atrophy differed from that predicted by clinical syndrome. Significant gains were observed at an individual level for treated items in both groups (2/3 tDCS and 2/3 Sham). No significant changes in untreated items were observed at an individual level. Significant naming improvement in untreated items was not observed for the tDCS group and was seen at one time point only for the Sham group. Conclusions: We have demonstrated the feasibility of a novel method for selecting neurostimulation targets for PPA at the individual level. A larger study would be required to determine the long-term therapeutic efficacy of this method. Full article
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25 pages, 1909 KB  
Article
Exploring Language Impairment in Catalan-Dominant Bilinguals with Primary Progressive Aphasia: Preliminary Data
by Io Salmons and Helena Muntané-Sánchez
Brain Sci. 2025, 15(11), 1193; https://doi.org/10.3390/brainsci15111193 - 4 Nov 2025
Cited by 1 | Viewed by 796
Abstract
Background: Research on primary progressive aphasia (PPA) in minority languages and bilingual speakers remains limited, which can compromise accurate diagnosis and intervention. This is the case for Catalan, which lacks standardized tools for this population. Objectives: This study aimed to evaluate the Catalan [...] Read more.
Background: Research on primary progressive aphasia (PPA) in minority languages and bilingual speakers remains limited, which can compromise accurate diagnosis and intervention. This is the case for Catalan, which lacks standardized tools for this population. Objectives: This study aimed to evaluate the Catalan version of the Comprehensive Aphasia Test (CAT-CAT) for detecting and characterizing PPA in Catalan-dominant bilingual individuals. Methods: We administered the CAT-CAT to four participants clinically diagnosed with PPA. Results: The test detected participants’ core linguistic impairments, such as anomia, and revealed distinct severity profiles consistent with PPA variant types. Moreover, it captured deficits that were not identified by routine clinical observation or informal assessment. Conclusions: This study provides the first detailed characterization of PPA in Catalan-speaking individuals and, although based on a small sample, its findings address a critical gap in neurodegenerative language research and highlight the importance of standardized tools to improve diagnosis and guide clinical interventions in bilingual speakers of minority languages. Full article
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11 pages, 851 KB  
Article
Distinguishing Among Variants of Primary Progressive Aphasia with a Brief Multimodal Test of Nouns and Verbs
by Marco A. Lambert, Melissa D. Stockbridge, Lindsey Kelly, Isidora Diaz-Carr, Voss Neal and Argye E. Hillis
Brain Sci. 2025, 15(10), 1108; https://doi.org/10.3390/brainsci15101108 - 15 Oct 2025
Viewed by 1264
Abstract
Background: Primary Progressive Aphasia (PPA) variants include the non-fluent agrammatic (nfvPPA), logopenic (lvPPA), and semantic (svPPA), which differ in their effects on speech production. However, their impact on modality (oral vs. written) and grammatical word class (nouns vs. verbs) remains controversial. A significant [...] Read more.
Background: Primary Progressive Aphasia (PPA) variants include the non-fluent agrammatic (nfvPPA), logopenic (lvPPA), and semantic (svPPA), which differ in their effects on speech production. However, their impact on modality (oral vs. written) and grammatical word class (nouns vs. verbs) remains controversial. A significant effect of these variables might assist in classification. Materials and Methods: This study used first-visit data from 300 participants with PPA who completed oral and written noun and verb naming (matched in surface word frequency across word class) to test the hypothesis that the three variants show differential impairment on word class or modality. Group differences were evaluated with rank-transformed repeated measures ANOVA. Within individual differences between nouns and verbs and between oral and written modalities were tested with Fisher’s exact tests. Results: A significant modality × variant interaction (p = 0.017) was observed. Participants with lvPPA and nfvPPA demonstrated greater oral than written naming, with nfvPPA also performing better on nouns than verbs. Those with svPPA showed no modality or word class effects but had an overall low accuracy. Three participants with svPPA (but no individuals with the other variants) demonstrated significantly (p = 0.003) more accurate verb than noun naming. Conclusions: Differing modality and word class patterns characterize PPA variants, with nfvPPA more accurate in nouns than verbs on average. Within individuals, only those with svPPA occasionally showed significantly more proficient verb than noun naming. Grammatical word class effects likely arise at distinct levels of cognitive processing underlying naming. Full article
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26 pages, 1276 KB  
Article
Adaptation of Better Conversations with Primary Progressive Aphasia to Norwegian
by Ingvild Winsnes, Monica Norvik and Anna Volkmer
Brain Sci. 2025, 15(9), 994; https://doi.org/10.3390/brainsci15090994 - 15 Sep 2025
Cited by 1 | Viewed by 1341
Abstract
Background/Objectives: People with primary progressive aphasia (PPA) and their communication partners report that having conversations becomes more difficult over time. They want speech and language therapy to help them have better conversations. Communication partner training has shown promise as an approach for people [...] Read more.
Background/Objectives: People with primary progressive aphasia (PPA) and their communication partners report that having conversations becomes more difficult over time. They want speech and language therapy to help them have better conversations. Communication partner training has shown promise as an approach for people with PPA and their communication partners. However, there are currently no communication partner training programs available in Norwegian for people with PPA. The Better Conversations with Primary Progressive Aphasia (BCPPA) is a communication partner training program developed in the UK. In this study, we aimed to culturally adapt the BCPPA to meet the needs of Norwegian people with PPA. Methods: Guided by adaptation elements identified in a systematic review of frameworks for cultural adaptation, we translated the BCPPA into Norwegian before piloting it with four participant dyads, comprising people with PPA and their communication partners. The translated BCPPA was compared to the original BCPPA to identify adherence with core intervention components. Semi-structured interviews were used to explore the acceptability of the intervention to participant dyads. Outcome data, including Goal Attainment Scaling, coding of conversation behaviours from video samples, the Aphasia Impact Questionnaire, and the Communicative Effectiveness Index, were recorded pre-, post-, and three months after intervention delivery to explore outcomes for Norwegian participant dyads. We used the Framework for Reporting Adaptations and Modifications-Enhanced to document the modifications. Results: The results indicate high adherence to the core components in the original BCPPA. The pilot demonstrated that the participant dyads found the BCPPA acceptable, but they made some additional suggestions to complete the cultural adaptation further. Despite the progressive nature of PPA, the participant dyads achieved their goals on the Goal Attainment Scaling, and group analysis demonstrated maintenance on the Aphasia Impact Questionnaire and the Communicative Effectiveness Index over the three time points. Conclusions: This study demonstrates that the Norwegian version of the BCPPA was acceptable to the participants with PPA and their communication partner in this study. As the first communication partner training program for people with PPA and their communication partners in Norwegian, the BCPPA has the potential to be a valuable treatment tool to support people affected by PPA to have better conversations. Full article
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Review

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15 pages, 1065 KB  
Review
Unifying Divergent Conceptions in Nonfluent/Agrammatic and Semantic Primary Progressive Aphasia
by Marc Teichmann and Kimihiro Nakamura
Brain Sci. 2026, 16(5), 509; https://doi.org/10.3390/brainsci16050509 (registering DOI) - 9 May 2026
Viewed by 179
Abstract
The nonfluent/agrammatic variant of primary progressive aphasia (nfav-PPA) and primary progressive apraxia of speech (PPAOS) are neurodegenerative syndromes that raise diagnostic challenges related to several issues. First, there are two divergent conceptions, one stipulating that (i) nfav-APP and PPAOS are distinct entities, and [...] Read more.
The nonfluent/agrammatic variant of primary progressive aphasia (nfav-PPA) and primary progressive apraxia of speech (PPAOS) are neurodegenerative syndromes that raise diagnostic challenges related to several issues. First, there are two divergent conceptions, one stipulating that (i) nfav-APP and PPAOS are distinct entities, and the other (ii) that PPAOS has to be integrated into the nfav-APP spectrum. A second related issue concerns the consideration of phonological dimensions, lying at the language interface with speech, which could potentially help overcome the nfva-PPA/PPAOS controversy. Third, there is a lack of internationally validated clinical tests assessing apraxia of speech and syntactic abilities with sufficient specificity and sensitivity. This narrative review discusses these issues taking into account clinical, neurocognitive and neurobiological dimensions. It proposes a conceptual-integrative framework conciliating competing nfav-APP/PPAOS accounts while suggesting a graded continuum with subdivisions, related to neurodegenerative expansion throughout language/speech production systems, ranging from syntactic to phonological to phonetic-articulatory impairments. A second controversy in the field of PPA arises from divergent conceptions of semantic PPA (sv-PPA), defined by primary damage to verbal semantics, and of semantic dementia (SD) characterized by multimodal semantic impairments. The current consensus criteria of PPA have deconstructed the initial SD conception by absorbing it into sv-PPA, hence leaving mixed and some non-verbal semantic phenotypes nosologically orphaned. Again, the article proposes a conceptual and integrative model, built on findings from clinical research and cognitive neuroscience, suggesting a graded continuum with subdivisions spanning from verbal to different non-verbal semantic impairments including social-semantic/behavioral phenotypes. Full article
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19 pages, 745 KB  
Review
Two Languages and One Aphasia: A Systematic Scoping Review of Primary Progressive Aphasia in Chinese Bilingual Speakers, and Implications for Diagnosis and Clinical Care
by Weifeng Han, Lin Zhou, Juan Lu and Shane Pill
Brain Sci. 2026, 16(1), 20; https://doi.org/10.3390/brainsci16010020 - 24 Dec 2025
Viewed by 981
Abstract
Background/Objectives: Primary progressive aphasia (PPA) is characterised by progressive decline in language and communication. However, existing diagnostic frameworks and assessment tools are largely based on Indo-European languages, which limits their applicability to Chinese bilingual speakers whose linguistic profiles differ markedly in tonal [...] Read more.
Background/Objectives: Primary progressive aphasia (PPA) is characterised by progressive decline in language and communication. However, existing diagnostic frameworks and assessment tools are largely based on Indo-European languages, which limits their applicability to Chinese bilingual speakers whose linguistic profiles differ markedly in tonal phonology, logographic writing, and bilingual organisation. This review aimed to (a) describe how PPA presents in Chinese bilingual speakers, (b) evaluate how well current speech–language and neuropsychological assessments capture these impairments, and (c) identify linguistically and culturally informed strategies to improve clinical practice. Methods: A systematic review was conducted in accordance with the PRISMA-ScR guidelines. Four databases (PubMed, Scopus, Web of Science, PsycINFO) were searched, complemented by backward and forward citation chaining. Eight empirical studies met the inclusion criteria. Data were extracted on participant characteristics, PPA variant, language background, speech–language and writing profiles, and assessment tools used. Thematic analysis was applied to address the research questions. Results: Across variants, Chinese bilingual speakers demonstrated universal PPA features expressed through language-specific pathways. Mandarin speakers exhibited tone-segment integration errors, tonal substitution, and disruptions in logographic writing. Lexical-semantic degradation reflected homophony and compounding characteristics. Bilingual individuals showed parallel or asymmetric decline influenced by dominance and usage. Standard English-based naming, repetition, and writing assessments did not reliably capture tone accuracy, radical-level writing errors, or bilingual patterns. Sociocultural factors, including stigma, delayed help-seeking, and family-centred care expectations, further shaped diagnostic pathways. Conclusions: Chinese PPA cannot be meaningfully assessed using tools designed for Indo-European languages. Findings highlight the need for tone-sensitive repetition tasks, logographic writing assessments, bilingual diagnostic protocols, and culturally responsive communication-partner support. This review provides a comprehensive synthesis to date on Chinese bilingual PPA and establishes a foundation for linguistically inclusive diagnostic and clinical models. Full article
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Other

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18 pages, 638 KB  
Case Report
Feasibility of Home-Based Transcranial Direct Current Stimulation with Telerehabilitation in Primary Progressive Aphasia—A Case Series
by Anna Uta Rysop, Tanja Grewe, Caterina Breitenstein, Ferdinand Binkofski, Mandy Roheger, Nina Unger, Agnes Flöel and Marcus Meinzer
Brain Sci. 2025, 15(7), 742; https://doi.org/10.3390/brainsci15070742 - 10 Jul 2025
Cited by 1 | Viewed by 1839
Abstract
Background: Primary progressive aphasia (PPA) is a neurodegenerative disease characterised by progressive impairment of speech and language abilities. Intensive speech and language teletherapy combined with remotely supervised, self-administered transcranial direct current stimulation (tDCS) may be suited to remove barriers to accessing potentially effective [...] Read more.
Background: Primary progressive aphasia (PPA) is a neurodegenerative disease characterised by progressive impairment of speech and language abilities. Intensive speech and language teletherapy combined with remotely supervised, self-administered transcranial direct current stimulation (tDCS) may be suited to remove barriers to accessing potentially effective treatments, but there is only limited evidence on the feasibility of this combined approach. Methods: This pilot case series investigated the feasibility, tolerability and preliminary efficacy of a novel telerehabilitation programme combined with home-based, self-administered tDCS for people with primary progressive aphasia (pwPPA). The intervention programme was co-developed with pwPPA and their caregivers, to reflect their priorities regarding treatment content and outcomes (i.e., naming, functional communication). Results: Two pwPPA successfully completed the telerehabilitation intervention with daily naming training and communicative-pragmatic therapy paired with tDCS, over 10 consecutive workdays. Caregivers assisted in the setup of equipment required for teletherapy and home-based tDCS. Participants successfully completed the programme with a 95% completion rate. Home-based tDCS was well tolerated. Both participants showed improvements in naming and communication, suggesting preliminary efficacy of the intervention. Conclusions: Overall, this study demonstrates the feasibility and potential benefit of a novel, easily accessible and patient-relevant telerehabilitation intervention for pwPPA, which requires confirmation in a future larger-scale exploratory trial. Full article
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