Predictors of Care Home Admission and Survival Rate in Patients With Syndromes Associated With Frontotemporal Lobar Degeneration in Europe.

BACKGROUND AND OBJECTIVES: Data on care home admission and survival rates of patients with syndromes associated with frontotemporal lobar degeneration (FTLD) are limited. However, their estimation is essential to plan trials and assess the efficacy of intervention. Population-based registers provide unique samples for this estimate. The aim of this study was to assess care home admission rate, survival rate, and their predictors in incident patients with FTLD-associated syndromes from the European FRONTIERS register-based study. METHODS: We conducted a prospective longitudinal multinational observational registry study, considering incident patients with FTLD-associated syndromes diagnosed between June 1, 2018, and May 31, 2019, and followed for up to 5 years till May 31, 2023. We enrolled patients fulfilling diagnosis of the behavioral variant frontotemporal dementia (bvFTD), primary progressive aphasia (PPA), progressive supranuclear palsy (PSP) or corticobasal syndrome (CBS), and FTD with motor neuron disease (FTD-MND). Kaplan-Meier analysis and Cox multivariable regression models were used to assess care home admission and survival rates. The survival probability score (SPS) was computed based on independent predictors of survivorship. RESULTS: A total of 266 incident patients with FTLD were included (mean age ± SD = 66.7 ± 9.0; female = 41.4%). The median care home admission rate was 97 months (95% CIs 86-98) from disease onset and 57 months (95% CIs 56-58) from diagnosis. The median survival was 90 months (95% CIs 77-97) from disease onset and 49 months (95% CIs 44-58) from diagnosis. Survival from diagnosis was shorter in FTD-MND (hazard ratio [HR] 4.59, 95% CIs 2.49-8.76, p < 0.001) and PSP/CBS (HR 1.56, 95% CIs 1.01-2.42, p = 0.044) compared with bvFTD; no differences between PPA and bvFTD were found. The SPS proved high accuracy in predicting 1-year survival probability (area under the receiver operating characteristic curve = 0.789, 95% CIs 0.69-0.87), when defined by age, European area of residency, extrapyramidal symptoms, and MND at diagnosis. DISCUSSION: In FTLD-associated syndromes, survival rates differ according to clinical features and geography. The SPS was able to predict prognosis at individual patient level with an accuracy of ∼80% and may help to improve patient stratification in clinical trials. Future confirmatory studies considering different populations are needed.

Global perspectives on the management of primary progressive aphasia.

Speech-language therapists/pathologists (SLT/Ps) are key professionals in the management and treatment of primary progressive aphasia (PPA), however, there are gaps in education and training within the discipline, with implications for skills, confidence, and clinical decision-making. This survey aimed to explore the areas of need amongst SLT/Ps working with people living with PPA (PwPPA) internationally to upskill the current and future workforce working with progressive communication disorders. One hundred eighty-six SLT/Ps from 27 countries who work with PwPPA participated in an anonymous online survey about their educational and clinical experiences, clinical decision-making, and self-reported areas of need when working with this population. Best practice principles for SLT/Ps working with PwPPA were used to frame the latter two sections of this survey. Only 40.7% of respondents indicated that their university education prepared them for their current work with PwPPA. Competency areas of "knowing people deeply," "practical issues," "connectedness," and "preventing disasters" were identified as the basic areas of priority and need. Respondents identified instructional online courses (92.5%), sample tools and activities for interventions (64.8%), and concrete training on providing care for advanced stages and end of life (58.3%) as central areas of need in their current work. This is the first international survey to comprehensively explore the perspectives of SLT/Ps working with PwPPA. Based on survey outcomes, there is a pressing need to enhance current educational and ongoing training opportunities to better promote the well-being of PwPPA and their families, and to ensure appropriate preparation of the current and future SLT/P workforce.

A Critical Review of Noninvasive Brain Stimulation Technologies in Alzheimer's Dementia and Primary Progressive Aphasia.

Multiple pharmacologic agents now have been approved in the United States and other countries as treatment to slow disease and clinical progression for Alzheimer's disease. Given these treatments have not been proven to lessen the cognitive deficits already manifested in the Alzheimer's Clinical Syndrome (ACS), and none are aimed for another debilitating dementia syndrome identified as primary progressive aphasia (PPA), there is an urgent need for new, safe, tolerable, and efficacious treatments to mitigate the cognitive deficits experienced in ACS and PPA. Noninvasive brain stimulation has shown promise for enhancing cognitive functioning, and there has been interest in its potential therapeutic value in ACS and PPA. This review critically examines the evidence of five technologies in ACS and PPA: transcranial direct current stimulation (tDCS), transcranial alternating current stimulation (tACS), transcranial random noise stimulation (tRNS), repetitive transcranial magnetic stimulation (rTMS), and noninvasive vagus nerve stimulation (nVNS). Many randomized controlled trials of tDCS and rTMS report positive treatment effects on cognition in ACS and PPA that persist out to at least 8 weeks, whereas there are few trials for tACS and none for tRNS and nVNS. However, most positive trials did not identify clinically meaningful changes, underscoring that clinical efficacy has yet to be established in ACS and PPA. Much is still to be learned about noninvasive brain stimulation in ACS and PPA, and shifting the focus to prioritize clinical significance in addition to statistical significance in trials could yield greater success in understanding its potential cognitive effects and optimal parameters.

Applications of the R.A.I.S.E. Assessment Framework to Support the Process of Assessment in Primary Progressive Aphasia.

PURPOSE: To establish the extent to which person-centered processes are integrated in assessment procedures, the Relationship, Assessment, Inclusion, Support, Evolve (R.A.I.S.E.) Assessment framework was used to evaluate measures that are typically used when assessing people living with primary progressive aphasia (PPA). METHOD: Forty-five assessment tools were evaluated through the lens of the five R.A.I.S.E. principles: building the client-clinician Relationship, Assessment choices, Including the client and care partners, providing Support, and Evolving procedures to match client capability and progression. The principles were operationalized as questions for raters to evaluate whether a measure met this aspect of the R.A.I.S.E. Assessment framework. RESULTS: Ten measures commonly used in the assessment of people living with PPA met all R.A.I.S.E. principles. These measures centered upon the elicitation of naturalistic discourse, conversation, client self-report, and clinician ratings. Thirteen measures did not meet any of the criteria, and represented standardized evaluation procedures do not provide the opportunity to connect to the client, elicit or provide feedback or support, nor to adapt in response to need or performance. CONCLUSIONS: Whether using standardized or informal assessment tools, a relational and qualitative approach to providing assessment is paramount to promote client success and therapeutic engagement. We provide guidance through the R.A.I.S.E. framework on practices to cultivate person-centered processes of assessment in the care of people living with PPA.

Non-pharmacological interventions for improving language and communication in people with primary progressive aphasia.

BACKGROUND: Primary progressive aphasia (PPA) accounts for approximately 43% of frontotemporal dementias and is mainly characterised by a progressive impairment of speech and communication abilities. Three clinical variants have been identified: (a) non-fluent/agrammatic, (b) semantic, and (c) logopenic/phonological PPA variants. There is currently no curative treatment for PPA, and the disease progresses inexorably over time, with devastating effects on speech and communication ability, functional status, and quality of life. Several non-pharmacological interventions that may improve symptoms (e.g. different forms of language training and non-invasive brain stimulation) have been investigated in people with PPA. OBJECTIVES: To assess the effects of non-pharmacological interventions for people with PPA on word retrieval (our primary outcome), global language functions, cognition, quality of life, and adverse events. SEARCH METHODS: We searched the Cochrane Dementia and Cognitive Improvement Group's trial register, MEDLINE (Ovid SP), Embase (Ovid SP), four other databases and two other trial registers. The latest searches were run on 26 January 2024. SELECTION CRITERIA: We included randomised controlled trials (RCTs) evaluating the effects of non-pharmacological interventions in people with PPA. DATA COLLECTION AND ANALYSIS: We used standard methodological procedures expected by Cochrane. MAIN RESULTS: There were insufficient data available to conduct the network meta-analyses that we had originally planned (due to trial data being insufficiently reported or not reported at all, as well as the heterogeneous content of the included interventions). Therefore, we provide a descriptive summary of the included studies and results. We included 10 studies, with a total of 132 participants, evaluating non-pharmacological interventions. These were: transcranial direct current stimulation (tDCS) or repetitive transcranial magnetic stimulation (rTMS) as stand-alone treatments (used by two and one studies, respectively); tDCS combined with semantic and phonological word-retrieval training (five studies); tDCS combined with semantic word-retrieval training (one study); and tDCS combined with phonological word-retrieval training (one study). Results for our primary outcome of word retrieval were mixed. For the two studies that investigated the effects of tDCS as stand-alone treatment compared to placebo ("sham") tDCS, we rated the results as having very low-certainty evidence. One study found a significant beneficial effect on word retrieval after active tDCS; one study did not report any significant effects in favour of the active tDCS group. Five studies investigated tDCS administered to the dorsolateral prefrontal cortex, inferior frontal cortex, left frontotemporal region, or the temporoparietal cortex, combined with semantic and phonological word-retrieval training. The most consistent finding was enhancement of word-retrieval ability for trained items immediately after the intervention, when behavioural training was combined with active tDCS compared to behavioural training plus sham tDCS. We found mixed effects for untrained items and maintenance of treatment effects during follow-up assessments. We rated the certainty of the evidence as very low in all studies. One study investigated tDCS combined with semantic word-retrieval training. Training was provided across 15 sessions with a frequency of three to five sessions per week, depending on the personal preferences of the participants. tDCS targeted the left frontotemporal region. The study included three participants: two received 1 mA stimulation and one received 2 mA stimulation. The study showed mixed results. We rated it as very low-certainty evidence. One study investigated tDCS combined with phonological word-retrieval training. Training was again provided across 15 sessions over a period of three weeks. tDCS targeted the left inferior frontal gyrus. This study showed a significantly more pronounced improvement for trained and untrained words in favour of the group that had received active tDCS, but we rated the certainty of the evidence as very low. One study compared active rTMS applied to an individually determined target site to active rTMS applied to a control site (vertex) for effects on participants' word retrieval. This study demonstrated better word retrieval for active rTMS administered to individually determined target brain regions than in the control intervention, but we rated the results as having a very low certainty of evidence. Four studies assessed overall language ability, three studies assessed cognition, five studies assessed potential adverse effects of brain stimulation, and one study investigated quality of life. AUTHORS' CONCLUSIONS: There is currently no high-certainty evidence to inform clinical decision-making regarding non-pharmacological treatment selection for people with PPA. Preliminary evidence suggests that the combination of active tDCS with specific language therapy may improve impaired word retrieval for specifically trained items beyond the effects of behavioural treatment alone. However, more research is needed, including high-quality RCTs with detailed descriptions of participants and methods, and consideration of outcomes such as quality of life, depressive symptoms, and overall cognitive functioning. Moreover, studies assessing optimal treatments (i.e. behavioural interventions, brain stimulation interventions, and their combinations) for individual patients and PPA subtypes are needed. We were not able to conduct the planned (network) meta-analyses due to missing data that could not be obtained from most of the authors, a general lack of RCTs in the field, and heterogeneous interventions in eligible trials. Journals should implement a mandatory data-sharing requirement to assure transparency and accessibility of data from clinical trials.

COS-PPA: protocol to develop a core outcome set for primary progressive aphasia.

INTRODUCTION: The term primary progressive aphasia (PPA) describes a group of language-led dementias. Disease-modifying treatments that delay, slow or reverse progression of PPA are currently lacking, though a number of interventions to manage the symptoms of PPA have been developed in recent years. Unfortunately, studies exploring the effectiveness of these interventions have used a variety of different outcome measures, limiting comparability. There are more constructs, apart from word retrieval, that are important for people with PPA that have not received much attention in the research literature. Existing core outcome sets (COS) for dementia and non-progressive aphasia do not meet the needs of people with PPA, highlighting a need to develop a specific COS for PPA. METHODS AND ANALYSIS: This protocol describes a three-stage study to identify a COS for PPA interventions in research and clinical practice. The stage 1 systematic review will identify existing speech, language and communication measures used to examine the effectiveness of interventions for PPA in the research literature. Employing a nominal group technique, stage 2 will identify the most important outcomes for people with PPA and their families. The data collected in stages 1 and 2 will be jointly analysed with the project PPI group and will inform the stage 2 modified Delphi consensus study to identify a core outcome measurement set for PPA among a range of research disciplines undertaking intervention studies for people with PPA. ETHICS AND DISSEMINATION: Ethical approval for stage 2 of the study has been sought individually in each country at collaborating institutions and is stated in detail in the manuscript. Stage 3 has been granted ethical approval by the Chairs of UCL Language and Cognition Department Ethics, Project ID LCD-2023-06. Work undertaken at stages 1, 2 and 3 will be published in open-access peer-reviewed journal articles and presented at international scientific conferences. PROSPERO REGISTRATION NUMBER: CRD42022367565.

Computer-Based Naming Treatment for Semantic Variant Primary Progressive Aphasia With History of Traumatic Brain Injury: A Single-Case Experimental Design.

PURPOSE: The purpose of this study was to investigate the effectiveness of a self-administered naming treatment for one individual, B.N., presenting with semantic variant primary progressive aphasia (svPPA) and a history of traumatic brain injury (TBI). METHOD: Naming treatment included components of Lexical Retrieval Cascade Treatment and was self-administered using an adaptive spaced retrieval software, Anki. Using a multiple-baseline, single-case experimental design, naming accuracy probes were taken during pretreatment, treatment, posttreatment, and follow-up (through 12 months) for 60 trained words and 10 untrained words. Item-level Bayesian generalized mixed-effects models were used to estimate (a) the treatment effect for trained words, (b) change in untrained words, and (c) maintenance of treatment effects from posttreatment to each subsequent follow-up. RESULTS: Statistical analyses revealed that a gain of 35 out of 60 trained words (35.3; 90% CI [30.6, 39.5]) was directly attributable to treatment. Following treatment, evidence of generalization to untrained words was not observed. During the follow-up period, there was gradual decline in naming accuracy of trained items. CONCLUSIONS: The positive treatment results reported here support the use of self-administered naming treatments for those with svPPA and a history of TBI. Although the utility of this treatment approach is constrained by patient factors including motivation, self-administered naming treatments represent a unique opportunity to expand access to speech-language intervention for people with svPPA, including those with concomitant diagnoses. SUPPLEMENTAL MATERIAL: https://doi.org/10.23641/asha.25119080.

Primary progressive aphasia: six questions in search of an answer.

Here, we review recent progress in the diagnosis and management of primary progressive aphasia-the language-led dementias. We pose six key unanswered questions that challenge current assumptions and highlight the unresolved difficulties that surround these diseases. How many syndromes of primary progressive aphasia are there-and is syndromic diagnosis even useful? Are these truly 'language-led' dementias? How can we diagnose (and track) primary progressive aphasia better? Can brain pathology be predicted in these diseases? What is their core pathophysiology? In addition, how can primary progressive aphasia best be treated? We propose that pathophysiological mechanisms linking proteinopathies to phenotypes may help resolve the clinical complexity of primary progressive aphasia, and may suggest novel diagnostic tools and markers and guide the deployment of effective therapies.

Bilingual Primary Progressive Aphasia: A Scoping Review of Assessment and Treatment Practices.

BACKGROUND: Primary progressive aphasia (PPA) is a neurodegenerative syndrome characterized by speech and/or language impairment with relatively spared cognition. Research investigating behavioral speech-language intervention and methods for cognitive-linguistic assessment in PPA has predominantly centered around monolingual speakers. This gap hinders the widespread adoption of evidence-based approaches and exacerbates the inequities faced by culturally and linguistically diverse populations living with PPA. OBJECTIVE: This scoping review synthesizes the current evidence for assessment and treatment practices in bilingual PPA as well as the operationalization of bilingualism in PPA. METHODS: Arksey & O'Malley's scoping review methodology was utilized. Information was extracted from each study and entered into a data-charting template designed to capture information regarding operationalization of bilingualism in PPA and assessment and treatment practices. RESULTS: Of the 16 identified studies, 14 reported the results of assessments conducted in both languages. Three studies reported positive naming treatment outcomes. Thirteen studies included English-speaking participants, revealing linguistic bias. Most studies reported age of acquisition, proficiency, and patterns of language use rather than providing an operational definition for bilingualism. CONCLUSIONS: Neither formal assessment measures nor clear guidelines for assessment of bilingual PPA currently exist; however, language-specific measures are emerging. Speech-language intervention in bilingual PPA has been relatively unexplored, representing a significant gap in the literature. In order to improve diagnostic and treatment options for bilingual PPA, targeted efforts to increase representation of bilinguals from various sociocultural contexts, as well as those who speak a variety of language pairs, is necessary.

Differential approach to stroke aphasia and primary progressive aphasia using transcranial magnetic stimulation: A systematic review.

Language disorders can occur as a consequence of stroke or neurodegenerative disorders, among other causes. Post­stroke aphasia (PSA) and primary progressive aphasia (PPA) are syndromes that, despite having common features, differ in the brain mechanisms that cause their symptoms. These differences in the underlying functional neuroanatomical changes may influence the way they are addressed by different non­invasive brain stimulation techniques and, in particular, by repetitive transcranial magnetic stimulation (rTMS). The aim of this systematic review is to evaluate the efficacy of rTMS in the treatment of PSA and PPA, as well as the differences in the approach to these disorders using rTMS. To this end, a total of 36 articles were found in the Web of Science, Scopus, and PubMed. The results obtained suggest that whereas in PSA, the selection of the stimulation paradigm is based on bi­hemispheric functional reorganisation models with a tendency towards the application of inhibitory rTMS in the contralateral right hemisphere, in PPA, the application of excitatory rTMS in functionally compromised areas seems to show promising changes. It is concluded that rTMS is a potential treatment in the therapy of both disorders, although differences in the underlying brain mechanisms differentiate the rTMS approach in each case.

Noninvasive Brain Stimulation in Primary Progressive Aphasia: A Literature Review.

Primary progressive aphasia (PPA) is a gradually progressive clinical syndrome in which the first and predominant symptoms involve language and/or speech production that interfere with daily activities. Transcranial magnetic stimulation (TMS) and transcranial direct current stimulation (tDCS) appear to have a beneficial impact on many neurodegenerative pathologies. The current review investigated the impact of rTMS and tDCS on PPA patients. English language articles that have been published in the databases PubMed, and Scopus from 2007 to 2022 were included. Fifteen single-case or small-group studies were analyzed and presented. The majority of the literature findings point toward that the application of rTMS or tDCS may have a positive effect in improving symptoms such as verb production, action naming, phonemic-verbal fluency, grammatical comprehension, written spelling, and semantic features. In conclusion, our review provides additional evidence supporting that both types of stimulation may improve linguistic deficits, especially if they combined, speech therapy.

Feasibility study of Internet video-based speech-language activity for outpatients with primary progressive aphasia.

BACKGROUND: Primary progressive aphasia is a clinical dementia syndrome secondary to neurodegenerative disease characterized by language-related difficulties. Currently, there is no effective treatment for language impairment in primary progressive aphasia. In the present study, we investigated the feasibility of Internet video-based speech-language activities for this condition. METHODS: Twenty-three people with primary progressive aphasia (pwPPA) participated in the study and were provided with twelve speech-language activity videos on a dedicated website, with three sessions per week. The group that chose to continue with participation after three months of intervention received Internet activities for one year. Cognitive domains associated with persistence, treatment motivation, and video difficulty settings were statistically analyzed. RESULTS: After three months, 17 out of 23 participants opted to continue with the activities. The ability to follow oral commands which was measured pre intervention was higher in the group that continued compared with those participants who discontinued activity. The scores of two Standard Language Test of Aphasia subtests, sentence repetition and narrative writing-associated with the ability to comprehend and produce sentence structure-were highly correlated with motivation, interest and concentration in activity. Participants with different levels of primary progressive aphasia progression could participate in the same video-based activities when high-frequency words were used in the video. CONCLUSIONS: Internet video-based speech-language activity at home has potential as a useful tool for future primary progressive aphasia treatment because it provides a cost-effective approach to intensive intervention and overcomes barriers associated with traditional therapy approaches.

Specificity in Generalization Effects of Transcranial Direct Current Stimulation Over the Left Inferior Frontal Gyrus in Primary Progressive Aphasia.

OBJECTIVES: Generalization (or near-transfer) effects of an intervention to tasks not explicitly trained are the most desirable intervention outcomes. However, they are rarely reported and even more rarely explained. One hypothesis for generalization effects is that the tasks improved share the same brain function/computation with the intervention task. We tested this hypothesis in this study of transcranial direct current stimulation (tDCS) over the left inferior frontal gyrus (IFG) that is claimed to be involved in selective semantic retrieval of information from the temporal lobes. MATERIALS AND METHODS: In this study, we examined whether tDCS over the left IFG in a group of patients with primary progressive aphasia (PPA), paired with a lexical/semantic retrieval intervention (oral and written naming), may specifically improve semantic fluency, a nontrained near-transfer task that relies on selective semantic retrieval, in patients with PPA. RESULTS: Semantic fluency improved significantly more in the active tDCS than in the sham tDCS condition immediately after and two weeks after treatment. This improvement was marginally significant two months after treatment. We also found that the active tDCS effect was specific to tasks that require this IFG computation (selective semantic retrieval) but not to other tasks that may require different computations of the frontal lobes. CONCLUSIONS: We provided interventional evidence that the left IFG is critical for selective semantic retrieval, and tDCS over the left IFG may have a near-transfer effect on tasks that depend on the same computation, even if they are not specifically trained. CLINICAL TRIAL REGISTRATION: The Clinicaltrials.gov registration number for the study is NCT02606422.

Multifocal Transcranial Direct Current Stimulation in Primary Progressive Aphasia Does Not Provide a Clinical Benefit Over Speech Therapy.

BACKGROUND: Primary progressive aphasia (PPA) is a group of neurodegenerative disorders including Alzheimer's disease and frontotemporal dementia characterized by language deterioration. Transcranial direct current stimulation (tDCS) is a non-invasive intervention for brain dysfunction. OBJECTIVE: To evaluate the tolerability and efficacy of tDCS combined with speech therapy in the three variants of PPA. We evaluate changes in fMRI activity in a subset of patients. METHODS: Double-blinded, randomized, cross-over, and sham-controlled tDCS study. 15 patients with PPA were included. Each patient underwent two interventions: a) speech therapy + active tDCS and b) speech therapy + sham tDCS stimulation. A multifocal strategy with anodes placed in the left frontal and parietal regions was used to stimulate the entire language network. Efficacy was evaluated by comparing the results of two independent sets of neuropsychological assessments administered at baseline, immediately after the intervention, and at 1 month and 3 months after the intervention. In a subsample, fMRI scanning was performed before and after each intervention. RESULTS: The interventions were well tolerated. Participants in both arms showed clinical improvement, but no differences were found between active and sham tDCS interventions in any of the evaluations. There were trends toward better outcomes in the active tDCS group for semantic association and reading skills. fMRI identified an activity increase in the right frontal medial cortex and the bilateral paracingulate gyrus after the active tDCS intervention. CONCLUSION: We did not find differences between active and sham tDCS stimulation in clinical scores of language function in PPA patients.

"They are dealing with people's lives ": Diagnostic and post-diagnostic healthcare experiences in primary progressive aphasia.

PURPOSE: The healthcare experience is a multifaceted and varied process, particularly for people living with complex conditions such as primary progressive aphasia (PPA). Different experiences influence pathways through the health system, impacting client outcomes. To our knowledge, no previous studies have directly explored the healthcare experiences of people with PPA and their families. This study aimed to explore the experiences of people living with PPA from the perspective of both the person with PPA and their families during diagnostic and post-diagnostic phases, and to identify factors influencing service access and perceptions of quality of care. METHOD: The study followed an Interpretive Phenomenological Analysis (IPA) approach. In-depth, semi-structured interviews were completed with three people with PPA and their primary care partner, and two further care partners of people with PPA. RESULT: Five superordinate themes were identified: characterising the assessment experience, getting a diagnosis, moving beyond the diagnosis, participant interactions with clinicians, and overall service provision. The five superordinate themes comprised 14 subthemes. CONCLUSION: The study provides preliminary insights into the complexity of the PPA healthcare journey, and the need for increased accessibility of information and supports following diagnosis. The findings inform recommendations for improving quality of care and the development of a PPA service framework or care pathway.

"Please Don't Assess Him to Destruction": The R.A.I.S.E. Assessment Framework for Primary Progressive Aphasia.

PURPOSE: Speech-language pathologists (SLPs) assess people with primary progressive aphasia (PPA) through measurements of speech, language, communication, and well-being, with the aims of identifying challenges and strengths, monitoring change, and informing treatment directions and supports. The purpose of this clinical focus article is to highlight the necessity for person-centered assessment specific to PPA and to conceptualize a framework that acknowledges the multifaceted nature of assessment for this population. In this framework, the unique challenges posed by a diagnosis of PPA are addressed with the aim to provide practical guidance for clinicians and to support reflection on current practices. METHOD: In clinical and research practice, assessment of people with PPA requires an ever-evolving approach that is centered on the client. In this clinical focus article, a discussion-based consensus process was used to synthesize authentic longitudinal experiences of people with PPA to explore assessment approaches, tools, and philosophies. RESULTS: This analysis of person-centered assessment identifies seven essential components of assessment in PPA that set the foundation for the five steps of the R.A.I.S.E. Assessment framework. These components each contribute to a clear definition of assessment that reveals clients' competencies with a strengths-based focus; prioritizes the reciprocity of benefits; promotes dynamic assessment; and recognizes the complexity, evolution of assessment over time, and advocacy. CONCLUSIONS: This clinical focus article takes a novel look at assessment in PPA by stepping away from assessment practices that focus on revealing deficits and decline and, instead, provides practical recommendations through the conceptualization of a PPA-specific assessment framework. The R.A.I.S.E. Assessment framework is grounded in principles of uplifting clients through person-centered assessment, keeps pace with best practice in PPA intervention, and contributes to a supportive experience for clients and families in the face of a progressive diagnosis over time.

Access, referral, service provision and management of individuals with primary progressive aphasia: A survey of speech-language therapists in Italy.

BACKGROUND AND OBJECTIVES: In Italy, approximately 650 individuals receive a diagnosis of primary progressive aphasia (PPA) every year. Unfortunately, the frequency with which patients are referred to speech-language services is suboptimal, likely due to skepticism regarding the value of speech-language therapy in the context of neurodegeneration. MATERIALS AND METHODS: We conducted a virtual survey of speech and language therapists (SLTs) across Italy, to collect information about the assessment, intervention and management of patients with PPA. To ensure that as many SLTs as possible received the survey, the Italian Federation of SLTs (Federazione Logopedisti Italiani, FLI) aided in disseminating the survey. RESULTS: In total, 336 respondents participated in the online survey, 140 of whom had previous experience with PPA patients. Respondents indicated having seen a total of 428 PPA patients in the previous 24 months (three patients on average, range: 0-40). SLTs who reported never working with PPA identified underdiagnoses, low referral rates and the rarity of the clinical syndrome as major reasons for their lack of experience with PPA. SLTs with experience working with PPA indicated that patients may not have accessed services because of service dysfunction and geographical barriers. Respondents reported using informal interviews during assessments and tests developed for post-stroke aphasia, while impairment-based/restitutive interventions were utilised most often. CONCLUSION: Findings may serve to inform health policy organisations regarding the current shortcomings and needed recommendations for improving the care of individuals with PPA in Italy. Improving awareness of the utility of rehabilitation among SLTs and other clinical service providers may serve to facilitate access to intervention, which in turn will serve to better support individuals living with PPA. WHAT THIS PAPER ADDS: What is already known on the subject Speech and language therapists (SLTs) play a crucial role in the assessment, diagnosis and treatment of people with primary progressive aphasia (PPA). However, the frequency with which individuals with PPA are referred for speech and language services is suboptimal due to skepticism regarding the value of speech and language therapy in the context of neurodegeneration, the scarcity of SLTs with expertise in the treatment of PPA and the lack of awareness of the SLT role amongst referrers. What this paper adds to existing knowledge In recognition of the lack of published information on the provision of speech and language therapy services and clinicians' approaches to the assessment and treatment of individuals with PPA in Italy, we conducted an online survey to evaluate the current referral patterns for speech and language therapy services and to examine the current barriers to access these services for individuals with PPA in Italy. What are the potential or actual clinical implications of this work? The data presented here support that SLTs view treatment as useful for individuals with PPA and other professional figures and may serve to improve access to intervention, which in turn will serve to better support individuals living with PPA. The results highlight the need to inform health policy organisations about current gaps and aid in developing recommendations for improving the care of individuals with PPA, in order to understand how SLTs can best support individuals with PPA and their families.

Sex differences in effects of tDCS and language treatments on brain functional connectivity in primary progressive aphasia.

Primary Progressive Aphasia (PPA) is a neurodegenerative disorder primarily affecting language functions. Neuromodulatory techniques (e.g., transcranial direct current stimulation, active-tDCS) and behavioral (speech-language) therapy have shown promising results in treating speech and language deficits in PPA patients. One mechanism of active-tDCS efficacy is through modulation of network functional connectivity (FC). It remains unknown how biological sex influences FC and active-tDCS or language treatment(s). In the current study, we compared sex differences, induced by active-tDCS and language therapy alone, in the default mode and language networks, acquired during resting-state fMRI in 36 PPA patients. Using a novel statistical method, the covariate-assisted-principal-regression (CAPs) technique, we found sex and age differences in FC changes following active-tDCS. In the default mode network (DMN): (1) men (in both conditions) showed greater FC in DMN than women. (2) men who received active-tDCS showed greater FC in the DMN than men who received language-treatment only. In the language network: (1) women who received active-tDCS showed significantly greater FC across the language network than women who received sham-tDCS. As age increases, regardless of sex and treatment condition, FC in language regions decreases. The current findings suggest active-tDCS treatment in PPA alters network-specific FC in a sex-dependent manner.

Treatment of primary progressive aphasia by repetitive transcranial magnetic stimulation: a randomized, double-blind, placebo-controlled study.

To evaluate the efficacy of high-frequency repetitive transcranial magnetic stimulation (rTMS) in patients with primary progressive aphasia (PPA). In this randomized, double-blind trial in a single center, patients who were diagnosed with PPA were randomly assigned to receive either real rTMS or sham rTMS treatment. High-frequency rTMS was delivered to the dorsolateral prefrontal cortex (DLPFC). The primary outcome was the change in Boston Naming Test (BNT) score at each follow-up compared to the baseline. The secondary outcomes included change in CAL (Communicative Activity Log) and WAB (Western Aphasia Battery) compared to baseline and neuropsychological assessments. Forty patients (16 with nonfluent, 12 with semantic and 12 with logopenic variant PPA) were enrolled and randomly assigned to the rTMS or sham rTMS group, with 20 patients in each group. Thirty-five patients (87.5%) completed a 6-month follow-up. Compared to the sham rTMS group, the BNT improvement and WAB improvement in the real rTMS group were significantly higher. These significant improvements could be observed throughout the entire 6-month follow-up. At 1 month and 3 months after treatment, CAL improvements of real rTMS were significantly higher than sham rTMS. The improvements in BNT, CAL and WAB did not significantly differ among PPA variants. No significant improvement in neuropsychological assessments was observed. High-frequency rTMS delivered to DLPFC improved language functions in patients with different PPA variants. The efficacy was still observed after 6 months of treatment. Trial registration: NCT04431401 ( https://clinicaltrials.gov/ct2/show/NCT04431401 ).

Development of a Palliative Care Approach for Primary Progressive Aphasia: My Experience as a Person Living With This Rare Disorder.

Frontotemporal disorders are a group of rare young-onset dementias for which there is no cure, nor is there any way to slow the underlying progressive brain degeneration. To date those affected have typically received very little, if any, follow-up care after diagnosis, particularly in the early stages of their disease. I have received a clinical diagnosis, supported by imaging, of primary progressive aphasia, a form of frontotemporal degeneration characterized in the initial phase by progressive impairment of language ability. From the onset, I have been fortunate to receive excellent ongoing palliative care from a multidisciplinary team, some of whom had never previously seen anyone with this disorder. My quality of life has been enhanced by an evolving range of creative strategies and adaptations targeted to my deficits as they have arisen. In this paper, I discuss my experience of the process underlying this personalized plan, which serves as a paradigm for the development of novel palliative care approaches for people living with rare disorders, both neurodegenerative diseases and other conditions.