Binary reversals: a diagnostic sign in primary progressive aphasia.

BACKGROUND: Binary reversals (exemplified by 'yes'/'no' confusions) have been described in patients with primary progressive aphasia (PPA) but their diagnostic value and phenotypic correlates have not been defined. METHODS: We conducted a retrospective cohort study analysing demographic, clinical, neuropsychological, linguistic and behavioural data from patients representing all major PPA syndromes (non-fluent/agrammatic variant, nfvPPA; logopenic variant, lvPPA; semantic variant, svPPA) and behavioural variant frontotemporal dementia (bvFTD). The prevalence of binary reversals and behavioural abnormalities, illness duration, parkinsonian features and neuropsychological test scores were compared between neurodegenerative syndromes, and the diagnostic predictive value of binary reversals was assessed using logistic regression. RESULTS: Data were obtained for 83 patients (21 nfvPPA, 13 lvPPA, 22 svPPA, 27 bvFTD). Binary reversals occurred in all patients with nfvPPA, but significantly less frequently and later in lvPPA (54%), svPPA (9%) and bvFTD (44%). Patients with bvFTD with binary reversals had significantly more severe language (but not general executive or behavioural) deficits than those without reversals. Controlling for potentially confounding variables, binary reversals strongly predicted a diagnosis of nfvPPA over other syndromes. CONCLUSIONS: Binary reversals are a sensitive (though not specific) neurolinguistic feature of nfvPPA, and should suggest this diagnosis if present as a prominent early symptom.

Dysphagia in primary progressive aphasia: Clinical predictors and neuroanatomical basis.

BACKGROUND AND PURPOSE: Dysphagia is an important feature of neurodegenerative diseases and potentially life-threatening in primary progressive aphasia (PPA) but remains poorly characterized in these syndromes. We hypothesized that dysphagia would be more prevalent in nonfluent/agrammatic variant (nfv)PPA than other PPA syndromes, predicted by accompanying motor features, and associated with atrophy affecting regions implicated in swallowing control. METHODS: In a retrospective case-control study at our tertiary referral centre, we recruited 56 patients with PPA (21 nfvPPA, 22 semantic variant [sv]PPA, 13 logopenic variant [lv]PPA). Using a pro forma based on caregiver surveys and clinical records, we documented dysphagia (present/absent) and associated, potentially predictive clinical, cognitive, and behavioural features. These were used to train a machine learning model. Patients' brain magnetic resonance imaging scans were assessed using voxel-based morphometry and region-of-interest analyses comparing differential atrophy profiles associated with dysphagia presence/absence. RESULTS: Dysphagia was significantly more prevalent in nfvPPA (43% vs. 5% svPPA and no lvPPA). The machine learning model revealed a hierarchy of features predicting dysphagia in the nfvPPA group, with excellent classification accuracy (90.5%, 95% confidence interval = 77.9-100); the strongest predictor was orofacial apraxia, followed by older age, parkinsonism, more severe behavioural disturbance, and more severe cognitive impairment. Significant grey matter atrophy correlates of dysphagia in nfvPPA were identified in left middle frontal, right superior frontal, and right supramarginal gyri and right caudate. CONCLUSIONS: Dysphagia is a common feature of nfvPPA, linked to underlying corticosubcortical network dysfunction. Clinicians should anticipate this symptom particularly in the context of other motor features and more severe disease.

Symptom-based staging for logopenic variant primary progressive aphasia.

BACKGROUND AND PURPOSE: Logopenic variant primary progressive aphasia (lvPPA) is a major variant presentation of Alzheimer's disease (AD) that signals the importance of communication dysfunction across AD phenotypes. A clinical staging system is lacking for the evolution of AD-associated communication difficulties that could guide diagnosis and care planning. Our aim was to create a symptom-based staging scheme for lvPPA, identifying functional milestones relevant to the broader AD spectrum. METHODS: An international lvPPA caregiver cohort was surveyed on symptom development under an 'exploratory' survey (34 UK caregivers). Feedback from this survey informed the development of a 'consolidation' survey (27 UK, 10 Australian caregivers) in which caregivers were presented with six provisional clinical stages and feedback was analysed using a mixed-methods approach. RESULTS: Six clinical stages were endorsed. Early symptoms included word-finding difficulty, with loss of message comprehension and speech intelligibility signalling later-stage progression. Additionally, problems with hearing in noise, memory and route-finding were prominent early non-verbal symptoms. 'Milestone' symptoms were identified that anticipate daily-life functional transitions and care needs. CONCLUSIONS: This work introduces a new symptom-based staging scheme for lvPPA, and highlights milestone symptoms that could inform future clinical scales for anticipating and managing communication dysfunction across the AD spectrum.

Comprehension of acoustically degraded speech in Alzheimer's disease and primary progressive aphasia.

Successful communication in daily life depends on accurate decoding of speech signals that are acoustically degraded by challenging listening conditions. This process presents the brain with a demanding computational task that is vulnerable to neurodegenerative pathologies. However, despite recent intense interest in the link between hearing impairment and dementia, comprehension of acoustically degraded speech in these diseases has been little studied. Here we addressed this issue in a cohort of 19 patients with typical Alzheimer's disease and 30 patients representing the three canonical syndromes of primary progressive aphasia (non-fluent/agrammatic variant primary progressive aphasia; semantic variant primary progressive aphasia; logopenic variant primary progressive aphasia), compared to 25 healthy age-matched controls. As a paradigm for the acoustically degraded speech signals of daily life, we used noise-vocoding: synthetic division of the speech signal into frequency channels constituted from amplitude-modulated white noise, such that fewer channels convey less spectrotemporal detail thereby reducing intelligibility. We investigated the impact of noise-vocoding on recognition of spoken three-digit numbers and used psychometric modelling to ascertain the threshold number of noise-vocoding channels required for 50% intelligibility by each participant. Associations of noise-vocoded speech intelligibility threshold with general demographic, clinical and neuropsychological characteristics and regional grey matter volume (defined by voxel-based morphometry of patients' brain images) were also assessed. Mean noise-vocoded speech intelligibility threshold was significantly higher in all patient groups than healthy controls, and significantly higher in Alzheimer's disease and logopenic variant primary progressive aphasia than semantic variant primary progressive aphasia (all P < 0.05). In a receiver operating characteristic analysis, vocoded intelligibility threshold discriminated Alzheimer's disease, non-fluent variant and logopenic variant primary progressive aphasia patients very well from healthy controls. Further, this central hearing measure correlated with overall disease severity but not with peripheral hearing or clear speech perception. Neuroanatomically, after correcting for multiple voxel-wise comparisons in predefined regions of interest, impaired noise-vocoded speech comprehension across syndromes was significantly associated (P < 0.05) with atrophy of left planum temporale, angular gyrus and anterior cingulate gyrus: a cortical network that has previously been widely implicated in processing degraded speech signals. Our findings suggest that the comprehension of acoustically altered speech captures an auditory brain process relevant to daily hearing and communication in major dementia syndromes, with novel diagnostic and therapeutic implications.

The landscape of inequalities in dementia across Europe: First insights from the INTERDEM taskforce.

BACKGROUND: Getting a diagnosis of dementia does not equate to equitable access to care. People with dementia and unpaid carers face many barriers to care, which can vary within, and across, different countries and cultures. With little evidence across different countries, the aim of this scoping exercise was to identify the different and similar types of inequalities in dementia across Europe, and provide recommendations for addressing these. METHODS: We conducted a brief online survey with INTERDEM and INTERDEM Academy members across Europe, and with members of Alzheimer Europe's European Working Group of People with Dementia and Carers in February and March 2023. Members were asked about whether inequalities in dementia care existed within their country; if yes, to highlight three key inequalities. Responses on barriers were coded into groups, and frequencies of inequalities were calculated. Highlighted inequalities were discussed and prioritised at face-to-face and virtual consensus meetings in England, Ireland, Italy, and Poland, involving people with dementia, unpaid carers, health and social care providers, and non-profit organisations. RESULTS: Forty-nine academics, PhD students, people with dementia and unpaid carers from 10 countries (Belgium, Denmark, Germany, Greece, Ireland, Italy, Poland, Malta, Netherlands, UK) completed the survey. The most frequently identified inequalities focused on unawareness and lack of information, higher level system issues (i.e. lack of communication among care professionals), lack of service suitability, and stigma. Other barriers included workforce training and knowledge, financial costs, culture and language, lack of single-point-of-contact person, age, and living location/postcode lottery. There was general consensus among people living dementia and care providers of unawareness as a key barrier in different European countries, with varied priorities in Ireland depending on geographical location. CONCLUSIONS: These findings provide a first insight on dementia inequalities across Europe, generate cross-country learnings on how to address these inequalities in dementia, and can underpin further solution-focused research that informs policy and key decision makers to implement changes.

Determining capacity of people with dementia to take part in research: an electronic survey study of researcher confidence, competence and training needs.

BACKGROUND: Researchers are required to determine whether a person has capacity to consent to a research study before they are able to participate. The Mental Capacity Act and accompanying Code of Practice for England and Wales provide some guidance on this process, but researchers have identified that it can be difficult to determine capacity to consent when a person has complex cognitive or communication needs. This study aimed to understand the experiences and opinions of researchers who recruit people with dementia to research projects, to inform the future development of training resources. METHODS: A mixed method, cross-sectional, electronic survey was circulated via social media and research networks in England and Wales. The survey remained open for ten weeks and included open and closed questions exploring respondents' confidence in determining capacity in the context of recruiting people with dementia to consent, their views on training and support they have experienced and their suggestions for future training and support needs. RESULTS: 60 respondents completed the survey from across England and Wales. Although 75% of respondents had experience of determining capacity to consent with people with dementia to research, only 13% rated themselves as feeling 'very confident' in this. Qualitative content analysis of open responses led to the generation of six themes, explaining researchers' confidence, competence and future training needs in this area: (1) Researcher uncertainties, (2) Lack of time, (3) Balancing information complexity with accessibility, (4) Gatekeepers, (5) Existing enablers and (6) Envisioning future training. CONCLUSIONS: Researchers would benefit from specific training in undertaking conversations around consent with people with dementia. People with dementia may have fluctuating capacity, and despite support from caregivers, researchers have little practical guidance on methods of determining a person's ability to understand or appreciate the information they have provided during the consent process. Given the development of large complex trials within dementia research, there is an urgency to develop specific and practical guidance and training for researchers working with people with dementia and their families.

Using Conversation Analysis to explore assessments of decision-making capacity in a hospital setting.

BACKGROUND: Healthcare professionals (HCPs) have a responsibility to conduct assessments of decision-making capacity that comply with the Mental Capacity Act 2005 (MCA). Current best-practice guidance, such as the Mental Capacity Code of Practice and National Institute for Health and Care Excellence decision-making and mental capacity guidance, does not stipulate how to accomplish this in practice, for example, what questions should be asked, how options and information should be provided. In addition, HCPs struggle to assess the capacity of individuals with communication difficulties. AIMS: This study was a service evaluation that aimed to objectively analyse, using Conversation Analysis (CA), how real-life capacity assessments were conducted in a hospital setting with patients with acquired brain injury (ABI)-related communication difficulties. A second aim was to establish the feasibility of using CA to advance knowledge of the conduct of capacity assessment. METHODS & PROCEDURES: Four naturally occurring capacity assessments were video-recorded. Recordings involved speech and language therapists, occupational therapists, neuropsychologists and patients with communication difficulties as a result of ABI. The methods and findings of CA were used to investigate the interactional behaviours of HCPs and patients during assessments of decision-making capacity. The analysis was informed by our knowledge of the MCA best practice guidance. OUTCOMES & RESULTS: An overall structure of capacity assessment that enacted some of the best-practice MCA guidance was identified in one recording, consisting of six phases: (i) opening, (ii) preparation, (iii) option-listing, (iv) test, (v) decision, and (vi) close. The preparation phase consisted of two sub-components: information gathering and information giving. Variation from this structure was observed across the dataset, notably in the way in which options were (or were not) presented. CONCLUSIONS & IMPLICATIONS: CA is a feasible empirical method for exploring the structure and conduct of capacity assessments. CA identifies and provides ways of describing interactional behaviours that align with and diverge from best-practice MCA guidance. Future CA studies including a wider range of health and social care professionals and patients have the potential to inform evidence based training for HCPs who conduct assessments of decision-making capacity. WHAT THIS PAPER ADDS: What is already known on this subject The Mental Capacity Act (MCA) is poorly implemented in practice. Healthcare professionals (HCPs) find it challenging to assess the decision-making capacity of individuals with communication difficulties, and people with communication difficulties are often excluded from or insufficiently supported during capacity assessment. Research is limited to self-report methods. Observational studies of capacity assessment are required. What this study adds This is the first study to use Conversation Analysis (CA) to explore how capacity assessments are conducted in a hospital setting by HCPs with people with communication difficulties as a result of acquired brain injury. One video-recorded capacity assessment was structured in six phases that aligned with best practice MCA guidance. However, other capacity assessments deviated from this structure. One phase, option listing, varied in practice and options were not always presented. What are the clinical implications of this work? CA revealed interactional behaviours that align with and diverge from best-practice MCA guidance. Future CA studies are warranted to inform training for health and social care professionals who conduct capacity assessments.

'Communication is difficult': Speech, language and communication needs of people with young onset or rarer forms of non-language led dementia.

BACKGROUND: People with behavioural variant frontotemporal dementia, Lewy body dementia, posterior cortical atrophy and young onset Alzheimer's disease may experience language and communication difficulties. However, the role of speech and language interventions for people with these non-language led dementias has received little attention. AIMS: This study aimed to explore the experiences and perspectives of people living with these conditions, and their families, regarding their language and communication difficulties and how speech and language therapy could address these needs. METHODS: This study employed a qualitative design to explore the experiences of people living with or caring for somebody with behavioural variant frontotemporal dementia, Lewy body dementia, posterior cortical atrophy or young onset Alzheimer's disease, and to understand their opinions about speech and language therapy. Participants were recruited from a support service connected to a dementia clinic to attend one of five focus group meetings. Videorecorded focus groups and interviews were transcribed, and reflexive thematic analysis was used to analyse data from people affected by each type of dementia. RESULTS: A total of 25 participants were recruited to the study, with representation across the different forms of non-language led dementias. The four main themes identified were: (1) communication difficulties as a key difficulty, (2) loss and loneliness, (3) speech and language therapy, and (4) the role of the caregiver. Sixteen subthemes were also identified which highlighted individual issues across disease types. DISCUSSION: Although all the forms of dementia studied here are not considered to be language-led, people with these conditions and/or their care partners identified speech, language and communication as common challenges. These communication difficulties were reported to have a negative impact on their social participation and mental health and participants felt speech and language interventions could help. There is a need for research exploring speech and language interventions developed for and with people with non-language led dementias and their care partners, to ensure they meet the needs of the people they are designed for. WHAT THIS PAPER ADDS: What is already known on the subject People with primary progressive aphasia present with speech, language and communication difficulties, and several speech and language interventions have been developed to meet the needs of this population. However, people with non-language led dementias may also experience speech, language and communication difficulties, and little is known about interventions that may address these difficulties. What this paper adds to existing knowledge People living with or caring for somebody with behavioural variant frontotemporal dementia, Lewy body dementia, posterior cortical atrophy and young onset Alzheimer's disease report experiencing speech, language and communication difficulties that impact on the person with dementia's social participation and mood. Participants in this study also shared their opinions about how speech and language interventions could help, from the earliest stages of the disease. What are the potential or actual clinical implications of this work? Speech and language therapists need to address the individual speech, language and communication needs of people with dementias, even those that are not thought to be language-led. Current speech and language therapy service provision does not meet the needs of people with non-language led dementias and further research is required to develop interventions and services to meet these needs.

Symptom-led staging for semantic and non-fluent/agrammatic variants of primary progressive aphasia.

INTRODUCTION: Here we set out to create a symptom-led staging system for the canonical semantic and non-fluent/agrammatic variants of primary progressive aphasia (PPA), which present unique diagnostic and management challenges not well captured by functional scales developed for Alzheimer's disease and other dementias. METHODS: An international PPA caregiver cohort was surveyed on symptom development under six provisional clinical stages and feedback was analyzed using a mixed-methods sequential explanatory design. RESULTS: Both PPA syndromes were characterized by initial communication dysfunction and non-verbal behavioral changes, with increasing syndromic convergence and functional dependency at later stages. Milestone symptoms were distilled to create a prototypical progression and severity scale of functional impairment: the PPA Progression Planning Aid ("PPA-Squared"). DISCUSSION: This work introduces a symptom-led staging scheme and functional scale for semantic and non-fluent/agrammatic variants of PPA. Our findings have implications for diagnostic and care pathway guidelines, trial design, and personalized prognosis and treatment for PPA. HIGHLIGHTS: We introduce new symptom-led perspectives on primary progressive aphasia (PPA). The focus is on non-fluent/agrammatic (nfvPPA) and semantic (svPPA) variants. Foregrounding of early and non-verbal features of PPA and clinical trajectories is featured. We introduce a symptom-led staging scheme for PPA. We propose a prototype for a functional impairment scale, the PPA Progression Planning Aid.

Speaking up for the lost voices: representation and inclusion of people with communication impairment in brain tumour research.

Brain tumours and their associated treatments can lead to progressive impairments of communication, adversely affecting quality-of-life. This commentary explores our concerns that people with speech, language, and communication needs face barriers to representation and inclusion in brain tumour research; we then offer possible solutions to support their participation. Our main concerns are that there is currently poor recognition of the nature of communication difficulties following brain tumours, limited focus on the psychosocial impact, and lack of transparency on why people with speech, language, and communication needs were excluded from research or how they were supported to take part. We propose solutions focusing on working towards more accurate reporting of symptoms and the impact of impairment, using innovative qualitative methods to collect data on the lived experiences of speech, language, and communication needs, and empowering speech and language therapists to become part of research teams as experts and advocates for this population. These solutions would support the accurate representation and inclusion of people with communication needs after brain tumour in research, allowing healthcare professionals to learn more about their priorities and needs.

'Like going into a chocolate shop, blindfolded': What do people with primary progressive aphasia want from speech and language therapy?

BACKGROUND: Primary progressive aphasia (PPA) describes a group of language-led dementias. PPAs are complex, diverse and difficult to diagnose, and therefore conventional models of aphasia and dementia treatment do not meet their needs. The research evidence on intervention for PPA is developing, but to date there are only a few case studies exploring the experiences of people with PPA (PwPPA) themselves. AIMS: To explore the experiences and opinions of PwPPA and their communication partners (CPs) to understand how speech and language therapy (SLT) services can better meet their needs. METHODS & PROCEDURES: A qualitative research approach was used whereby PwPPA and their friends or family members were recruited to participate in focus groups, via advertisements in the Rare Dementia Support PPA group newsletters. Consenting participants were allocated to attend one of four focus groups hosted on an online video conferencing platform. Participants were asked about their communication difficulties, and how SLT could address these needs. All meetings were transcribed, and data were examined using reflexive thematic analysis. OUTCOMES & RESULTS: Six PwPPA and 14 CPs representing all three PPA variants and mixed PPA participated in the focus groups. Four main themes were identified during the analysis of the focus group discussions: (1) CPs' burden, (2) adjusting to the diagnosis, (3) communication abilities and difficulties and (4) beyond language. A further 10 subthemes were identified. CONCLUSIONS & IMPLICATIONS: This study provides a greater understanding of the experiences and needs of PwPPA and their families in relation to SLT. This work underlines the importance of a person-centred approach that considers the broader needs of both the PwPPA and the people around them. This will enable service providers to deliver SLT that meets the needs of PwPPA and their families and will also inform future research in this field. WHAT THIS PAPER ADDS: What is already known on this subject We know that PwPPA can maintain or even make improvements in word retrieval and speech fluency with SLT exercises. There is also developing evidence of the benefits of interventions such as CP training, communication aid support and other functional interventions. What this paper adds to existing knowledge This study provides an understanding of the experiences and opinions of people living with PPA and their families in relation to SLT. Results demonstrate that PwPPA and their families have to navigate a complex journey, identifying strategies to support communication but also the influence of personality and other cognitive symptoms. SLT was useful, but not always available. What are the potential or actual clinical implications of this work? This study will enable service providers to better plan, justify funding for and delivery of SLT that will meet the needs of PwPPA and their families. Most importantly this work underlines the importance of a person-centred approach, incorporating the broader needs of the person with PPA and those around them.

Measuring communication as a core outcome in aphasia trials: Results of the ROMA-2 international core outcome set development meeting.

BACKGROUND: Evidence-based recommendations for a core outcome set (COS; minimum set of outcomes) for aphasia treatment research have been developed (the Research Outcome Measurement in Aphasia-ROMA, COS). Five recommended core outcome constructs: communication, language, quality of life, emotional well-being and patient-reported satisfaction/impact of treatment, were identified through three international consensus studies. Constructs were paired with outcome measurement instruments (OMIs) during an international consensus meeting (ROMA-1). Before the current study (ROMA-2), agreement had not been reached on OMIs for the constructs of communication or patient-reported satisfaction/impact of treatment. AIM: To establish consensus on a communication OMI for inclusion in the ROMA COS. METHODS & PROCEDURES: Research methods were based on recommendations from the Core Outcome Measures in Effectiveness Trials (COMET) Initiative. Participants with expertise in design and conduct of aphasia trials, measurement instrument development/testing and/or communication outcome measurement were recruited through an open call. Before the consensus meeting, participants agreed on a definition of communication, identified appropriate OMIs, extracted their measurement properties and established criteria for their quality assessment. During the consensus meeting they short-listed OMIs and participants without conflicts of interest voted on the two most highly ranked instruments. Consensus was defined a priori as agreement by ≥ 70% of participants. OUTCOMES & RESULTS: In total, 40 researchers from nine countries participated in ROMA-2 (including four facilitators and three-panel members who participated in pre-meeting activities only). A total of 20 OMIs were identified and evaluated. Eight short-listed communication measures were further evaluated for their measurement properties and ranked. Participants in the consensus meeting (n = 33) who did not have conflicts of interest (n = 29) voted on the top two ranked OMIs: The Scenario Test (TST) and the Communication Activities of Daily Living-3 (CADL-3). TST received 72% (n = 21) of 'yes' votes and the CADL-3 received 28% (n = 8) of 'yes' votes. CONCLUSIONS & IMPLICATIONS: Consensus was achieved that TST was the preferred communication OMI for inclusion in the ROMA COS. It is currently available in the original Dutch version and has been adapted into English, German and Greek. Further consideration must be given to the best way to measure communication in people with mild aphasia. Development of a patient-reported measure for satisfaction with/impact of treatment and multilingual versions of all OMIs of the COS is still required. Implementation of the ROMA COS would improve research outcome measurement and the quality, relevance, transparency, replicability and efficiency of aphasia treatment research. WHAT THIS PAPER ADDS: What is already known on this subject International consensus has been reached on five core constructs to be routinely measured in aphasia treatment studies. International consensus has also been established for OMIs for the three constructs of language, quality of life and emotional well-being. Before this study, OMIs for the constructs of communication and patient-reported satisfaction/impact of treatment were not established. What this paper adds to existing knowledge We gained international consensus on an OMI for the construct of communication. TST is recommended for inclusion in the ROMA COS for routine use in aphasia treatment research. What are the potential or actual clinical implications of this work? The ROMA COS recommends OMIs for a minimum set of outcomes for adults with post-stroke aphasia within phases I-IV aphasia treatment research. Although not intended for clinical use, clinicians may employ the instruments of the ROMA COS, considering the quality of their measurement properties. The systematic inclusion of a measure of communication, such as TST, in clinical practice could ultimately support the implementation of research evidence and best practices.

Translating Ultrasound into Clinical Practice for the Assessment of Swallowing and Laryngeal Function: A Speech and Language Pathology-Led Consensus Study.

Ultrasound (US) has an emerging evidence base for the assessment of swallowing and laryngeal function. Accessibility and technological advances support the use of US as a clinical assessment tool; however, there is insufficient evidence to support its translation into clinical practice. This study aimed to establish consensus on the priorities for translation of US into clinical practice for the assessment of swallowing and laryngeal function. Nominal Group Technique (NGT) was used as a formal method of consensus development. Clinicians and academics, all members of an international US working group, were invited to participate in the study. Two NGT meetings were held, where participants silently generated and then shared ideas. Participants anonymously ranked items. Rankings were aggregated before participants re-ranked items in order of priority. Discussions regarding rankings were recorded and transcribed to inform analysis. Member-checking with participants informed the final analysis. Participants (n = 15) were speech and language pathologists, physiotherapists and sonographers representing six countries. Fifteen items were identified and prioritised 1-13 (including two equally ranked items). Reliability, validity and normative data emerged as key areas for research while development of training protocols and engagement with stakeholders were considered vital to progressing US into practice. Analysis revealed common themes that might be addressed together in research, in addition to the ranked priority. A measured approach to the translation of US into clinical practice will enable effective implementation of this tool. Priorities may evolve as clinical and professional contexts shift, but this study provides a framework to advance research and clinical practice in this field.

Primary Progressive Aphasia: Toward a Pathophysiological Synthesis.

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

Using the Medical Research Council framework and public involvement in the development of a communication partner training intervention for people with primary progressive aphasia (PPA): Better Conversations with PPA.

BACKGROUND: Primary progressive aphasia is a language-led dementia resulting in a gradual dissolution of language. Primary progressive aphasia has a significant psychosocial impact on both the person and their families. Speech and language therapy is one of the only available management options, and communication partner training interventions offer a practical approach to identify strategies to support conversation. The aim of this study was to define and refine a manual and an online training resource for speech and language therapists to deliver communication partner training to people with primary progressive aphasia and their communication partners called Better Conversations with primary progressive aphasia. METHODS: The Better Conversations with primary progressive aphasia manual and training program were developed using the Medical Research Council framework for developing complex interventions. The six-stage development process included 1. Exploratory review of existing literature including principles of applied Conversation Analysis, behaviour change theory and frameworks for chronic disease self-management, 2. Consultation and co-production over 12 meetings with the project steering group comprising representatives from key stakeholder groups, 3. Development of an initial draft, 4. Survey feedback followed by a consensus meeting using the Nominal Group Techniques with a group of speech and language therapists, 5. Two focus groups to gather opinions from people with PPA and their families were recorded, transcribed and Thematic Analysis used to examine the data, 6. Refinement. RESULTS: Co-production of the Better Conversations with primary progressive aphasia resulted in seven online training modules, and a manual describing four communication partner training intervention sessions with accompanying handouts. Eight important components of communication partner training were identified in the aggregation process of the Nominal Group Technique undertaken with 36 speech and language therapists, including use of video feedback to focus on strengths as well as areas of conversation breakdown. Analysis of the focus groups held with six people with primary progressive aphasia and seven family members identified three themes 1) Timing of intervention, 2) Speech and language therapists' understanding of types of dementia, and 3) Knowing what helps. These data informed refinements to the manual including additional practice activities and useful strategies for the future. CONCLUSIONS: Using the Medical Research Council framework to develop an intervention that is underpinned by a theoretical rationale of how communication partner training causes change allows for the key intervention components to be strengthened. Co-production of the manual and training materials ensures the intervention will meet the needs of people with primary progressive aphasia and their communication partners. Gathering further data from speech and language therapists and people living with primary progressive aphasia and their families to refine the manual and the training materials enhances the feasibility of delivering this in preparation for a phase II NHS-based randomised controlled pilot-feasibility study, currently underway.

Cognitive stimulation therapy (CST) for dementia: a systematic review of qualitative research.

INTRODUCTION: Cognitive Stimulation Therapy (CST) is a well-established intervention for people with dementia shown to improve cognition and quality of life. Past research includes development of a longer term 'maintenance CST' and an individual CST programme. Previous reviews of CST have focused on quantitative outcomes or excluded certain formats of CST. This review aimed to fill this gap by evaluating how the voices of facilitators, carers and people with dementia in qualitative studies of CST can contribute to our understanding of its implementation and how it is experienced. METHODS: The current systematic review explored the experience and perspectives of people with dementia, facilitators and carers. Thematic Analysis was used to analyse this data, alongside guidance on synthesising qualitative findings. RESULTS: A systematic literature search retrieved 10 relevant studies using qualitative methodology. Eighteen themes were generated, which were grouped into three categories: 'Acceptability and feasibility', 'Features of CST' and 'Key outcomes'. CONCLUSIONS: To our knowledge, this is the only review to explore solely qualitative studies of CST. Findings provided insight into the shared features, outcomes and factors affecting implementation, and suggested theories for discrepancies between quantitative and qualitative findings in the literature. Some of the common themes were also in keeping with past reviews.

Effects of functional communication interventions for people with primary progressive aphasia and their caregivers: a systematic review.

Objectives: Primary progressive aphasia (PPA) is a language led dementia characterised by progressive speech and language difficulties. Impairment focused PPA interventions that seek to remediate, alleviate or improve symptoms, dominate the research literature. Yet speech and language therapists (SLTs) report prioritising functional communication interventions (FCIs), which target engagement in an activity and participation in life situations. This systematic review investigates the research literature on FCIs for PPA to identify the key components of these interventions and their effectiveness.Method: A systematic search of databases identified 19 studies published between 1998 and 2018. Data were extracted from the articles using the Intervention Taxonomy adaptation (ITAX).Results: Results show that the two most common components of FCIs are to build on communication strategies people currently use, and to practise these strategies with a communication partner. There are variations in the interventions, such as location and dosage. All 19 studies report improvements, of which eight report statistically significant results. Forty-two different measures are used across the 19 studies.Conclusion: This study highlights that building on existing strategies and practising these with a CP, are key components of FCIs for people with PPA, yet there remains a lack of clarity around optimal dosage. Further rigorous research using a core set of outcome measures is a priority in this area.

Speech and language therapy approaches to managing primary progressive aphasia.

The term primary progressive aphasia (PPA) describes a group of neurodegenerative disorders with predominant speech and language dysfunction as their main feature. There are three main variants - the semantic variant, the nonfluent or agrammatic variant and the logopenic variant - each with specific linguistic deficits and different neuroanatomical involvement. There are currently no curative treatments or symptomatic pharmacological therapies. However, speech and language therapists have developed several impairment-based interventions and compensatory strategies for use in the clinic. Unfortunately, multiple barriers still need to be overcome to improve access to care for people with PPA, including increasing awareness among referring clinicians, improving training of speech and language therapists and developing evidence-based guidelines for therapeutic interventions. This review highlights this inequity and the reasons why neurologists should refer people with PPA to speech and language therapists.

Speech and language therapy for primary progressive aphasia across the UK: A survey of current practice.

BACKGROUND: Primary progressive aphasia (PPA) describes a heterogeneous group of language-led dementias. People with this type of dementia are increasingly being referred to speech and language therapy (SLT) services. Yet, there is a paucity of research evidence focusing on PPA interventions and little is known about SLT practice in terms of assessment and provision of intervention. AIMS: To survey the practices of SLTs in the areas of assessment and intervention for people with PPA. METHODS & PROCEDURES: A 37-item, pilot-tested survey was distributed electronically through the Royal College of Speech and Language Therapists (RCSLT), Clinical Excellence Networks (CENs) and social media networks. Survey items included questions on care pathways, assessment and intervention approaches, and future planning. Analysis was conducted using descriptive statistics and thematic analysis. OUTCOMES & RESULTS: A total of 105 SLTs completed the survey. Respondents reported more frequently using formal assessment tools designed for stroke-related aphasia than for dementia. Informal interviews were reportedly always used during assessment by almost 80% of respondents. Respondents were significantly more likely to use communication partner training than impairment-focused interventions. Goal attainment was the most commonly used outcome measure. Respondents provided 88 goal examples, which fell into six themes: communication aid; conversation; functional communication; impairment focused; specific strategy; and communication partner. Additionally, respondents reported addressing areas such as future deterioration in communication and cognition, decision-making and mental capacity, and driving. Ten (9.4%) respondents reported the existence of a care pathway for people with PPA within their service. CONCLUSIONS & IMPLICATIONS: This survey highlights the range of current PPA assessment and intervention practices in use by the respondents. Communication partner training is commonly used by the surveyed SLTs, despite the lack of research evidence examining its effectiveness for PPA. There is a need to develop evidence-based care pathways for people with PPA in order to advocate for further commissioning of clinical services.

Development of fidelity of delivery and enactment measures for interventions in communication disorders.

OBJECTIVES: This study was part of a process evaluation for a single-blind, randomized controlled pilot study comparing Better Conversations with Primary Progressive Aphasia (BCPPA), an approach to communication partner training, with no speech and language therapy treatment. It was necessary to explore fidelity of delivery (delivery of intervention components) and intervention enactment (participants' use of intervention skills in the form of conversation behaviours comprising facilitators, that enhance the conversational flow, and barriers, that impeded the flow of conversation). This study aimed to: (1) Outline an adapted methodological process that uses video observation, to measure both fidelity of delivery and enactment. (2) Measure the extent to which the BCPPA pilot study was delivered as planned, and enacted. DESIGN: Observational methods were used alongside statistical analysis to explore the fidelity of intervention and enactment using video recordings obtained from the BCPPA pilot study. METHODS: A 5-step methodology, was developed to measure fidelity of delivery and enactment for the BCPPA study using video-recorded data. To identify delivery of intervention components, a random sample of eight video recorded and transcribed BCPPA intervention sessions was coded. To examine the enactment of conversation behaviours, 108 transcribed 10 -min-video recorded conversations were coded from 18 participants across the control and intervention group. RESULTS: Checklists and guidelines for measurement of fidelity of treatment delivery and coding spreadsheets and guidelines for measurement of enactment are presented. Local collaborators demonstrated 87.2% fidelity to the BCPPA protocol. Participants in the BCPPA treatment group increased their use of facilitator behaviours enacted in conversation from a mean of 13.5 pre-intervention to 14.2 post-intervention, whilst control group facilitators decreased from a mean of 15.5 to 14.4, over the same timescale. CONCLUSIONS: This study proposes a novel and robust methods, using video recorded intervention sessions and conversation samples, to measure both fidelity of intervention delivery and enactment. The learnings from this intervention are transferable to other communication interventions.