The primary progressive aphasias are a heterogeneous group of focal ‘language-led’ dementias that pose substantial challenges for diagnosis and management. Here we present a clinical approach to the progressive aphasias, based on our experience of these disorders and directed at non-specialists. We first outline a framework for assessing language, tailored to the common presentations of progressive aphasia. We then consider the defining features of the canonical progressive nonfluent, semantic and logopenic aphasic syndromes, including ‘clinical pearls’ that we have found diagnostically useful and neuroanatomical and other key associations of each syndrome. We review potential diagnostic pitfalls and problematic presentations not well captured by conventional classifications and propose a diagnostic ‘roadmap’. After outlining principles of management, we conclude with a prospect for future progress in these diseases, emphasising generic information processing deficits and novel pathophysiological biomarkers.Electronic supplementary materialThe online version of this article (10.1007/s00415-018-8762-6) contains supplementary material, which is available to authorized users.

Objective To assess the extent of UK speech and language therapy engagement in assessment and management of primary progressive aphasia, determine the factors contributing to any shortfall and explore a gap in the research literature on current speech and language therapy practices with people with primary progressive aphasia. Methods A 37-item, pilot-tested survey was distributed electronically via clinical networks and through the Royal College of Speech and Language Therapists. Survey items included questions on intervention approaches, referral numbers and demographics, referral sources and access to services. Results One hundred and five speech and language therapists completed the survey. Over the previous 24 months, respondents reported seeing a total of 353 people with primary progressive aphasia (an average of 3.27 per speech and language therapist). Neurologists were the most commonly reported referrers to speech and language therapy (22.5%). Seventy-eight percent of respondents reported that people with primary progressive aphasia experienced barriers to accessing speech and language therapy. Key barriers were a lack of referrer awareness of a speech and language therapist's role, and restrictive eligibility criteria for services. Conclusions This study highlighted inequities in access to speech and language therapy for people with primary progressive aphasia. The medical and speech and language therapy professions need to develop appropriate care pathways for people with primary progressive aphasia. Speech and language therapists have a duty to develop a relevant evidence base for speech and language interventions for people with 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.

BackgroundPrimary progressive aphasia is a language-led dementia, often associated with frontotemporal dementia. It presents as insidious deterioration of language skills (e.g. naming objects and understanding complex sentences), with relative sparing of cognitive skills initially. There is little research examining the effectiveness of communication skills training for primary progressive aphasia, yet speech and language therapists (SLTs) report regularly using this in clinical practice. ‘Better Conversations with Primary Progressive Aphasia’ has potential to reduce barriers and increase facilitators to conversation and consequently improve confidence in communication and quality of life for people living with primary progressive aphasia and their conversation partners. The aim of this pilot study is to examine the feasibility of running a trial of the ‘Better Conversations with Primary Progressive Aphasia’ intervention.MethodsA single blind, randomised controlled pilot study will recruit 42 participants with primary progressive aphasia and their conversation partners across seven UK National Health Service Trusts. Participants will be randomised on a 1:1 basis, stratified by site, to receive either the ‘Better Conversations with Primary Progressive Aphasia’ intervention (21 couples) or no speech and language therapy treatment (21 couples). Participants are recruited by SLTs who will conduct pre-intervention assessment (week 1) and deliver the intervention (weeks 2 to 5). Junior researchers, who are blinded to allocation, will complete post-intervention measures (week 6). SLTs complete 9 h of training to prepare them to deliver the intervention. The primary objective of the study is to establish for a phase III effectiveness study whether the program can be delivered as intended in a UK National Health Service setting. Specifically, it will establish (1) the acceptability of randomisation, (2) an assessment of treatment fidelity to determine necessary levels of SLT training, (3) the most appropriate primary outcome measure, (4) sample size requirements, (5) predicted patient recruitment and retention rates and (6) refined inclusion criteria.DiscussionInsights from this study will be of relevance to guide development of future research and in particular, trials of therapeutic interventions in PPA, as well as for clinical care for this population.Trial registrationRetrospectively registered 28/02/2018 ISRCTN10148247Electronic supplementary materialThe online version of this article (10.1186/s40814-018-0349-6) contains supplementary material, which is available to authorized users.

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. What this paper addsWhat is already known on the subject People with PPA present with communication difficulties, yet they find it difficult to access SLT services. Research literature examining the effectiveness of PPA interventions remains sparse and is dominated by impairment-based approaches to word relearning. Little is known about the nature of services delivered by SLTs to people with PPA in the UK.What this paper adds to existing knowledge SLT respondents report using informal assessment tools such as interviewing, or those designed for stroke-related aphasia, not for dementia. SLTs report that they were more likely to select communication partner training than

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, O'Rourke, Power, O'Halloran, & Rietdijk, 2018). 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.

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

Logopenic variant primary progressive aphasia (lvPPA) is the least well defined of the major primary progressive aphasia (PPA) syndromes. We assessed phoneme discrimination in patients with PPA (semantic, nonfluent/agrammatic, and logopenic variants) and typical Alzheimer's disease, relative to healthy age-matched participants. The lvPPA group performed significantly worse than all other groups apart from tAD, after adjusting for auditory verbal working memory. In the combined PPA cohort, voxel-based morphometry correlated phonemic discrimination score with grey matter in left angular gyrus. Our findings suggest that impaired phonemic discrimination may help differentiate lvPPA from other PPA subtypes, with important diagnostic and management implications.