BACKGROUND:Linguistic and acculturation variables are known to influence cognitive performance on neuropsychological measures used to assess cognitive impairment and dementia. It is not clear, however, how to best measure these variables in an Australian context to improve cognitive assessment of older adults from culturally and linguistically diverse (CALD) backgrounds. We therefore developed a new measure, the Characterising Language Experience and Acculturation Questionnaire (CLEAr-Q) and used Exploratory Factor Analysis (EFA) to determine the underlying factor structure. METHOD:An online version of the CLEAr-Q was developed using items drawn or modified from the literature with consultation from a CALD community working group via an adapted participatory research framework. It was administered in English via an anonymous survey. From the initial 52 items, 31 were considered for further analysis. EFA with oblique rotation (geomin) was used. RESULT:The validation sample included 256 participants aged 60-93 (Table 1) born outside of Australia and reporting a native Language Other Than English (LOTE). EFA suggested a four-factor solution: Educational Exposure to English, English Proficiency and Identification with Majority Culture, Dominance of English Language and Culture, and Language Switching. The model fitted well with the data, with Comparative Fit Index (CFI) = 0.98 and Standardised Root Mean Square Residual (SRMR) = 0.07. In combination, these factors explained 58.9% of the variance. Table 2 presents factor loadings. CONCLUSION:The present study demonstrates the utility of the CLEAr-Q in characterising the diversity in linguistic and acculturation variables in an Australian CALD sample. Our results also inform item selection for a streamlined version of the CLEAr-Q. The factor structure provides evidence for the interwoven relationship between linguistic and acculturation variables and supports the need for a comprehensive tool, rather than simple proxies, to understand the influence of cultural and linguistic diversity on cognitive assessment. In future work we will investigate the relative importance of the four CLEAr-Q factors in predicting cognitive performance, which is expected to improve diagnostic accuracy of current neuropsychological measures in assessing cognitive impairment and dementia in older adults from CALD backgrounds.

Linguistic and acculturation variables are known to influence cognitive performance on neuropsychological measures used to assess cognitive impairment and dementia. It is not clear, however, how to best measure these variables in an Australian context to improve cognitive assessment of older adults from culturally and linguistically diverse (CALD) backgrounds. We therefore developed a new measure, the Characterising Language Experience and Acculturation Questionnaire (CLEAr-Q) and used Exploratory Factor Analysis (EFA) to determine the underlying factor structure. An online version of the CLEAr-Q was developed using items drawn or modified from the literature with consultation from a CALD community working group via an adapted participatory research framework. It was administered in English via an anonymous survey. From the initial 52 items, 31 were considered for further analysis. EFA with oblique rotation (geomin) was used. The validation sample included 256 participants aged 60-93 (Table 1) born outside of Australia and reporting a native Language Other Than English (LOTE). EFA suggested a four-factor solution: Educational Exposure to English, English Proficiency and Identification with Majority Culture, Dominance of English Language and Culture, and Language Switching. The model fitted well with the data, with Comparative Fit Index (CFI) = 0.98 and Standardised Root Mean Square Residual (SRMR) = 0.07. In combination, these factors explained 58.9% of the variance. Table 2 presents factor loadings. The present study demonstrates the utility of the CLEAr- Q in characterising the diversity in linguistic and acculturation variables in an Australian CALD sample. Our results also inform item selection for a streamlined version of the CLEAr-Q . The factor structure provides evidence for the interwoven relationship between linguistic and acculturation variables and supports the need for a comprehensive tool, rather than simple proxies, to understand the influence of cultural and linguistic diversity on cognitive assessment. In future work we will investigate the relative importance of the four CLEAr-Q factors in predicting cognitive performance, which is expected to improve diagnostic accuracy of current neuropsychological measures in assessing cognitive impairment and dementia in older adults from CALD backgrounds.

Apraxia of speech is a sensorimotor speech disorder characterized by segment production errors and prosodic anomalies. Progressive apraxia of speech in the neurodegenerative disorders primary progressive aphasia and primary progressive apraxia of speech is typically associated with abnormalities in the lateral premotor cortex and supplementary motor area and their white matter substrates, and with neuropathologies in the frontotemporal lobar degeneration spectrum, especially corticobasal degeneration and progressive supranuclear palsy. Validated assessment tools are available to rate perceptual features associated with articulation, prosody, respiration, voice, and other speech behaviors. Therapeutic management of progressive apraxia of speech involves accurate diagnosis, relational-centered counseling, education and support, consideration of the suitability of behavioral restitutive interventions, introduction of compensatory strategies, and regular review to adapt care provision to the progressive course. Frontiers and challenges in the understanding of progressive apraxia of speech are discussed.

Language and cultural factors are known to influence cognitive performance on neuropsychological measures used to assess cognitive impairment and dementia. A new measure, the Characterising Language Experience and Acculturation Questionnaire (CLEAr-Q) was developed to address the gap in access to a brief measure of these factors in the Australian context. The aim is to validate and further develop the CLEAr-Q as a tool to capture linguistic and acculturation variables to improve measurement of cognition in older adults from Culturally and Linguistically Diverse (CALD) backgrounds. The CLEAr-Q was initially created as a paper questionnaire and completed by all older adults from CALD backgrounds in the CogSCAN Study. Items were drawn from the literature and adapted based on feedback from the CogSCAN group. An online version for validation with a larger sample was then developed with consultation from a CALD community working group via an adapted participatory research framework. The anonymous survey is completed online in English and data collection will conclude in February 2024. Preliminary analysis (e.g., Pearson correlation) will be conducted to check for redundant items and to select linguistic and acculturation variables (Table 1) to be included in an exploratory factor analysis; this will establish the factor structure and develop a streamlined, psychometrically validated version of the CLEAr-Q . The CogSCAN sample included 75 participants aged 60-95 who reported speaking and/or reading a language other than English (LOTE). The validation sample to date consists of 244 participants aged 60-90 who reported speaking and/or reading a LOTE at a functional level, were born in over 50 overseas countries (Figure 1) and speak on average more than two languages (Table 2). Data analysis is in progress. Results will contribute to validating the CLEAr-Q and demonstrating its utility to characterise known diversity in CALD samples. Future directions include examining the relative importance of linguistic and acculturation variables from the CLEAr-Q in predicting cognitive performance, which is expected to improve diagnostic accuracy of current neuropsychological measures for assessment of cognitive impairment and dementia in older adults from CALD backgrounds.

INTRODUCTIONInterventions to treat speech-language difficulties in primary progressive aphasia (PPA) often use word accuracy as a highly comparable outcome. However, there are more constructs of importance to people with PPA that have received less attention.METHODSFollowing Core Outcome Set Standards for Development Recommendations (COSSTAD), this study comprised: Stage 1 - systematic review to identify measures; Stage 2 - consensus groups to identify important outcome constructs for people with PPA (n = 82) and care partners (n = 91); Stage 3 - e-Delphi consensus with 57 researchers.RESULTSThe systematic review identified 84 Outcome Measurement Instruments. Core outcome constructs identified included: (1) Participate in conversations with family and friends, (2) get words out, (3) be more fluent, (4) convey a message by any means, and (5) understand what others are saying. Researchers were unable to reach a consensus on measurement instruments.DISCUSSIONFurther work is required to develop appropriate measurement instruments that address all core outcome constructs important to key stakeholders.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.

Computerised neuropsychological assessments (CNAs) are proposed as more accessible and culturally appropriate alternatives to traditional pencil-and-paper neuropsychological assessments (PnPAs) for diagnosing dementia in diverse populations. Whilst some PnPAs have demonstrated cultural and linguistic bias, research investigating the suitability of CNAs for culturally and linguistically diverse (CALD) individuals is limited, especially outside of the United States. This study compared cognitive performance in a heterogenous sample of older Australian CALD English-speakers and an L1 English-speaking background (ESB) sample. Cross-sectional data were drawn from the ongoing CogSCAN Study. Participants were 212 community-dwelling individuals aged 60-95 years without a dementia diagnosis (159 ESB/53 CALD). Participants completed a PnPA and/or CNAs, sessions were counterbalanced accordingly. CNA sessions involved two of four commercially-available CNAs in a randomised order. Differences in the level and pattern of cognitive performance between groups on two CNAs (CANTAB/Cogstate) and a comprehensive PnPA in English, controlling for sociodemographic and health factors, are reported here. Selection of key variables for examination were based on previous findings (PnPA) and test developer recommendations (CNAs). Sample characteristics are summarised in Table 1. CALD participants’ cognitive performance on individual tests (Figure 1) was largely comparable to ESB participants on PnPA and each CNA. Significant differences between groups, after Bonferroni correction for multiple comparisons, were observed for the Boston Naming Test (PnPA; a language test widely criticised for use with CALD populations due to culturally biased stimuli) and Rapid Visual Information Processing (CANTAB; a test of attention/processing speed). Among participants who completed PnPA and Cogstate, a significant interaction between group and assessment type on global cognitive performance was observed; ESB participants outperformed CALD participants on PnPA, but not on Cogstate (Figure 2). No significant main effects or interaction were observed in PnPA versus CANTAB. These results showed fewer and smaller discrepancies across groups and assessment types than has been found in previous literature with other CALD samples, perhaps reflecting higher levels of English proficiency, acculturation, and education in English than previous cohorts. Work in progress is investigating the influence of linguistic and acculturation factors on cognitive performance in a larger and more diverse sample.

A valid measure of quality of life is important for clinical goal setting and for evaluating interventions. In the amnestic dementias, proxy-raters (e.g. friends, families, clinicians) typically rate quality of life lower than the self-ratings given by the person with dementia - a proxy bias. This study investigated whether the same proxy bias occurs in Primary Progressive Aphasia (PPA), a language-led dementia.Quality of life was measured in 18 individuals with PPA using self-ratings, and proxy-ratings by their main communication partner, using the Quality of Life in Alzheimer's Disease Scale.There was no strong evidence for proxy bias at a group level, with no consistent pattern across dyads, where proxy- and self-ratings did not show good levels of agreement. We suggest that self-ratings and proxy-ratings of quality of life in PPA are not interchangeable. Higher-powered investigation of the patterns observed here is warranted in future studies.

Primary progressive aphasia (PPA) and primary progressive apraxia of speech (PPAOS) are neurodegenerative syndromes characterized by progressive decline in language or speech. There is a growing number of studies investigating speech-language interventions for PPA/PPAOS. An updated systematic evaluation of the treatment evidence is warranted to inform best clinical practice and guide future treatment research. We systematically reviewed the evidence for behavioral treatment for speech and language in this population. Reviewed articles were published in peer-reviewed journals through 31 May 2021. We evaluated level of evidence, reporting quality, and risk of bias using a modified version of the American Speech-Language Hearing Association (ASHA) Levels of Evidence, an appraisal point system, additional reporting quality and internal/external validity items, and, as appropriate, the Single Case Experimental Design Scale or the Physiotherapy Evidence Database – PsycBITE Rating Scale for Randomized and Non-Randomized Controlled Trials . Results were synthesized using quantitative summaries and narrative review. A total of 103 studies reported treatment outcomes for 626 individuals with PPA; no studies used the diagnostic label PPAOS. Most studies evaluated interventions for word retrieval. The highest-quality evidence was provided by 45 experimental and quasi-experimental studies (16 controlled group studies, 29 single-subject designs). All ( k = 45/45) reported improvement on a primary outcome measure; most reported generalization ( k = 34/43), maintenance ( k = 34/39), or social validity ( k = 17/19) of treatment for at least one participant. The available evidence supports speech-language intervention for persons with PPA; however, treatment for PPAOS awaits systematic investigation. Implications and limitations of the evidence and the review are discussed.

Objectives: Computerised neuropsychological assessments (CNAs) are proposed as an alternative method of assessing cognition to traditional pencil-and-paper assessment (PnPA), which are considered the "gold standard" for diagnosing dementia. However, limited research has been conducted with culturally and linguistically diverse (CALD) individuals. This study investigated the suitability of PnPAs and CNAs for measuring cognitive performance in a heterogenous sample of older, Australian CALD English-speakers compared to a native English-speaking background (ESB) sample. Methods: Participants were 1037 community-dwelling individuals aged 70-90 years without a dementia diagnosis from the Sydney Memory and Ageing Study (873 ESB, 164 CALD). Differences in the level and pattern of cognitive performance in the CALD group were compared to the ESB group on a newly developed CNA and a comprehensive PnPA in English, controlling for covariates. Multiple hierarchical regression was used to identify the extent to which linguistic and acculturation variables explained performance variance. Results: CALD participants' performance was consistently poorer than ESB participants on both PnPA and CNA, and more so on PnPA than CNA, controlling for socio-demographic and health factors. Linguistic and acculturation variables together explained approximately 20% and 25% of CALD performance on PnPA and CNA respectively, above demographics and self-reported computer use. Conclusions: Performances of CALD and ESB groups differed more on PnPAs than CNAs, but caution is needed in concluding that CNAs are more culturally-appropriate for assessing cognitive decline in older CALD individuals. Our findings extend current literature by confirming the influence of linguistic and acculturation variables on cognitive assessment outcomes for older CALD Australians.

Evidence of generalization to connected speech following lexical retrieval treatment in Primary Progressive Aphasia (PPA) is scarce. Consequently, this study systematically investigated changes in verb phrase production following lexical retrieval treatment in a series of single case experimental design studies. Four individuals with PPA (three semantic- and one logopenic variant PPA) who had previously demonstrated that they could integrate verbs and nouns into sentence structures in a cueing paradigm, undertook a sequence of verb and noun lexical retrieval treatments using Repetition and Reading in the Presence of a Picture. Production of treated nouns- and/or verbs-in-isolation significantly improved following treatment for three of the four participants. Verb phrase production did not improve for one of these participants (logopenic PPA), perhaps due to the relatively small treatment dose. Two participants (semantic variant PPA) did, however, demonstrate across-level generalization, with improvement in treated verbs and using those verbs in (untreated) verb phrases. Their verb phrase production improved most after lexical retrieval treatment for both nouns and verbs, suggesting this combined approach may benefit across-level generalization for some individuals in clinical practice.

INTRODUCTION:Primary progressive aphasia (PPA) is a complex language-led dementia syndrome whereby disproportionate deterioration of speech and language occurs subsequent to neurodegenerative disease in the early to mid-stages of the condition. As no effective pharmacotherapies are currently available, speech and language therapies are the optimum treatment to maximize communication for as long as possible.AREAS COVERED:The authors present an overview of current speech-language therapy practices in PPA, highlighting recent research on effective treatments.EXPERT OPINION:The challenges in this complex field of practice are described. We highlight the challenge of improving access to speech-language therapy by advocating for increased referral rates. The authors also suggest effective incorporation of innovative technologies in treatment and an enhanced evidence base for the utility of lexical retrieval treatment in improving everyday communication as challenges for the future. Finally, increased provision of PPA-specific education and support for individuals and their families is required.

There is little evidence that, for people with aphasia, successful outcomes following lexical retrieval treatment generalise beyond single word retrieval to sentence production or daily communication. This study aimed to develop greater understanding of the mechanisms of generalisation. We employed a cueing task to simulate the effects of lexical retrieval treatment. A single noun or verb was provided and the effect on production of a corresponding verb phrase examined. Sixteen individuals with primary progressive aphasia (PPA) were asked to produce verb phrases to describe action pictures accompanied by i) a verb cue where a spoken and written verb was also presented with the picture; ii) a noun cue where a spoken and written noun was presented with the picture; iii) a no cue condition where only the picture was presented. Across the case series, both verb and noun cueing improved verb phrase production relative to no cue, with verb cueing being most effective. At the level of the single case, thirteen individuals showed significantly increased production of verb phrases with verb cueing, and seven individuals with noun cueing. In addition, seven individuals showed significantly greater benefit from verb cueing compared to noun cueing, and none showed the reverse. This suggests that improvements in verb phrase production may also be achievable following treatment-induced improvements in lexical retrieval. Greater benefit from verb cues than noun cues raises important theoretical issues regarding sentence construction and clinical issues around the most effective treatment techniques for people with aphasia.

Amelioration of communication impairment in primary progressive aphasia (PPA) is an area of clinical importance and current research. Speech-language pathologists (SLPs) have a range of skills and interventions to support communication in PPA; however, underrecognition of their role and low referral rates is an ongoing concern. The E3BP conceptualization of evidence-based practice comprises 3 components: research-based evidence, practice-based evidence, and informed patient preferences. Here, we will describe how evidence for managing the communication difficulties experienced by individuals with PPA exists at all 3 levels of the (E3BP) model, highlighting how this allows SLP interventions to be both evidence based and patient centered. We encourage health professionals to value and utilize the wide range of services that SLPs can offer when working with individuals with PPA, to educate, remediate everyday linguistic skills, increase daily participation, and maximize overall quality of life.

Amelioration of communication impairment in primary progressive aphasia (PPA) is an area of clinical importance and current research. Speech-language pathologists (SLPs) have a range of skills and interventions to support communication in PPA; however, underrecognition of their role and low referral rates is an ongoing concern. The E 3 BP conceptualization of evidence-based practice comprises 3 components: research-based evidence, practice-based evidence, and informed patient preferences. Here, we will describe how evidence for managing the communication difficulties experienced by individuals with PPA exists at all 3 levels of the (E 3 BP) model, highlighting how this allows SLP interventions to be both evidence based and patient centered. We encourage health professionals to value and utilize the wide range of services that SLPs can offer when working with individuals with PPA, to educate, remediate everyday linguistic skills, increase daily participation, and maximize overall quality of life. Keywords primary progressive aphasia , evidence-based practice , evidence-based medicine , speech and language pathology , communication

OBJECTIVES:To evaluate the benefits of a primary progressive aphasia (PPA) education and support session for people with PPA (pwPPA) and their caregivers. METHOD:Thirty-eight individuals (20 pwPPA, 18 caregivers) were invited to participate in the study. Twenty-five individuals (12 pwPPA, 13 caregivers) completed questionnaires before and after an education and support group session provided by a speech pathologist and a clinical psychologist. Seven individuals (2 pwPPA, 5 caregivers) participated in follow-up interviews. RESULTS:After one attendance, caregivers reported significant improvement in knowledge of PPA, strategies to manage worry and low mood, and opportunities to meet peers. Themes at interview were reduced feelings of isolation, increased feelings of support, increased knowledge of coping strategies, and improved understanding of PPA. Caregivers who had attended previous sessions reported increased feelings of well-being and support. IMPLICATIONS:Primary progressive aphasia education and support group sessions in the postdiagnostic period constitute a valuable component of comprehensive care for PPA.

Computer‐administered neuropsychological assessment batteries (CNAs) have the potential to allow large‐scale cognitive screening and monitoring, increasing older adults’ access to cognitive assessment, and earlier diagnosis of and intervention for cognitive impairment. There is, however, little research on whether experience with and attitudes to computers and technology affect the validity, reliability and acceptability of CNAs in older adults. Here, we report the development and validation of an instrument to measure computer attitudes and computer experience in an older adult cohort from the CogSCAN study.

Word-finding difficulty is typically an early and frustrating symptom of primary progressive aphasia (PPA), prompting investigations of lexical retrieval treatment in PPA. This study aimed to investigate immediate treatment gains following two versus four weeks of treatment, item generalisation, and maintenance of gains with ongoing treatment in a single case series of eight individuals with heterogeneous PPA presentations (three non-fluent/agrammatic, two logopenic, two semantic, and one mixed PPA). Three individuals made initial gains in picture naming and maintained them over 6 months or more with ongoing treatment. By contrast, three individuals made marginal initial gains but were unable to continue treatment, and two individuals did not make the typically-reported initial gains with two or four weeks of treatment. There was little evidence of generalisation to untreated items. Our results add to the evidence that daily home practice of Repetition and Reading in the Presence of a Picture over extended periods can increase and maintain retrieval of personally-relevant words in picture naming for some individuals with semantic or nonfluent/agrammatic variant PPA. Further research is needed into the factors associated with long-term treatment adherence and gains, and the factors associated with nonadherence to treatment.

Event Abstract Back to Event Perspectives on Living Positively with Primary Progressive Aphasia Leanne Ruggero1*, Lyndsey A. Nickels1 and Karen Croot2 1 Macquarie University, Australia 2 University of Sydney, Australia Individuals with primary progressive aphasia (PPA) are living with the realities of aphasia and other communication difficulties, with the additional knowledge that they have incurable neurological disease. As awareness of the communicative challenges and behavioural treatment options for PPA increases, so should our understanding of the personal impacts of living with the condition. Despite the devastating diagnosis, some individuals with PPA are, anecdotally, inclined to be positive about their situation and engage in more positive behaviours and activities than others, however, to date no studies have empirically investigated this issue. Similar conclusions have been drawn for related but distinct populations such as Alzheimer’s or post-stroke aphasia (Brown et al, 2010; Wolverson et al., 2016). The aim of this study was therefore to use interview and qualitative analysis to explore the lived experience of PPA and self-reported determinants of ‘living positively’ with the condition. Ten individuals with PPA participated in in-depth, semi-structured interviews about living positively with PPA, lasting approximately one hour. They were between 14 to 48 months post symptom-onset at the time of interview. Interview topics and questions closely mirrored those described by Grohn et al. (2012) for exploring positive living in stroke-aphasia, and covered topics around living positively with PPA and what factors the participants believed led to this. Transcripts were analysed using thematic analysis (Braun & Clarke, 2006) and an inductive approach. Interview content focussed on communication; “It takes away, for me, um, nouns, I have difficulty with nouns, spelling and maths and they were probably my – were all my life the best subjects”, the challenges of living with PPA; “There was [after diagnosis] a – a really close army friend who just dropped off really which was very sad”, and, how to remain positive despite these; “But, um, and the other thing is that what I ponder on occasionally is that, um, it could have been very much worse”. Thematic analysis is ongoing and themes identified in the data with accompanying statements from individuals with PPA will be presented. Early results indicate that the ability to live positively with PPA may be related to: education about PPA, social, environmental and professional support, meeting others with PPA, philosophical perspective and keeping busy: “I definitely had a positive outlook on it… and a determination to be busy”. The results from this study will increase awareness of the experience of PPA, and through triangulation with quantitative data on Quality of Life will allow greater understanding of the lived experience of PPA. This will enable health professionals to manage PPA in a holistic way, designing services which can promote positive living and quality of life. Acknowledgements This abstract is part of the symposium “Aphasia in neurodegenerative conditions.” References Braun, V., & Clarke, V. (2006). Using thematic analysis in psychology. Qualitative Research in Psychology, 3(2), 77–101. https://doi.org/10.1191/1478088706qp063oa Brown, K., Worrall, L. E., Davidson, B., & Howe, T. (2010). Snapshots of success: An insider perspective on living successfully with aphasia. Aphasiology, 24(10), 1267–1295. https://doi.org/10.1080/02687031003755429 Grohn, B., Worrall, L. E., Simmons-Mackie, N., & Brown, K. (2012). The first 3-months post-stroke: What facilitates successfully living with aphasia? International Journal of Speech-Language Pathology, 14(4), 390–400. https://doi.org/10.3109/17549507.2012.692813 Wolverson, E. L., Clarke, C., & Moniz-Cook, E. D. (2016). Living positively with dementia: a systematic review and synthesis of the qualitative literature. Aging & Mental Health, 20(7), 676–699. https://doi.org/10.1080/13607863.2015.1052777 Keywords: Progressive aphasia, Quality of Life, Dementia, Aphasia, Lived experience Conference: Academy of Aphasia 57th Annual Meeting, Macau, Macao, SAR China, 27 Oct - 29 Oct, 2019. Presentation Type: Symposium Topic: Eligible for student award Citation: Ruggero L, Nickels LA and Croot K (2019). Perspectives on Living Positively with Primary Progressive Aphasia. Front. Hum. Neurosci. Conference Abstract: Academy of Aphasia 57th Annual Meeting. doi: 10.3389/conf.fnhum.2019.01.00043 Copyright: The abstracts in this collection have not been subject to any Frontiers peer review or checks, and are not endorsed by Frontiers. They are made available through the Frontiers publishing platform as a service to conference organizers and presenters. The copyright in the individual abstracts is owned by the author of each abstract or his/her employer unless otherwise stated. Each abstract, as well as the collection of abstracts, are published under a Creative Commons CC-BY 4.0 (attribution) licence (https://creativecommons.org/licenses/by/4.0/) and may thus be reproduced, translated, adapted and be the subject of derivative works provided the authors and Frontiers are attributed. For Frontiers’ terms and conditions please see https://www.frontiersin.org/legal/terms-and-conditions. Received: 03 May 2019; Published Online: 09 Oct 2019. * Correspondence: Ms. Leanne Ruggero, Macquarie University, Sydney, Australia, leanne.ruggero@students.mq.edu.au Login Required This action requires you to be registered with Frontiers and logged in. To register or login click here. Abstract Info Abstract The Authors in Frontiers Leanne Ruggero Lyndsey A Nickels Karen Croot Google Leanne Ruggero Lyndsey A Nickels Karen Croot Google Scholar Leanne Ruggero Lyndsey A Nickels Karen Croot PubMed Leanne Ruggero Lyndsey A Nickels Karen Croot Related Article in Frontiers Google Scholar PubMed Abstract Close Back to top Javascript is disabled. Please enable Javascript in your browser settings in order to see all the content on this page.

Background: A quality of life approach to managing communication and neurodegenerative conditions is increasingly encouraged by professional bodies and healthcare models. Clinical services for primary progressive aphasia (PPA) continue to expand and the research base continues to grow; however, there is little research specifically investigating quality of life in PPA. Aims: This critical review aimed to determine the available knowledge about quality of life in PPA and gaps in that knowledge, and to consider how PPA researchers might best address those gaps. Main contribution: Two unpublished single case series studies and one published case study were identified that directly investigated aspects of quality of life in PPA. These suggested that quality of life is heterogenous and determined by a range of factors, supporting a person-centred approach to care. The current literature also includes five accounts of living with PPA authored by individuals with PPA or their carers, discussing factors that influenced these individuals' quality of life. Seventeen empirical investigations of mood were identified, suggesting that approximately 40% of individuals with PPA develop depression at some point. Finally, several recent studies have noted the importance of promoting quality of life in PPA and/or have utilised quality of life outcome measures; however, most lack a clear conceptualisation of the construct of quality of life, and how this construct is related to the instruments and methods chosen to measure it. Conclusions: Literature on quality of life in PPA is beginning to emerge, although little is available in indexed databases. More research is needed that better conceptualises the construct of quality of life, critically appraises the way quality of life is assessed in PPA, builds on findings from the current small samples, and involves individuals with PPA in formulating research directions and research tools that will enable discussion about their quality of life.