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.

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.

Symptom-led staging for primary progressive aphasia.

The primary progressive aphasias (PPA) present complex and diverse challenges of diagnosis, management and prognosis. A clinically-informed, syndromic staging system for PPA would take a substantial step toward meeting these challenges. This study addressed this need using detailed, multi-domain mixed-methods symptom surveys of people with lived experience in a large international PPA cohort. We administered structured online surveys to caregivers of patients with a canonical PPA syndromic variant (nonfluent/agrammatic (nvPPA), semantic (svPPA) or logopenic (lvPPA)). In an 'exploratory' survey, a putative list and ordering of verbal communication and nonverbal functioning (nonverbal thinking, conduct and wellbeing, physical) symptoms was administered to 118 caregiver members of the UK national PPA Support Group. Based on feedback, we expanded the symptom list and created six provisional clinical stages for each PPA subtype. In a 'consolidation' survey, these stages were presented to 110 caregiver members of UK and Australian PPA Support Groups, and refined based on quantitative and qualitative feedback. Symptoms were retained if rated as 'present' by a majority (at least 50%) of respondents representing that PPA syndrome, and assigned to a consolidated stage based on majority consensus; the confidence of assignment was estimated for each symptom as the proportion of respondents in agreement with the final staging for that symptom. Qualitative responses were analysed using framework analysis. For each PPA syndrome, six stages ranging from 1 ('Very mild') to 6 ('Profound') were identified; earliest stages were distinguished by syndromic hallmark symptoms of communication dysfunction, with increasing trans-syndromic convergence and dependency for basic activities of daily living at later stages. Spelling errors, hearing changes and nonverbal behavioural features were reported at early stages in all syndromes. As the illness evolved, swallowing and mobility problems were reported earlier in nfvPPA than other syndromes, while difficulty recognising familiar people and household items characterised svPPA and visuospatial symptoms were more prominent in lvPPA. Overall confidence of symptom staging was higher for svPPA than other syndromes. Across syndromes, functional milestones were identified as key deficits that predict the sequence of major daily life impacts and associated management needs. Qualitatively, we identified five major themes encompassing 15 subthemes capturing respondents' experiences of PPA and suggestions for staging implementation. This work introduces a prototypical, symptom-led staging scheme for canonical PPA syndromes: the PPA Progression Planning Aid (PPA 2 ). Our findings have implications for diagnostic and care pathway guidelines, trial design and personalised prognosis and treatment for people living with these diseases.

Evaluating Implementation and Outcomes of a Person-Centered Care Model for People with Dementia in the Rehabilitation In-Patient Setting: Project Protocol.

BACKGROUND: While Australian guidelines promote person-centered healthcare (PCC) for persons with dementia, healthcare systems, routines, rules, and workplace cultures can pose challenges in the provision of PCC. OBJECTIVE: To present a knowledge translation protocol of the PCC model in a sub-acute rehabilitation hospital. METHODS: The two-year pre/post/follow-up translation project will include (n = 80) persons with dementia, (n = 80) adult family/carers of patient participants, (n = 60) healthcare staff (medical, nursing, allied health), and (n = 8) PCC staff champions. Champions will complete six half-days' training in PCC. Medical, nursing, and allied health staff will be provided with PCC learning manuals, complete six hours of online PCC education and attend six face-to-face PCC education sessions. Champions will provide ongoing support to staff in PCC practice. The Reach, Effectiveness, Adoption, Implementation, and Maintenance (RE-AIM) framework will be used to evaluate: i) outcomes for prospective patients provided with PCC, compared with a matched sample of retrospective patients (primary outcomes agitation incidence and severity); 2) champion and staff PCC knowledge, confidence, engagement, and practice quality; 3) person, family/carer, champion, and staff satisfaction with PCC; 4) PCC costs and benefits; and 5) organizational structures, systems and policies required to implement and maintain PCC in sub-acute healthcare. RESULTS: We will identify if PCC benefits persons with dementia, staff, and healthcare services, and we will generate evidence on the educational and organizational resources required to embed PCC in practice. CONCLUSION: Project findings will inform tailored PCC education applications for dissemination in healthcare and produce evidence-based PCC practice guidelines to improve healthcare for persons with dementia.

Exploring the effects of verb and noun treatment on verb phrase production in primary progressive aphasia: A series of single case experimental design studies.

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.

Semantic Variant Primary Progressive Aphasia: Practical Recommendations for Treatment from 20 Years of Behavioural Research.

People with semantic variant primary progressive aphasia (svPPA) present with a characteristic progressive breakdown of semantic knowledge. There are currently no pharmacological interventions to cure or slow svPPA, but promising behavioural approaches are increasingly reported. This article offers an overview of the last two decades of research into interventions to support language in people with svPPA including recommendations for clinical practice and future research based on the best available evidence. We offer a lay summary in English, Spanish and French for education and dissemination purposes. This paper discusses the implications of right- versus left-predominant atrophy in svPPA, which naming therapies offer the best outcomes and how to capitalise on preserved long-term memory systems. Current knowledge regarding the maintenance and generalisation of language therapy gains is described in detail along with the development of compensatory approaches and educational and support group programmes. It is concluded that there is evidence to support an integrative framework of treatment and care as best practice for svPPA. Such an approach should combine rehabilitation interventions addressing the language impairment, compensatory approaches to support activities of daily living and provision of education and support within the context of dementia.

Verbal Short-Term Memory Disturbance in the Primary Progressive Aphasias: Challenges and Distinctions in a Clinical Setting.

Impaired verbal 'phonological' short-term memory is considered a cardinal feature of the logopenic variant of primary progressive aphasia (lv-PPA) and is assumed to underpin most of the language deficits in this syndrome. Clinically, examination of verbal short-term memory in individuals presenting with PPA is common practice and serves two objectives: (i) to help understand the possible mechanisms underlying the patient's language profile and (ii) to help differentiate lv-PPA from other PPA variants or from other dementia syndromes. Distinction between lv-PPA and the non-fluent variant of PPA (nfv-PPA), however, can be especially challenging due to overlapping language profiles and comparable psychometric performances on verbal short-term memory tests. Here, we present case vignettes of the three PPA variants (lv-PPA, nfv-PPA, and the semantic variant (sv-PPA)) and typical Alzheimer's disease (AD). These vignettes provide a detailed description of the short-term and working memory profiles typically found in these patients and highlight how speech output and language comprehension deficits across the PPA variants differentially interfere with verbal memory performance. We demonstrate that a combination of verbal short-term and working memory measures provides crucial information regarding the cognitive mechanisms underlying language disturbances in PPA. In addition, we propose that analogous visuospatial span tasks are essential for the assessment of PPA as they measure memory capacity without language contamination.

Is word learning enough? Improved verb phrase production following cueing of verbs and nouns in primary progressive aphasia.

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.

Speech and language therapy in primary progressive aphasia: a critical review of current 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.

Primary Progressive Aphasia Education and Support Groups: A Clinical Evaluation.

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.

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.

Treatment for spoken and written word retrieval in the semantic variant of primary progressive aphasia.

This study investigated whether a treatment programme for spoken word retrieval, supplemented with written naming, was beneficial for an individual with right-hemisphere dominant semantic variant of PPA (svPPA). Assessment and treatment were delivered remotely through Skype. Treatment consisted of two phases of lexical retrieval therapy (Repetition and Reading in the Presence of a Picture: RRIPP), with and without written responses (Phases 1 and 2 respectively), and a third treatment phase based on the procedures of Conceptual Enrichment (COEN) therapy. The first two phases of treatment resulted in short-lasting improvements in spoken and written word retrieval, with greater improvement in Phase 2 when written production was also required. Both treatment phases resulted in gains only for treated items, but generalised to different depictions to those treated. However, Phase 2 also resulted in significant improvement of treated items on a comprehension task. COEN treatment did not result in significant gains in word retrieval or comprehension. This study reinforces the value of a simple lexical retrieval treatment delivered remotely. It adds to the current evidence that anomia in svPPA can be responsive to treatment, but also shows that challenges remain regarding maintenance effects and the generalisation of treatment effects to connected speech.

Lexical retrieval treatment in primary progressive aphasia: An investigation of treatment duration in a heterogeneous case series.

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.

Communication behaviors associated with successful conversation in semantic variant primary progressive aphasia.

BACKGROUND: Primary progressive aphasia (PPA) affects a range of language and cognitive domains that impact on conversation. Little is known about conversation breakdown in the semantic variant of PPA (svPPA, also known as semantic dementia). This study investigates conversation of people with svPPA. METHODS: Dyadic conversations about everyday activities between seven individuals with svPPA and their partners, and seven control pairs were video recorded and transcribed. Number of words, turns, and length of turns were measured. Trouble-indicating behaviors (TIBs) and repair behaviors were categorized and identified as successful or not for each participant in each dyad. RESULTS: In general, individuals with svPPA were active participants in conversation, taking an equal proportion of turns, but indicating a great deal of more trouble in conversation, shown by the significantly higher number of TIBs than evidenced by partners or control participants. TIBs were interactive (asking for confirmation with a shorter repetition of the original utterance or a repetition which included a request for specific information) and non-interactive (such as failing to take up or continue the topic or a minimal response) and unlike those previously reported for people with other PPA variants and dementia of the Alzheimer type. Communication behaviors of the partner were critical to conversational success. CONCLUSIONS: Examination of trouble and repair in 10-min conversations of individuals with svPPA and their important communication partners has potential to inform speech pathology interventions to enhance successful conversation, in svPPA and should be an integral part of the comprehensive care plan.