Behavioral Treatment for Speech and Language in Primary Progressive Aphasia and Primary Progressive Apraxia of Speech: A Systematic Review.

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.

Quality of Life Ratings and Proxy Bias in Primary Progressive Aphasia: Two Sides to the Story?

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.

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.

Comparison of Computerised and Pencil-and-Paper Neuropsychological Assessments in Older Culturally and Linguistically Diverse Australians.

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.

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.

How Evidence-Based Practice (E3BP) Informs Speech-Language Pathology for Primary Progressive 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.

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.

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.

Segmental speech error data elicited at prosodically-defined locations in tongue twisters.

Tongue twister error data described here were collected from 44 unimpaired speakers producing six repetitions each of 40 tongue-twisters that manipulated the position of error-prone segments within two prosodic domains: intonational phrases and utterances. Data are counts of perseveration and anticipation errors on the initial segments of phrase-initial words or phrase-final words. The location of the phrase-level prominence and other factors associated with speech errors were carefully controlled. For more details about the design, materials and methods, and for interpretation and discussion, see Beirne and Croot (2018) [1].

Treatment for Lexical Retrieval Impairments in Primary Progressive Aphasia: A Research Update with Implications for Clinical Practice.

Lexical retrieval impairments (also known as anomia or word-finding deficits) are an early and prominent symptom in primary progressive aphasia (PPA), causing distress and frustration to individuals with PPA and their communication partners, and prompting research on lexical retrieval treatment. This paper reviews the research on lexical retrieval treatment in PPA from the earliest reports in the 1990s to early 2018 and considers the implications of this research for clinical practice. The number of published studies has increased markedly over the past decade, consisting primarily of behavioral studies, with rapid recent growth in noninvasive brain stimulation studies. Five general treatment techniques were identified in the behavioral studies, described here as standard naming treatment, Look, Listen, Repeat treatment, cueing hierarchies, semantically focused treatments, and lexical retrieval in context. Across techniques, behavioral studies targeting difficult-to-retrieve items typically report immediate gains, and there is evidence these gains can be maintained over months to years by some participants who continue with long-term treatment. There is also evidence that prophylactic treatment supports retrieval of treated items compared with untreated items. There is limited evidence for generalization of treatment to untreated items, suggesting the primary aim of lexical retrieval treatment in this population is to maintain retrieval of a core vocabulary for as long as possible. Language and cognitive assessment and piloting of the intended treatment can inform decisions about treatment selection and participant suitability for long-term lexical retrieval treatment. The paper concludes with some questions to guide clinical decision making about whether to implement or continue with a behavioral lexical retrieval treatment.

The prosodic domain of phonological encoding: Evidence from speech errors.

Phonological encoding of segments is thought to occur within a prosodically-defined frame, but it is not clear which of the constituent/s within the prosodic hierarchy (syllables, phonological words, intonational phrases and utterances) serve/s as the domain of phonological encoding. This experiment investigated whether segmental speech errors elicited in tongue-twisters were influenced by position within prosodic constituents above the level of the phonological word. Forty-four participants produced six repetitions each of 40 two-intonational phrase tongue-twisters with error-prone word-initial "target" segments in phrase-initial and phrase-final words. If the domain of phonological encoding is the intonational phrase, we hypothesised that segments within a current intonational phrase would interact in more errors than would segments across intonational phrase boundaries. Participants made more anticipatory than perseveratory errors on target segments in phrase-initial words as predicted. They also made more perseveratory than anticipatory errors on targets in phrase-final words, but only in utterance-final phrases. These results suggest that the intonational phrase is one domain of phonological encoding, and that segments for upcoming phrases are activated while current phrases are being articulated.

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.

Predictive Factors for the Uptake of Coping Strategies by Spousal Dementia Caregivers: A Systematic Review.

An understanding of spousal dementia caregivers' coping strategies and their predictive factors is imperative for caregivers' well-being. Although several reviews have explored the relationship between coping strategies and outcomes, no review has investigated factors that predict caregivers' use of one type of coping strategy over another. The current review aimed to identify factors that predict caregivers' coping strategies. Within this, we attempted to identify caregivers who are more likely to adopt dysfunctional coping strategies and be at risk of adverse outcomes. Several electronic databases were systematically searched. Twenty-one studies were eligible for review, describing 18 caregiver and care-recipient factors related to the 3 coping strategies. No factors were classified "predictive," however, 16 factors were "potentially predictive." Younger, more highly educated caregivers with greater emotional supports and knowledge of dementia were associated with solution-focused coping. Younger, less educated caregivers were associated with emotional support/acceptance-based coping strategies. Whereas nonwhite caregivers with less emotional supports caregiving for persons with more behavioral problems were associated with dysfunctional coping strategies. Enhancing caregiver self-efficacy, knowledge of dementia, improving social supports, linking to support groups, managing behavioral problems, as well as coaching adaptive coping strategies while flagging caregivers at risk for dysfunctional coping may improve outcomes for caregivers.

The Effect of Blocked, Random and Mixed Practice Schedules on Speech Motor Learning of Tongue Twisters in Unimpaired Speakers.

There are few investigations comparing practice schedules in speech motor learning, despite certain schedules being recommended for the clinical treatment of speech motor disorders. This study compared effects of random, blocked and mixed practice on tongue twister accuracy in unimpaired speakers. We hypothesized that blocked practice would benefit acquisition of learning, but that random practice and mixed blocked-then-random practice would yield superior retention of learning. We found that the random and blocked-random practice schedules yielded superior accuracy at the end of the acquisition phase of learning and at a 1-week retention test. Exploratory post hoc analyses raised the possibility that the retention effects were most evident when tongue twisters were elicited in a random schedule. Theoretical accounts of these results are discussed.

The Role of Coping Strategies in Psychological Outcomes for Frontotemporal Dementia Caregivers.

BACKGROUND: Caregiving for a person with frontotemporal dementia (FTD) is related to poor caregiver outcomes. Coping strategies adopted by caregivers are known to influence psychological outcomes in other dementia caregiver populations, however, their influence on psychological outcomes in FTD caregivers is poorly understood at present. METHODS: Questionnaire data for 94 German primary caregivers (mean [M] 59.11 years, 68 females) of FTD care-recipients living in the community (M 63.94, 30 females) were investigated. Standardized measures completed by the caregiver included the Caregiver Strain Index (CSI), Beck Depression Inventory II (BDI-II), Quality of Life-Alzheimer's Disease (QoL-AD), and the Brief Coping Orientations to Problems Experienced (COPE). Care-recipients' nursing care level was collected as a measure of the intensity of their care needs. RESULTS: Mediation analyses showed that the effect of the intensity of care-recipients' care needs on caregiver well-being depended on caregivers' experience of strain. High levels of caregiver strain did not predict depression (-0.22, 95% confidence interval CI: [0.16 to 2.04]) but predicted reduced QoL (-0.44, CI: [-1.15 to -.16]). Moreover, caregivers' experience of strain was exacerbated by their use of dysfunctional coping (ß = .21; p = .04), care-recipients' intensity of care needs (ß = .25; p = .01), and fewer financial resources (ß = .23; p = .02). In turn, caregivers' use of dysfunctional coping as a response to their strain increased the levels of depression (0.46, CI: [0.19-0.82]). By contrast, use of problem-focused coping strategies increased caregivers' QoL (0.10, CI: [0.00 to 0.31]). SIGNIFICANCE: This study identifies variables amenable to clinical interventions that can improve caregivers' well-being: specifically, caregiver strain and coping strategies. For a disease without cure yet increasing prevalence and cost, ameliorating the caregiver experience through targeted interventions is essential.

Phonological Encoding in Mandarin Chinese: Evidence from Tongue Twisters.

Models of connected speech production in Mandarin Chinese must specify how lexical tone, speech segments, and phrase-level prosody are integrated in speech production. This study used tongue twisters to test predictions of the two different models of word form encoding. Tongue twisters were constructed from 5 sets of characters that rotated pairs of initial segments or pairs of tones, or both, across format (ABAB, ABBA), and across position of the characters in four-character tongue twister strings. Fifty two native Mandarin Chinese speakers read aloud 120 tongue twisters, repeating each one six times in a row. They made a total of 3503 (2.34%) segment errors and 1372 (.92%) tone errors. Segment errors occurred on the onsets of the first and third characters in the ABBA but not ABAB segment-alternating tongue twisters, and on the onsets of the second and fourth characters of the tone-alternating tongue twisters. Tone errors were highest on the third and fourth characters in the tone-alternating tongue twisters. The pattern of tone errors is consistent with the claim that tone is associated to a metrical frame prior to segment encoding, while the format by position interaction found for the segment-alternating tongue twisters suggest articulatory gestures oscillate in segment production as proposed by gestural phonology.