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.

Muddles and puzzles: Metaphor use associated with disease progression in Primary Progressive Aphasia.

Background: Primary Progressive Aphasia describes a language-led dementia and its variants. There is little research exploring the experiences of living with this disease. Metaphor, words that represent something else, have been studied extensively in health-related narratives to gain a more intimate insight into health experiences. Aims: This study explored the metaphors used spontaneously by people with PPA, their care partners (family), and speech and language therapists/pathologists (SLT/Ps) providing support along the continuum of care. Methods & Procedures: This study examined two previously collected data sets comprising naturalistic talk where metaphors were not the specific focus, the first from focus groups conducted with people with PPA and their families and the second from focus groups conducted with SLT/Ps working with people with PPA. Transcribed data were analysed for metaphor use through an iterative narrative approach. Outcomes & Results: In all, 237 examples of metaphorical language were identified in the data, with 14 metaphors from people with PPA, 116 from the families and 106 from SLT/Ps. Different metaphors were used by participants to describe their experiences depending on which variant of PPA they were living with, and people also described their disease differently over time. SLT/Ps also used metaphors, however, their language reflected the structured, professional perspective of delivering speech and language therapy services. Conclusions & Implications: SLT/Ps should listen for and recognise the metaphorical language used by people with PPA and their families to ensure therapeutic alignment, see beyond the PPA to recognise the individual's needs, and provide person-centred and empathic support.

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.

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.

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.

Improving working memory in children with low language abilities.

This study investigated whether working memory training is effective in enhancing verbal memory in children with low language abilities (LLA). Cogmed Working Memory Training was completed by a community sample of children aged 8-11 years with LLA and a comparison group with matched non-verbal abilities and age-typical language performance. Short-term memory (STM), working memory, language, and IQ were assessed before and after training. Significant and equivalent post-training gains were found in visuo-spatial short-term memory in both groups. Exploratory analyses across the sample established that low verbal IQ scores were strongly and highly specifically associated with greater gains in verbal STM, and that children with higher verbal IQs made greater gains in visuo-spatial short-term memory following training. This provides preliminary evidence that intensive working memory training may be effective for enhancing the weakest aspects of STM in children with low verbal abilities, and may also be of value in developing compensatory strategies.

Paediatric speech-language pathology service delivery: an exploratory survey of Australian parents.

Consideration of client values and preferences for service delivery is integral to engaging with the evidence-based practice triangle (E(3)BP), but as yet such preferences are under-researched. This exploratory study canvassed paediatric speech-language pathology services around Australia through an online survey of parents and compared reported service delivery to preferences, satisfaction, and external research evidence on recommended service delivery. Respondents were 154 parents with 192 children, living across a range of Australian locations and socio-economic status areas. Children had a range of speech and language disorders. A quarter of children waited over 6 months to receive initial assessment. Reported session type, frequency, and length were incongruent with both research recommendations and parents' wishes. Sixty per cent of parents were happy or very happy with their experiences, while 27% were unhappy. Qualitative responses revealed concerns such as; a lack of available, frequent, or local services, long waiting times, cut-off ages for eligibility, discharge processes, and an inability to afford private services. These findings challenge the profession to actively engage with E(3)BP including; being cognisant of evidence-based service delivery literature, keeping clients informed of service delivery policies, individualizing services, and exploring alternative service delivery methods.