Abstract
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.
Highlights
In the 1970s, Warrington’s description of three individuals with a selective and profound inability to name and recognise objects [1] laid the foundation for what years later, in 1989, would be coined “semantic dementia” [2]
In up to 91% of cases with right-semantic variant primary progressive aphasia (svPPA), the clinical picture is characterised by prosopagnosia that for these individuals is associated with person-specific semantic knowledge breakdown [42,45,46,47,48,49]
The generalisation of benefits in svPPA has been usually evaluated in two ways: (1) whether naming improvements extend from trained to untrained words and (2) whether words can be used by the person with svPPA in contexts that differ from the training format
Summary
In the 1970s, Warrington’s description of three individuals with a selective and profound inability to name and recognise objects [1] laid the foundation for what years later, in 1989, would be coined “semantic dementia” [2]. Therapeutic approaches targeting conversation [24], tasks and activities of daily living [25,26,27], psychoeducation programmes [28,29] and peer support groups [30] have made headway and are on the increase. They have set the stage for an integrative framework of clinical treatment and care in svPPA that combines rehabilitation interventions, compensatory approaches and provision of education and support, addressing the language impairment in svPPA within the context of dementia [31]. For the purposes of education and dissemination beyond an academic audience, this article includes a lay summary available in English, Spanish and French (Supplementary Materials S1–S3)
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