Primary progressive aphasia: six questions in search of an answer

Abstract

Here, we review recent progress in the diagnosis and management of primary progressive aphasia—the language-led dementias. We pose six key unanswered questions that challenge current assumptions and highlight the unresolved difficulties that surround these diseases. How many syndromes of primary progressive aphasia are there—and is syndromic diagnosis even useful? Are these truly ‘language-led’ dementias? How can we diagnose (and track) primary progressive aphasia better? Can brain pathology be predicted in these diseases? What is their core pathophysiology? In addition, how can primary progressive aphasia best be treated? We propose that pathophysiological mechanisms linking proteinopathies to phenotypes may help resolve the clinical complexity of primary progressive aphasia, and may suggest novel diagnostic tools and markers and guide the deployment of effective therapies.

COS-PPA: protocol to develop a core outcome set for primary progressive aphasia

“…19 We have no roadmap at present for determining or evaluating intervention outcomes in PPA and it presents radically different challenges to stroke aphasia (the current standard for aphasia interventions)-both due to its intrinsically progressive nature and also because it entails significant issues with non-verbal cognition and behaviour over the course of the illness that interacts with communication functionthus, there is a fundamental need to reorient researchers and clinicians to PPA. [18][19][20] In summary, there is a need for a specific COS, that details key measures addressing the needs of people with PPA.…”

Primary progressive aphasia in Italian and English: a cross-linguistic cohort study

“…Primary progressive aphasia (PPA) designates a diverse group of neurodegenerative disorders led by insidious deterioration of language skills 1 . Current consensus diagnostic criteria for PPA enshrine three canonical syndromic variants based on specific profiles of linguistic impairments 2 : nonfluent/agrammatic variant PPA (nfvPPA), semantic variant PPA (svPPA), and logopenic variant PPA (lvPPA).…”

“…Current consensus diagnostic criteria for PPA enshrine three canonical syndromic variants based on specific profiles of linguistic impairments 2 : nonfluent/agrammatic variant PPA (nfvPPA), semantic variant PPA (svPPA), and logopenic variant PPA (lvPPA). Diagnostic formulations and our wider understanding of PPA have largely been shaped by studies involving native English speakers [1][2][3] . This is a serious limitation: languages vary widely in their linguistic, articulatory, acoustic and orthographic characteristics, and neurodegenerative pathologies targeting brain language networks are unlikely a priori to manifest uniformly across languages.…”

The Functional Communication Checklist for People Living with Primary Progressive Aphasia: Study Protocol

“…Fifty thousand Americans are currently estimated to be living with primary progressive aphasia (PPA) [1], a clinical syndrome that initially presents with focal language decline and is typically attributable to pathological findings consistent with frontotemporal degeneration or Alzheimer's disease (AD) [2][3][4][5]. People living with PPA (PwPPA) experience progressive decline in focal aspects of speech, language, and communication in the mild to moderate stages [2][3][4][5]. To date, there are three PPA variants established in the literature: the nonfluent/agrammatic, semantic, and logopenic with differentiated syndromic characteristics (see Table 1) [2][3][4][5].…”

“…People living with PPA (PwPPA) experience progressive decline in focal aspects of speech, language, and communication in the mild to moderate stages [2][3][4][5]. To date, there are three PPA variants established in the literature: the nonfluent/agrammatic, semantic, and logopenic with differentiated syndromic characteristics (see Table 1) [2][3][4][5].…”