ArticleAlzheimer's & dementia : the journal of the Alzheimer's Association2024
Symptom-led staging for semantic and non-fluent/agrammatic variants of primary progressive aphasia.
Article in Alzheimer's & dementia : the journal of the Alzheimer's Association, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 36 papers, 2 of them syntheses that pooled it.
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36 citing papers in PubMed, 2 syntheses or guidelines pooled it, 48 citations in OpenAlex.
- Neuroimaging Meta-Analyses Reveal Convergence of Interoception, Emotion, and Social Cognition Across Neurodegenerative Diseases.Biological psychiatry · 2025Pooled it
- An international core outcome set for primary progressive aphasia (COS-PPA): Consensus-based recommendations for communication interventions across research and clinical settings.Alzheimer's & dementia : the journal of the Alzheimer's Association · 2025Pooled it
- Care Partner Perceptions of Conversation Difficulties, Strategy Use, and Psychosocial Impacts in Primary Progressive Aphasia: A Descriptive Study Using the PCI-DAT.Brain sciences · 2026Article
- Speech-In-Noise Perception in Alzheimer's Disease and Primary Progressive Aphasia.European journal of neurology · 2026Article
- Staging Language-Led Neurodegenerative Diseases in a Language-Based World: The Challenge of the PPA-Squared Scale.European journal of neurology · 2026Article
- What Is in a Label? The Importance of Clinical Specificity and Challenges of Diagnostic Evolution in Progressive Speech-Language Disorders.American journal of speech-language pathology · 2026Article
- 'I Am No Longer Anxious When I Speak': Experiences of People with Primary Progressive Aphasia Taking Part in a Biographic-Narrative Therapy (Brain sciences · 2026Article
- Functional neuroanatomy of musical object processing in Alzheimer's disease and frontotemporal dementia.Brain communications · 2026Article
- Applicability and Correlates of a Symptom-Led Staging System for Primary Progressive Aphasia.European journal of neurology · 2025Article
- Exploring Language Impairment in Catalan-Dominant Bilinguals with Primary Progressive Aphasia: Preliminary Data.Brain sciences · 2025Article
- Article
- Longitudinal evaluation of common and unique brain-networks in variants of primary progressive aphasia.Alzheimer's research & therapy · 2025Article
- Picture Description and Functional Communication Rating Correlates in Variants of Primary Progressive Aphasia.Aphasiology · 2025Article
- Hearing impairment and dementia: cause, catalyst or consequence?Journal of neurology · 2025Review
- Safety and Efficacy of Different Therapeutic Interventions for Primary Progressive Aphasia: A Systematic Review.Journal of clinical medicine · 2025Review
- Data-driven neuroanatomical subtypes of primary progressive aphasia.Brain : a journal of neurology · 2025Article
- The graded multidimensional geometry of phenotypic variation and progression in neurodegenerative syndromes.Brain : a journal of neurology · 2025Article
- Decline in activities of daily living in the rarer dementias.General psychiatry · 2025Review
- Comprehension of acoustically degraded emotional prosody in Alzheimer's disease and primary progressive aphasia.Scientific reports · 2024Article
- Transdiagnostic Network Localization of Social, Language, and Motor Symptoms in Patients With Frontotemporal Lobar Degeneration.Neurology · 2024Article
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Authors and funding
25 authors at 11 institutions in 5 countries.
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Abstract
introductionHere 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.
methodsAn 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.
resultsBoth 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.
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