ArticleJournal of neurology2022
The natural history of primary progressive aphasia: beyond aphasia.
Article in Journal of neurology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 37 papers, 1 of them a synthesis that pooled it.
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Who cites it
37 citing papers in PubMed, 1 synthesis or guideline pooled it, 56 citations in OpenAlex.
- FDG-PET in the diagnosis of primary progressive aphasia: a systematic review.Annals of nuclear medicine · 2024Pooled it
- Neuropsychiatric Symptoms in Primary Progressive Aphasia: Phenomenology, Circuits, and Clinical Management.Current neurology and neuroscience reports · 2026Review
- Behavior determines times to care home admission and death in semantic dementia: Insights from a cohort of 90 cases.Alzheimer's & dementia : the journal of the Alzheimer's Association · 2026Article
- A Longitudinal Analysis of Frontotemporal Dementia Patients in Spain throughout the COVID-19 Pandemic: A Descriptive Study.Dementia and geriatric cognitive disorders · 2026Article
- Writing Abilities in Primary Progressive Aphasia: A Scoping Literature Review.Brain sciences · 2026Review
- Patterns and Trajectories of Behavioral and Neuropsychiatric Symptoms in Frontotemporal Dementia and Primary Progressive Aphasia.Neurology · 2026Observational
- Mixed primary progressive aphasia and alcohol use disorder: a case of detailed clinical phenotyping outperforming molecular imaging.Neurocase · 2026Article
- The Progressive Aphasia Communication Toolkit (PACT): A Strengths-Based Approach to Multidomain Evaluation for Intervention.medRxiv : the preprint server for health sciences · 2025Article
- Applicability and Correlates of a Symptom-Led Staging System for Primary Progressive Aphasia.European journal of neurology · 2025Article
- Adaptation of Better Conversations with Primary Progressive Aphasia to Norwegian.Brain sciences · 2025Article
- Longitudinal evaluation of common and unique brain-networks in variants of primary progressive aphasia.Alzheimer's research & therapy · 2025Article
- Psycholinguistic Variables and Spelling Accuracy for People with Logopenic Variant Primary Progressive Aphasia: A Cross-Sectional Study.Aphasiology · 2025Article
- Screening for Executive Impairment in Patients with Frontotemporal Dementia: Evidence from the Greek Version of the Frontier Executive Screen.Archives of clinical neuropsychology : the official journal of the National Academy of Neuropsychologists · 2025Article
- The graded multidimensional geometry of phenotypic variation and progression in neurodegenerative syndromes.Brain : a journal of neurology · 2025Article
- Rethinking the diagnosis of primary progressive aphasia: current challenges and future directions.Frontiers in aging neuroscience · 2025Article
- Advancing Neuropsychological Rehabilitation in Primary Progressive Aphasia Based on Principles of Cognitive Neuroscience: A Scoping Review and Systematic Analysis of the Data.Brain sciences · 2024Article
- Clinical and imaging correlates of hyperorality in syndromes associated with frontotemporal lobar degeneration.Psychiatry and clinical neurosciences · 2024Article
- Incidence of Primary Progressive Apraxia of Speech and Primary Progressive Aphasia in Olmsted County, MN, 2011-2022.Neurology · 2024Article
- Progression to corticobasal syndrome: a longitudinal study of patients with nonfluent primary progressive aphasia and primary progressive apraxia of speech.Journal of neurology · 2024Article
- Binary reversals: a diagnostic sign in primary progressive aphasia.Journal of neurology, neurosurgery, and psychiatry · 2024Article
Corrections and comments
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Authors and funding
16 authors at 4 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
introductionPrimary progressive aphasia (PPA) is divided into three prototypical subtypes that are all characterized by their single core symptom of aphasia. Although later in their course, other cognitive, behavioral, and motor domains may become involved, little is known about the progression profile of each subtype relative to the other subtypes.
methodsIn this longitudinal retrospective cohort study, based on the recent biomarker-supported diagnostic criteria, 24 subjects diagnosed with semantic variant (svPPA), 22 with non-fluent variant (nfvPPA), and 18 with logopenic variant (lvPPA) were collected and followed up for 1-6 years. Symptom distribution, cognitive test and neuropsychiatric inventory scores, and progression into another syndrome were assessed.
resultsOver time, lvPPA progressed with broader language problems (PPA-extended) and nfvPPA progressed to mutism, whereas semantic impairment remained the major problem in svPPA. Apart from linguistic problems, svPPA developed pronounced behavioral disturbances, whereas lvPPA exhibited a greater cognitive decline. By contrast, in nfvPPA motor deficits were more common. Furthermore, within 5 years (IQR = 2.5) after clinical onset, 65.6% of the patients additionally fulfilled the clinical criteria for another neurodegenerative syndrome (PPA-plus). Fourteen out of 24 (58%) svPPA patients additionally met the diagnostic criteria of behavioral variant frontotemporal dementia (5.1 years, IQR = 1.1), whereas the clinical features of 15/18 (83%) lvPPA patients were consistent with Alzheimer disease dementia (4.5 years IQR = 3.4). Furthermore, 12/22 (54%) of the subjects with the nfvPPA progressed to meet the diagnostic criteria of corticobasal syndrome, progressive supranuclear palsy, or motor neuron disease (5.1 years IQR = 3.4). DISCUSSION: Despite aphasia being the initial and unique hallmark of the syndrome, our longitudinal results showed that PPA is not a language limited disorder and progression differs widely for each subtype, both with respect to the nature of symptoms and disease duration.
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