ArticleJournal of neurology, neurosurgery, and psychiatry2024
Clinicoradiological and neuropathological evaluation of primary progressive aphasia.
Article in Journal of neurology, neurosurgery, and psychiatry, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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Who cites it
15 citing papers in PubMed, 11 citations in OpenAlex.
- Atypical Alzheimer disease: a multi-axis framework toward defining heterogeneity.Nature reviews. Neurology · 2026Review
- Eligibility for Anti-Amyloid Therapies in Patients With Biomarker-Confirmed Atypical Alzheimer Disease Phenotypes.Neurology · 2026Article
- Neuropathology-specific language features in primary progressive aphasia.Alzheimer's & dementia : the journal of the Alzheimer's Association · 2026Article
- Improving the clinical trial landscape for patients with atypical variants of Alzheimer's disease: a call to action.Alzheimer's & dementia : the journal of the Alzheimer's Association · 2026Review
- Idiotypic-susceptible Alzheimer's disease: a clinically relevant, neurofibrillary tangle subtype.Acta neuropathologica · 2026Article
- High-Level Alzheimer Disease Neuropathological Change Following Iatrogenic Exposure.JAMA neurology · 2026Article
- FDG-PET in the Evaluation of Primary Progressive Aphasia: A Narrative Review.Medicina (Kaunas, Lithuania) · 2026Review
- Amyloid and Tau Co-pathology in Parkinson Disease and Atypical Parkinsonism.Current neurology and neuroscience reports · 2026Review
- Identifying neuropathologic disease in primary progressive aphasia using narrative speech.Alzheimer's & dementia : the journal of the Alzheimer's Association · 2026Article
- The Pathology, Alzheimer's and Related Dementias Study (PARDoS): Design and Characteristics of the First 4,700+ Brazilian Participants.Neuroepidemiology · 2025Article
- Clinicopathological and Neuroimaging Correlates of Disease Duration in Primary Progressive Apraxia of Speech.European journal of neurology · 2025Article
- Pick's disease presenting as progressive apraxia of speech: Atypical clinical and neuroimaging features in three autopsy-confirmed cases.Clinical neurology and neurosurgery · 2025Article
- Protocol for a multisite study on the efficacy of transcranial direct current stimulation as an adjuvant to naming and spelling therapy in the treatment of oral and written naming in individuals with primary progressive aphasia.Frontiers in human neuroscience · 2025Article
- Clinical Manifestations and Neural Basis of Semantic Dementia: Converging Evidences From Brain Imaging Studies.Clinical interventions in aging · 2025Review
- Rethinking the diagnosis of primary progressive aphasia: current challenges and future directions.Frontiers in aging neuroscience · 2025Article
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Authors and funding
25 authors at 3 institutions in 1 country.
Funding
Abstract
backgroundPrimary progressive aphasia (PPA) defines a group of neurodegenerative disorders characterised by language decline. Three PPA variants correlate with distinct underlying pathologies: semantic variant PPA (svPPA) with transactive response DNA-binding protein of 43 kD (TDP-43) proteinopathy, agrammatic variant PPA (agPPA) with tau deposition and logopenic variant PPA (lvPPA) with Alzheimer's disease (AD). Our objectives were to differentiate PPA variants using clinical and neuroimaging features, assess progression and evaluate structural MRI and a novel 18-F fluorodeoxyglucose positron emission tomography (FDG-PET) image decomposition machine learning algorithm for neuropathology prediction.
methodsWe analysed 82 autopsied patients diagnosed with PPA from 1998 to 2022. Clinical histories, language characteristics, neuropsychological results and brain imaging were reviewed. A machine learning framework using a
resultsPPA variant distribution: 35 lvPPA (80% AD), 28 agPPA (89% tauopathy) and 18 svPPA (72% frontotemporal lobar degeneration-TAR DNA-binding protein (FTLD-TDP)). Apraxia of speech was associated with 4R-tauopathy in agPPA, while pure agrammatic PPA without apraxia was linked to 3R-tauopathy. Longitudinal data revealed language dysfunction remained the predominant deficit for patients with lvPPA, agPPA evolved to corticobasal or progressive supranuclear palsy syndrome (64%) and svPPA progressed to behavioural variant frontotemporal dementia (44%). agPPA-4R-tauopathy exhibited limited pre-supplementary motor area atrophy, lvPPA-AD displayed temporal atrophy extending to the superior temporal sulcus and svPPA-FTLD-TDP had severe temporal pole atrophy. The FDG-PET-based machine learning algorithm accurately predicted clinical diagnoses and underlying pathologies.
conclusionsDistinguishing 3R-taupathy and 4R-tauopathy in agPPA may rely on apraxia of speech presence. Additional linguistic and clinical features can aid neuropathology prediction. Our data-driven brain metabolism decomposition approach effectively predicts underlying neuropathology.
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