Observational studyNeurology2024
Primary Progressive Aphasia Lacking Core Features of Nonfluent and Semantic Variants: Clinical, Neuroimaging, and Neuropathologic Features.
Observational study in Neurology, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers.
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Who cites it
7 citing papers in PubMed.
- 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
- Imposter in the brain: aetiological, clinical and neuroimaging characteristics of Capgras syndrome.Brain : a journal of neurology · 2026Article
- Semantic control across Alzheimer's disease phenotypes: cognitive mechanisms and neural correlates.Frontiers in aging neuroscience · 2026Article
- Distinct cerebral perfusion patterns and linguistic profiles in Alzheimer's disease-related primary progressive aphasia.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2025Article
- Psycholinguistic Variables and Spelling Accuracy for People with Logopenic Variant Primary Progressive Aphasia: A Cross-Sectional Study.Aphasiology · 2025Article
- Primary Progressive Aphasia Treatment: Current Treatment Options in Neurology Article Topic: Management of Primary Progressive Aphasia.Current treatment options in neurology · 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
10 authors.
Funding
Abstract
BACKGROUND AND
objectivesEvidence has accumulated that the 2011 consensus criteria for primary progressive aphasia (PPA) do not fully capture features of logopenic variant PPA (lvPPA/LPA). We aimed to examine clinical, neuroimaging, and neuropathologic features of PPA lacking features of nonfluent/semantic variants and to provide practical additions to the 2011 consensus criteria.
methodsThis was a retrospective examination of data from 2 observational cohort studies where patients with PPA were prospectively recruited at Mayo Clinic. Based on performance on 2 cardinal features (repetition and comprehension), patients were classified as: pure-LPA (poor repetition, acceptable comprehension), Wernicke-like (poor in both), anomic-like (acceptable in both), and transcortical sensory aphasia-like (TCSA-like) (acceptable repetition, poor comprehension).
resultsThe entire cohort consisted of 102 patients with PPA lacking features of nonfluent/semantic variants (median age at onset 63.5 years, 56% female). Thirty-one patients were followed up at 1 year. Twenty-three patients were included in a neuropathologic cohort. The proportion of repetition-preserved PPA (anomic-like and TCSA-like) was more than double that of repetition-impaired PPA (pure-LPA and Wernicke-like) (73% vs 27%). Regarding clinical course, the anomic-like subgroup was a prodromal state of the pure-LPA or TCSA-like subgroup, whereas the pure-LPA and TCSA-like subgroups were a prodromal state of the Wernicke-like subgroup. There was left temporoparietal atrophy on MRI and/or hypometabolism on DISCUSSION: This observed clinical heterogeneity reflects different time points/severities of the same disease process and hence can be reconceptualized as an AD-related aphasia spectrum, incorporating lvPPA and the 4 subgroups (pure-LPA, Wernicke-like, anomic-like, and TCSA-like). Specifying moderate/severe repetition deficits as a core feature of lvPPA in the 2011 consensus criteria can enhance its pathologic correlations. Recognizing progressive anomic aphasia (anomic-like) as an additional PPA variant could lessen pathologic heterogeneity of lvPPA.
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