ArticleJournal of neurology2024
Clinical and neuroanatomical characterization of the semantic behavioral variant of frontotemporal dementia in a multicenter Italian cohort.
Article in Journal of neurology, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers.
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Who cites it
11 citing papers in PubMed, 10 citations in OpenAlex.
- Frontotemporal dementia with right temporal predominance: a clinical comparison with left-predominant FTD.Journal of neurology · 2026Article
- Review
- Clinical recognition of frontotemporal dementia with right temporal predominance: a consensus statement from the International Working Group.Communications medicine · 2025Review
- Putative mitochondrial components of frontotemporal lobar degeneration: topological correlations between mitochondrial density and atrophy in FTLD/FTD phenotypes.Journal of neurology · 2025Article
- Distinctive Progression Patterns of Brain Structural Damage Aid Classification of Frontotemporal Dementia Variants.European journal of neurology · 2025Article
- Validation of criteria for frontotemporal dementia with right anterior temporal lobe predominance.Alzheimer's & dementia : the journal of the Alzheimer's Association · 2025Article
- Plasma placental growth factor as a biomarker for subcortical ischemic vascular dementia and its cognitive correlation mediated by white matter hyperintensities.Alzheimer's & dementia : the journal of the Alzheimer's Association · 2025Article
- Modelling pathological spread through the structural connectome in the frontotemporal dementia clinical spectrum.Brain : a journal of neurology · 2025Article
- Cerebellar dysfunction in frontotemporal dementia: intra-cerebellar pathology and cerebellar network degeneration.Journal of neurology · 2025Article
- A common marker of affect recognition dysfunction in the FTD spectrum of disorders.European journal of neurology · 2025Article
- Clinical, Cortical, Subcortical, and White Matter Features of Right Temporal Variant FTD.Brain sciences · 2024Article
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Authors and funding
21 authors at 5 institutions in 1 country.
Funding
Abstract
backgroundSemantic behavioral variant frontotemporal dementia (sbvFTD) is a neurodegenerative condition presenting with specific behavioral and semantic derangements and predominant atrophy of the right anterior temporal lobe (ATL). The objective was to evaluate clinical, neuropsychological, neuroimaging, and genetic features of an Italian sbvFTD cohort, defined according to recently proposed guidelines, compared to semantic variant primary progressive aphasia (svPPA) and behavioral variant FTD (bvFTD) patients.
methodsFifteen sbvFTD, sixty-three bvFTD, and twenty-five svPPA patients and forty controls were enrolled. Patients underwent clinical, cognitive evaluations, and brain MRI. Symptoms of bvFTD patients between onset and first visit were retrospectively recorded and classified as early and late. Grey matter atrophy was investigated using voxel-based morphometry.
resultssbvFTD experienced early criteria-specific symptoms: world, object and person-specific semantic loss (67%), complex compulsions and rigid thought (60%). Sequentially, more behavioral symptoms emerged (apathy/inertia, loss of empathy) along with non-criteria-specific symptoms (anxiety, suspiciousness). sbvFTD showed sparing of attentive/executive functions, especially compared to bvFTD and better language functions compared to svPPA. All sbvFTD patients failed at the famous face recognition test and more than 80% failed in understanding written metaphors and humor. At MRI, sbvFTD had predominant right ATL atrophy, almost specular to svPPA. Three sbvFTD patients presented pathogenic genetic variants.
conclusionWe replicated the application of sbvFTD diagnostic guidelines in an independent Italian cohort, demonstrating that the presence of person-specific semantic knowledge loss and mental rigidity, along with preserved executive functions and a predominant right ATL atrophy with sparing of frontal lobes, should prompt a diagnosis of sbvFTD.
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