ArticleNeurocase2012
Clinical characterization of bvFTD due to FUS neuropathology.
Article in Neurocase, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers, 2 of them syntheses that pooled it.
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Who cites it
14 citing papers in PubMed, 2 syntheses or guidelines pooled it.
- Neuropsychological Profiles in Genetic Frontotemporal Dementia: A Meta-Analysis and Systematic Review.Aging and disease · 2024Pooled it
- Right temporal variant frontotemporal dementia is pathologically heterogeneous: a case-series and a systematic review.Acta neuropathologica communications · 2021Pooled it
- Clinical recognition of frontotemporal dementia with right temporal predominance: a consensus statement from the International Working Group.Communications medicine · 2025Review
- Understanding Theory of Mind-pragmatics relationship through the spectrum of neurodegenerative diseases.Philosophical transactions of the Royal Society of London. Series B, Biological sciences · 2025Review
- A clinical-radiological framework of the right temporal variant of frontotemporal dementia.Brain : a journal of neurology · 2020Article
- Neuroimaging in Dementia.Seminars in neurology · 2017Review
- A clinicopathological approach to the diagnosis of dementia.Nature reviews. Neurology · 2017Review
- Phenotypic Heterogeneity of Monogenic Frontotemporal Dementia.Frontiers in aging neuroscience · 2015Review
- Longitudinal white matter changes in frontotemporal dementia subtypes.Human brain mapping · 2014Article
- Diagnosis and management of behavioral variant frontotemporal dementia.Biological psychiatry · 2014Review
- Neural substrates of socioemotional self-awareness in neurodegenerative disease.Brain and behavior · 2014Article
- Dynamical predictions of insular hubs for social cognition and their application to stroke.Frontiers in behavioral neuroscience · 2014Article
- Acute lesions that impair affective empathy.Brain : a journal of neurology · 2013Article
- Atypical, slowly progressive behavioural variant frontotemporal dementia associated with C9ORF72 hexanucleotide expansion.Journal of neurology, neurosurgery, and psychiatry · 2012Article
Corrections and comments
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Authors and funding
8 authors.
Funding
Abstract
In 2009, inclusions containing the fused in sarcoma (FUS) protein were identified as a third major molecular class of pathology underlying the behavioral variant frontotemporal dementia (bvFTD) syndrome. Due to the low prevalence of FUS pathology, few clinical descriptions have been published and none provides information about specific social-emotional deficits despite evidence for severe behavioral manifestations in this disorder. We evaluated a patient with bvFTD due to FUS pathology using a comprehensive battery of cognitive and social- emotional tests. A structural MRI scan and genetic tests for tau, progranulin, and FUS mutations were also performed. The patient showed preserved general cognitive functioning and superior working memory, but severe deficits in emotion attribution, sensitivity to punishment, and diminished capacity for interpersonal warmth and empathy. The gray matter atrophy pattern corresponded to this focal deficit profile, with preservation of dorsolateral fronto-parietal regions associated with executive functioning but severe damage to right worse than left frontoinsula, temporal pole, subgenual anterior cingulate, medial orbitofrontal cortex, amygdala, and caudate. This patient demonstrates the striking focality associated with FUS neuropathology in patients with bvFTD.
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Registered trials
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