title: The Language Profile of Behavioral Variant Frontotemporal Dementia.
abstract: BACKGROUND
  The language profile of behavioral variant frontotemporal dementia (bvFTD) remains to be fully defined.
  
  
  OBJECTIVE
  We aimed to quantify the extent of language deficits in this patient group.
  
  
  METHODS
  We assessed a cohort of patients with bvFTD (n = 24) in relation to patients with semantic variant primary progressive aphasia (svPPA; n = 14), nonfluent variant primary progressive aphasia (nfvPPA; n = 18), and healthy age-matched individuals (n = 24) cross-sectionally and longitudinally using a comprehensive battery of language and general neuropsychological tests. Neuroanatomical associations of language performance were assessed using voxel-based morphometry of patients' brain magnetic resonance images.
  
  
  RESULTS
  Relative to healthy controls, and after accounting for nonverbal executive performance, patients with bvFTD showed deficits of noun and verb naming and single word comprehension, diminished spontaneous propositional speech, and deterioration in naming performance over time. Within the bvFTD group, patients with MAPT mutations had more severe impairments of noun naming and single word comprehension than patients with C9orf72 mutations. Overall the bvFTD group had less severe language deficits than patients with PPA, but showed a language profile that was qualitatively similar to svPPA. Neuroanatomical correlates of naming and word comprehension performance in bvFTD were identified predominantly in inferior frontal and antero-inferior temporal cortices within the dominant hemispheric language network.
  
  
  CONCLUSIONS
  bvFTD is associated with a language profile including verbal semantic impairment that warrants further evaluation as a novel biomarker.
authors.affiliation: Dementia Research Centre, Department of Neurodegenerative Disease, UCL Institute of Neurology, University College London, London, UK.
authors.forename: Chris J D
authors.initials: CJ
authors.lastname: Hardy
authors.affiliation: Dementia Research Centre, Department of Neurodegenerative Disease, UCL Institute of Neurology, University College London, London, UK.
authors.forename: Aisling H
authors.initials: AH
authors.lastname: Buckley
authors.affiliation: Dementia Research Centre, Department of Neurodegenerative Disease, UCL Institute of Neurology, University College London, London, UK.
authors.forename: Laura E
authors.initials: LE
authors.lastname: Downey
authors.affiliation: Dementia Research Centre, Department of Neurodegenerative Disease, UCL Institute of Neurology, University College London, London, UK.
authors.forename: Manja
authors.initials: M
authors.lastname: Lehmann
authors.affiliation: Department of Language & Cognition, Division of Psychology & Language Sciences, University College London, London, UK.
authors.forename: Vitor C
authors.initials: VC
authors.lastname: Zimmerer
authors.affiliation: Department of Language & Cognition, Division of Psychology & Language Sciences, University College London, London, UK.
authors.forename: Rosemary A
authors.initials: RA
authors.lastname: Varley
authors.affiliation: Dementia Research Centre, Department of Neurodegenerative Disease, UCL Institute of Neurology, University College London, London, UK.
authors.forename: Sebastian J
authors.initials: SJ
authors.lastname: Crutch
authors.affiliation: Dementia Research Centre, Department of Neurodegenerative Disease, UCL Institute of Neurology, University College London, London, UK.
authors.forename: Jonathan D
authors.initials: JD
authors.lastname: Rohrer
authors.affiliation: Dementia Research Centre, Department of Neurodegenerative Disease, UCL Institute of Neurology, University College London, London, UK.
authors.forename: Elizabeth K
authors.initials: EK
authors.lastname: Warrington
authors.affiliation: Dementia Research Centre, Department of Neurodegenerative Disease, UCL Institute of Neurology, University College London, London, UK.
authors.forename: Jason D
authors.initials: JD
authors.lastname: Warren
mesh_terms: D000368:Aged; D018888:Aphasia, Primary Progressive / Q000473:pathology / Q000523:psychology*; D001284:Atrophy / Q000473:pathology; D001921:Brain / Q000473:pathology*; D003071:Cognition / Q000502:physiology; D032882:Comprehension / Q000502:physiology; D005260:Female; D057180:Frontotemporal Dementia / Q000473:pathology / Q000523:psychology*; D006801:Humans; D007802:Language; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D059906:Neuroimaging; D009483:Neuropsychological Tests; D057178:Primary Progressive Nonfluent Aphasia / Q000473:pathology / Q000523:psychology*
keywords: Behavioral variant frontotemporal dementia; frontotemporal dementia; primary progressive aphasia