title: New criteria for frontotemporal dementia syndromes: clinical and pathological diagnostic implications.
abstract: OBJECTIVE
  To assess the impact of new clinical diagnostic criteria for frontotemporal dementia (FTD) syndromes, including primary progressive aphasias (PPA), on prior clinical diagnosis and to explore clinicopathological correlations.
  
  
  METHODS
  178 consecutive neuropathologically ascertained cases initially diagnosed with a FTD syndrome were collected through specialist programmes: the Cambridge Brain Bank, UK, and Sydney Brain Bank, Australia. 135 cases were reclassified using the revised diagnostic criteria into behavioural variant (bvFTD), semantic variant PPA (sv-PPA), non-fluent/agrammatic variant PPA (nfv-PPA) and logopenic variant PPA (lv-PPA). Pathological diagnoses included FTLD-tau, FTLD-TDP, FTLD-FUS, FTLD-UPS, FLTD-ni and Alzheimer's disease (AD). Statistical analyses included χ(2) tests, analyses of variance and discriminant statistics.
  
  
  RESULTS
  Comparison of the original and revised diagnosis revealed no change in 90% of bvFTD and sv-PPA cases. By contrast, 51% of nfv-PPA cases were reclassified as lv-PPA, with apraxia of speech and sentence repetition assisting in differentiation. Previous patterns of pathology were confirmed, although more AD cases occurred in FTD syndromes (10% bvFTD, ∼15% sv-PPA and ∼30% nfv-PPA) than expected. AD was the dominant pathology (77%) of lv-PPA. Discriminant analyses revealed that object agnosia, phonological errors and neuropsychiatric features differentiated AD from FTLD.
  
  
  CONCLUSIONS
  This study provides pathological validation that the new criteria assist with separating PPA cases with AD pathology into the new lv-PPA syndrome and found that a number of diagnostic clinical features (disinhibition, food preferences and naming) did not assist in discriminating the different FTD syndromes.
authors.affiliation: Neuroscience Research Australia, Sydney, Australia UNSW Medicine, University of New South Wales, Sydney, Australia.
authors.forename: Leone
authors.initials: L
authors.lastname: Chare
authors.affiliation: Neuroscience Research Australia, Sydney, Australia UNSW Medicine, University of New South Wales, Sydney, Australia ARC Centre of Excellence in Cognition and its Disorders, Sydney, Australia.
authors.forename: John R
authors.initials: JR
authors.lastname: Hodges
authors.affiliation: Neuroscience Research Australia, Sydney, Australia ARC Centre of Excellence in Cognition and its Disorders, Sydney, Australia.
authors.forename: Cristian E
authors.initials: CE
authors.lastname: Leyton
authors.affiliation: Disciplines of Pathology, Sydney Medical School, The University of Sydney, Sydney, Australia.
authors.forename: Ciara
authors.initials: C
authors.lastname: McGinley
authors.affiliation: Neuroscience Research Australia, Sydney, Australia.
authors.forename: Rachel H
authors.initials: RH
authors.lastname: Tan
authors.affiliation: Disciplines of Pathology, Sydney Medical School, The University of Sydney, Sydney, Australia Disciplines of Medicine, Sydney Medical School, The University of Sydney, Sydney, Australia.
authors.forename: Jillian J
authors.initials: JJ
authors.lastname: Kril
authors.affiliation: Neuroscience Research Australia, Sydney, Australia UNSW Medicine, University of New South Wales, Sydney, Australia.
authors.forename: Glenda M
authors.initials: GM
authors.lastname: Halliday
mesh_terms: D017668:Age of Onset; D000368:Aged; D000544:Alzheimer Disease / Q000175:diagnosis; D001519:Behavior / Q000502:physiology; D003937:Diagnosis, Differential; D005260:Female; D057180:Frontotemporal Dementia / Q000145:classification / Q000175:diagnosis* / Q000523:psychology*; D006801:Humans; D007802:Language; D008297:Male; D001523:Mental Disorders / Q000209:etiology / Q000523:psychology; D008875:Middle Aged; D009068:Movement / Q000502:physiology
keywords: Alzheimer's Disease; Aphasia; Behavioural Disorder; Dementia; Neuropathology