Diagnostic accuracy of behavioral variant frontotemporal dementia consortium criteria (FTDC) in a clinicopathological cohort.
OBJECTIVE
The aims of this study were to assess the sensitivity of the International Behavioural Variant FTD Criteria Consortium (FTDC) revised criteria of behavioural variant frontotemporal dementia (bvFTD) in a pathological cohort and to determine their predictive values in a clinical context suggestive of bvFTD. In addition, the study aimed to assess the influence of the age at onset and underlying pathology in the clinicopathological correlations.


METHODS
Retrospective, blinded review of the clinical and neuropathological data from the Neurological Tissue Bank of the Biobank Hospital Clinic-IDIBAPS, Barcelona (Spain) was conducted, assessing the fulfilment of the diagnostic criteria on a case-by-case basis. Two separate nonexclusive cohorts were selected: Cohort 1 (n = 58) subjects with pathological diagnosis of frontotemporal lobar degeneration (FTLD) and Cohort 2 (n = 66) subjects with the premortem diagnosis of bvFTD.


RESULTS
The FTDC criteria reached a sensitivity of 93% for possible and 80% for probable bvFTD. Early-onset cases displayed significantly more disinhibition, loss of empathy and compulsive behaviour with respect to late-onset bvFTD, leading to a slightly higher sensitivity of the diagnostic criteria (97% vs. 91%). There were no differences in the diagnostic performance between tau-positive and tau-negative cases. In subjects clinically diagnosed as bvFTD, a 'possible bvFTD' diagnosis reached a positive predictive value for FTLD pathology of 90%, irrespective of underlying proteinopathy. False-positive clinical diagnoses were mainly Alzheimer's disease. These cases were significantly older, had less family history of dementia and had a predominantly apathetic clinical picture.


CONCLUSIONS
The revised bvFTD criteria present good sensitivity and positive predictive value in both early- and late-onset cases and regardless of the underlying FTLD pathology.
Alzheimer's Disease and Other Cognitive Disorders Unit, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain.
Mircea
M
Balasa
Neurological Tissue Bank of the Biobank, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain.
Ellen
E
Gelpi
Alzheimer's Disease and Other Cognitive Disorders Unit, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain.
Idaira
I
Martín
Alzheimer's Disease and Other Cognitive Disorders Unit, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain.
Anna
A
Antonell
Neurological Tissue Bank of the Biobank, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain.
Ma Jesus
MJ
Rey
Alzheimer's Disease and Other Cognitive Disorders Unit, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain.|Neurological Tissue Bank of the Biobank, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain.
Oriol
O
Grau-Rivera
Alzheimer's Disease and Other Cognitive Disorders Unit, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain.
José Luis
JL
Molinuevo
Alzheimer's Disease and Other Cognitive Disorders Unit, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain.
Raquel
R
Sánchez-Valle
Alzheimer's Disease and Other Cognitive Disorders Unit, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain.
Albert
A
Lladó
D000328:Adult; D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D018070:Biological Specimen Banks; D001921:Brain / Q000473:pathology*; D005260:Female; D057180:Frontotemporal Dementia / Q000175:diagnosis* / Q000473:pathology; D057174:Frontotemporal Lobar Degeneration / Q000175:diagnosis* / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009483:Neuropsychological Tests; D012189:Retrospective Studies
behavioural variant; diagnostic criteria; frontotemporal dementia