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Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia.

Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia.
Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia.
Based on the recent literature and collective experience, an international consortium developed revised guidelines for the diagnosis of behavioural variant frontotemporal dementia. The validation process retrospectively reviewed clinical records and compared the sensitivity of proposed and earlier criteria in a multi-site sample of patients with pathologically verified frontotemporal lobar degeneration. According to the revised criteria, ‘possible’ behavioural variant frontotemporal dementia requires three of six clinically discriminating features (disinhibition, apathy/inertia, loss of sympathy/empathy, perseverative/compulsive behaviours, hyperorality and dysexecutive neuropsychological profile). ‘Probable’ behavioural variant frontotemporal dementia adds functional disability and characteristic neuroimaging, while behavioural variant frontotemporal dementia ‘with definite frontotemporal lobar degeneration’ requires histopathological confirmation or a pathogenic mutation. Sixteen brain banks contributed cases meeting histopathological criteria for frontotemporal lobar degeneration and a clinical diagnosis of behavioural variant frontotemporal dementia, Alzheimer’s disease, dementia with Lewy bodies or vascular dementia at presentation. Cases with predominant primary progressive aphasia or extra-pyramidal syndromes were excluded. In these autopsy-confirmed cases, an experienced neurologist or psychiatrist ascertained clinical features necessary for making a diagnosis according to previous and proposed criteria at presentation. Of 137 cases where features were available for both proposed and previously established criteria, 118 (86%) met ‘possible’ criteria, and 104 (76%) met criteria for ‘probable’ behavioural variant frontotemporal dementia. In contrast, 72 cases (53%) met previously established criteria for the syndrome (P < 0.001 for comparison with ‘possible’ and ‘probable’ criteria). Patients who failed to meet revised criteria were significantly older and most had atypical presentations with marked memory impairment. In conclusion, the revised criteria for behavioural variant frontotemporal dementia improve diagnostic accuracy compared with previously established criteria in a sample with known frontotemporal lobar degeneration. Greater sensitivity of the proposed criteria may reflect the optimized diagnostic features, less restrictive exclusion features and a flexible structure that accommodates different initial clinical presentations. Future studies will be needed to establish the reliability and specificity of these revised diagnostic guidelines.
2456-2477
Rascovsky, Katya
f7d1b504-9425-433c-b54c-5fb5eed07339
Hodges, John R.
c17af0a9-82e7-4f5a-8a97-d50ec06bbb0a
Knopman, David
025c8a8c-9bb5-44e7-9549-9b1ee7f6ec03
Kipps, Christopher M.
e43be016-2dc2-45e6-9a02-ab2a0e0208d5
et al.
Rascovsky, Katya
f7d1b504-9425-433c-b54c-5fb5eed07339
Hodges, John R.
c17af0a9-82e7-4f5a-8a97-d50ec06bbb0a
Knopman, David
025c8a8c-9bb5-44e7-9549-9b1ee7f6ec03
Kipps, Christopher M.
e43be016-2dc2-45e6-9a02-ab2a0e0208d5

Rascovsky, Katya, Hodges, John R. and Knopman, David , et al. (2011) Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain, 134 (9), 2456-2477. (doi:10.1093/brain/awr179).

Record type: Article

Abstract

Based on the recent literature and collective experience, an international consortium developed revised guidelines for the diagnosis of behavioural variant frontotemporal dementia. The validation process retrospectively reviewed clinical records and compared the sensitivity of proposed and earlier criteria in a multi-site sample of patients with pathologically verified frontotemporal lobar degeneration. According to the revised criteria, ‘possible’ behavioural variant frontotemporal dementia requires three of six clinically discriminating features (disinhibition, apathy/inertia, loss of sympathy/empathy, perseverative/compulsive behaviours, hyperorality and dysexecutive neuropsychological profile). ‘Probable’ behavioural variant frontotemporal dementia adds functional disability and characteristic neuroimaging, while behavioural variant frontotemporal dementia ‘with definite frontotemporal lobar degeneration’ requires histopathological confirmation or a pathogenic mutation. Sixteen brain banks contributed cases meeting histopathological criteria for frontotemporal lobar degeneration and a clinical diagnosis of behavioural variant frontotemporal dementia, Alzheimer’s disease, dementia with Lewy bodies or vascular dementia at presentation. Cases with predominant primary progressive aphasia or extra-pyramidal syndromes were excluded. In these autopsy-confirmed cases, an experienced neurologist or psychiatrist ascertained clinical features necessary for making a diagnosis according to previous and proposed criteria at presentation. Of 137 cases where features were available for both proposed and previously established criteria, 118 (86%) met ‘possible’ criteria, and 104 (76%) met criteria for ‘probable’ behavioural variant frontotemporal dementia. In contrast, 72 cases (53%) met previously established criteria for the syndrome (P < 0.001 for comparison with ‘possible’ and ‘probable’ criteria). Patients who failed to meet revised criteria were significantly older and most had atypical presentations with marked memory impairment. In conclusion, the revised criteria for behavioural variant frontotemporal dementia improve diagnostic accuracy compared with previously established criteria in a sample with known frontotemporal lobar degeneration. Greater sensitivity of the proposed criteria may reflect the optimized diagnostic features, less restrictive exclusion features and a flexible structure that accommodates different initial clinical presentations. Future studies will be needed to establish the reliability and specificity of these revised diagnostic guidelines.

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More information

Accepted/In Press date: 13 June 2011
e-pub ahead of print date: 2 August 2011
Published date: September 2011

Identifiers

Local EPrints ID: 490609
URI: http://eprints.soton.ac.uk/id/eprint/490609
PURE UUID: 9d5be852-9153-4a30-9d91-d3bb78c4ee32
ORCID for Christopher M. Kipps: ORCID iD orcid.org/0000-0002-5205-9712

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Date deposited: 31 May 2024 16:41
Last modified: 01 Jun 2024 01:54

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Contributors

Author: Katya Rascovsky
Author: John R. Hodges
Author: David Knopman
Author: Christopher M. Kipps ORCID iD
Corporate Author: et al.

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