Dataset Information

Interrater reliability of the new criteria for behavioral variant frontotemporal dementia.

ABSTRACT:

Objective

To evaluate the interrater reliability of the new International Behavioural Variant FTD Criteria Consortium (FTDC) criteria for behavioral variant frontotemporal dementia (bvFTD).

Methods

Twenty standardized clinical case modules were developed for patients with a range of neurodegenerative diagnoses, including bvFTD, primary progressive aphasia (nonfluent, semantic, and logopenic variant), Alzheimer disease, and Lewy body dementia. Eighteen blinded raters reviewed the modules and 1) rated the presence or absence of core diagnostic features for the FTDC criteria, and 2) provided an overall diagnostic rating. Interrater reliability was determined by ? statistics for multiple raters with categorical ratings.

Results

The mean ? value for diagnostic agreement was 0.81 for possible bvFTD and 0.82 for probable bvFTD ("almost perfect agreement"). Interrater reliability for 4 of the 6 core features had "substantial" agreement (behavioral disinhibition, perseverative/compulsive, sympathy/empathy, hyperorality; ? = 0.61-0.80), whereas 2 had "moderate" agreement (apathy/inertia, neuropsychological; ? = 0.41-0.6). Clinician years of experience did not significantly influence rater accuracy.

Conclusions

The FTDC criteria show promise for improving the diagnostic accuracy and reliability of clinicians and researchers. As disease-altering therapies are developed, accurate differential diagnosis between bvFTD and other neurodegenerative diseases will become increasingly important.

SUBMITTER: Lamarre AK

PROVIDER: S-EPMC3716343 | biostudies-literature | 2013 May

REPOSITORIES: biostudies-literature

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Publications

Interrater reliability of the new criteria for behavioral variant frontotemporal dementia.

Lamarre Amanda K AK Rascovsky Katya K Bostrom Alan A Toofanian Parnian P Wilkins Sarah S Sha Sharon J SJ Perry David C DC Miller Zachary A ZA Naasan Georges G Laforce Robert R Hagen Jayne J Takada Leonel T LT Tartaglia Maria Carmela MC Kang Gail G Galasko Douglas D Salmon David P DP Farias Sarah Tomaszewski ST Kaur Berneet B Olichney John M JM Quitania Park Lovingly L Mendez Mario F MF Tsai Po-Heng PH Teng Edmond E Dickerson Bradford Clark BC Domoto-Reilly Kimiko K McGinnis Scott S Miller Bruce L BL Kramer Joel H JH

Neurology 20130501 21

<h4>Objective</h4>To evaluate the interrater reliability of the new International Behavioural Variant FTD Criteria Consortium (FTDC) criteria for behavioral variant frontotemporal dementia (bvFTD).<h4>Methods</h4>Twenty standardized clinical case modules were developed for patients with a range of neurodegenerative diagnoses, including bvFTD, primary progressive aphasia (nonfluent, semantic, and logopenic variant), Alzheimer disease, and Lewy body dementia. Eighteen blinded raters reviewed the m ...[more]

PMID: 23635967

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Project description:At present, no research criteria exist for the diagnosis of prodromal behavioural variant frontotemporal dementia (bvFTD), though early detection is of high research importance. Thus, we sought to develop and validate a proposed set of research criteria for prodromal bvFTD, termed 'mild behavioural and/or cognitive impairment in bvFTD' (MBCI-FTD). Participants included 72 participants deemed to have prodromal bvFTD; this comprised 55 carriers of a pathogenic mutation known to cause frontotemporal lobar degeneration, and 17 individuals with autopsy-confirmed frontotemporal lobar degeneration. All had mild behavioural and/or cognitive changes, as judged by an evaluating clinician. Based on extensive clinical workup, the prodromal bvFTD group was divided into a Development Group (n = 22) and a Validation Group (n = 50). The Development Group was selected to be the subset of the prodromal bvFTD group for whom we had the strongest longitudinal evidence of conversion to bvFTD, and was used to develop the MBCI-FTD criteria. The Validation Group was the remainder of the prodromal bvFTD group and was used as a separate sample on which to validate the criteria. Familial non-carriers were included as healthy controls (n = 165). The frequencies of behavioural and neuropsychiatric features, neuropsychological deficits, and social cognitive dysfunction in the prodromal bvFTD Development Group and healthy controls were assessed. Based on sensitivity and specificity analyses, seven core features were identified: apathy without moderate-severe dysphoria, behavioural disinhibition, irritability/agitation, reduced empathy/sympathy, repetitive behaviours (simple and/or complex), joviality/gregariousness, and appetite changes/hyperorality. Supportive features include a neuropsychological profile of impaired executive function or naming with intact orientation and visuospatial skills, reduced insight for cognitive or behavioural changes, and poor social cognition. Three core features or two core features plus one supportive feature are required for the diagnosis of possible MBCI-FTD; probable MBCI-FTD requires imaging or biomarker evidence, or a pathogenic genetic mutation. The proposed MBCI-FTD criteria correctly classified 95% of the prodromal bvFTD Development Group, and 74% of the prodromal bvFTD Validation Group, with a false positive rate of <10% in healthy controls. Finally, the MBCI-FTD criteria were tested on a cohort of individuals with prodromal Alzheimer's disease, and the false positive rate of diagnosis was 11-16%. Future research will need to refine the sensitivity and specificity of these criteria, and incorporate emerging biomarker evidence.

Dataset Information

Interrater reliability of the new criteria for behavioral variant frontotemporal dementia.

Objective

Methods

Results

Conclusions

Publications

Interrater reliability of the new criteria for behavioral variant frontotemporal dementia.

Similar Datasets

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