Diagnostic accuracy of research criteria for prodromal frontotemporal dementia.
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ABSTRACT: Background
The Genetic Frontotemporal Initiative Staging Group has proposed clinical criteria for the diagnosis of prodromal frontotemporal dementia (FTD), termed mild cognitive and/or behavioral and/or motor impairment (MCBMI). The objective of the study was to validate the proposed research criteria for MCBMI-FTD in a cohort of genetically confirmed FTD cases against healthy controls.Methods
A total of 398 participants were enrolled, 117 of whom were carriers of an FTD pathogenic variant with mild clinical symptoms, while 281 were non-carrier family members (healthy controls (HC)). A subgroup of patients underwent blood neurofilament light (NfL) levels and anterior cingulate atrophy assessment.Results
The core clinical criteria correctly classified MCBMI vs HC with an AUC of 0.79 (p < 0.001), while the addition of either blood NfL or anterior cingulate atrophy significantly increased the AUC to 0.84 and 0.82, respectively (p < 0.001). The addition of both markers further increased the AUC to 0.90 (p < 0.001).Conclusions
The proposed MCBMI criteria showed very good classification accuracy for identifying the prodromal stage of FTD.
SUBMITTER: Benussi A
PROVIDER: S-EPMC10785469 | biostudies-literature | 2024 Jan
REPOSITORIES: biostudies-literature
Publications
Diagnostic accuracy of research criteria for prodromal frontotemporal dementia.
Benussi Alberto A Premi Enrico E Grassi Mario M Alberici Antonella A Cantoni Valentina V Gazzina Stefano S Archetti Silvana S Gasparotti Roberto R Fumagalli Giorgio G GG Bouzigues Arabella A Russell Lucy L LL Samra Kiran K Cash David M DM Bocchetta Martina M Todd Emily G EG Convery Rhian S RS Swift Imogen I Sogorb-Esteve Aitana A Heller Carolin C van Swieten John C JC Jiskoot Lize C LC Seelaar Harro H Sanchez-Valle Raquel R Moreno Fermin F Laforce Robert Jr RJ Graff Caroline C Synofzik Matthis M Galimberti Daniela D Rowe James B JB Masellis Mario M Tartaglia Maria Carmela MC Finger Elizabeth E Vandenberghe Rik R Mendonça Alexandre A Tiraboschi Pietro P Butler Chris R CR Santana Isabel I Gerhard Alexander A Le Ber Isabelle I Pasquier Florence F Ducharme Simon S Levin Johannes J Sorbi Sandro S Otto Markus M Padovani Alessandro A Rohrer Jonathan D JD Borroni Barbara B
Alzheimer's research & therapy 20240112 1
<h4>Background</h4>The Genetic Frontotemporal Initiative Staging Group has proposed clinical criteria for the diagnosis of prodromal frontotemporal dementia (FTD), termed mild cognitive and/or behavioral and/or motor impairment (MCBMI). The objective of the study was to validate the proposed research criteria for MCBMI-FTD in a cohort of genetically confirmed FTD cases against healthy controls.<h4>Methods</h4>A total of 398 participants were enrolled, 117 of whom were carriers of an FTD pathogen ...[more]
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Project description:ObjectiveTo evaluate the interrater reliability of the new International Behavioural Variant FTD Criteria Consortium (FTDC) criteria for behavioral variant frontotemporal dementia (bvFTD).MethodsTwenty 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 κ statistics for multiple raters with categorical ratings.ResultsThe mean κ 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; κ = 0.61-0.80), whereas 2 had "moderate" agreement (apathy/inertia, neuropsychological; κ = 0.41-0.6). Clinician years of experience did not significantly influence rater accuracy.ConclusionsThe 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.
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