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Behavioral variant FTD (bvFTD), non-fluent variant primary progressive aphasia (nfvPPA) and semantic variant primary progressive aphasia (svPPA)

Behavioral variant FTD (bvFTD), non-fluent variant primary progressive aphasia (nfvPPA) and semantic variant primary progressive aphasia (svPPA). Clinical data for the following measures: Mini-Mental State Examination (MMSE), and Clinical Dementia Rating (CDR) scores are presented as Mean ± SD, with the percent of the group tested in parenthesis.

Language, Behavior and Global CDR Clinical Scores

Language, Behavior and Global CDR Clinical Scores

Statistical significance was assessed by two-way ANOVA and Tukey's test for multiple comparisons. ns= not significant;  * p<0.05; *** p<0.001; **** p<0.0001 (significant differences compared to control have been exclude).

Data Source

Frontotemporal Lobar Degeneration Neuroimaging Initiative (FTLDNI)

Data used in the preparation of this presentation were obtained from the Frontotemporal Lobar Degeneration Neuroimaging Initiative (FTLDNI) (https://4rtni-ftldni.ini.usc.edu/). FTLDNI is founded through the National Institute of Aging, and started in 2010. The primary goals of FTLDNI are to identify neuroimaging modalities and methods of analysis for tracking frontotemporal lobar degeneration (FTLD) and to assess the value of imaging versus other biomarkers in diagnostic roles. The Principal Investigator of NIFD is Dr. Howard Rosen, MD at the University of California, San Francisco. The data is the result of collaborative efforts at three sites in North America. For up-to-date information on participation and protocol, please visit: https://memory.ucsf.edu/research/studies/nifd.

Data collection and sharing for this project was funded by the Frontotemporal Lobar Degeneration Neuroimaging Initiative (National Institutes of Health Grant R01 AG032306). The study is coordinated through the University of California, San Francisco, Memory and Aging Center. FTLDNI data are disseminated by the Laboratory for Neuro Imaging at the University of Southern California.

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The FTD data used for this analysis was obtained from the Frontotemporal Lobar Degeneration Neuroimaging Initiative (FTLDNI). A primary goal of these studies is to identify neuroimaging and biomarker indicators of disease progression in this complex array of related neurodegenerative diseases.

As shown in the Table, 238 participants were analyzed, including 52 with behavioral variant FTD (bvFTD), 32 with non-fluent variant primary progressive aphasia (nfvPPA), and 35 with semantic variant primary progressive aphasia (svPPA), alongside 117 healthy controls. All subjects were evaluated by MRI at baseline, with multiple follow-up visits extending out to 24 months, with clinical rating scales assessed at similar intervals.

Participants in the FTD groups had higher CDR scores and lower MMSE scores compared to the healthy control participants. To avoid bias, only images from control subjects obtained with a similar scanning acquisition protocol were included in our analysis.

The Clinical Dementia Rating (CDR) scale has been expanded to include Behavior and Language scales to better assess patients on the frontotemporal dementia spectrum. Patients with the behavioral variant score significantly higher in the Behavior, Comportment and Personality domain, while non-fluent and semantic aphasia patients, which cannot be separated on the classic CDR global scale, can be discriminated by a combination of significant deficits in their Behavior and Language abilities, including communication, speech impairment, and language functions.

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