| Autor: |
Ferry‐Bolder, Eve, Bouzigues, Arabella, Foster, Phoebe H, Peakman, Georgia, Greaves, Caroline V, Convery, Rhian S, van Swieten, John C., Jiskoot, Lize C., Seelaar, Harro, Moreno, Fermin, Sanchez‐Valle, Raquel, Laforce, Robert, Graff, Caroline, Masellis, Mario, Tartaglia, Carmela, Rowe, James B., Borroni, Barbara, Finger, Elizabeth, Synofzik, Matthis, Galimberti, Daniela |
| Zdroj: |
Alzheimer's & Dementia: The Journal of the Alzheimer's Association; Dec2023 Supplement 18, Vol. 19, p1-3, 3p |
| Abstrakt: |
Background: Previous research in genetic frontotemporal dementia (FTD) has suggested that the FTD Rating Scale (FRS) may be a more sensitive measure of disease severity than the Clinical Dementia Rating scale plus National Alzheimer's Coordinating Centre Frontotemporal Lobar Degeneration score (CDR+NACC FTLD). This study aims to assess the potential of longitudinal measurement of the FRS to track disease trajectory, using data from the Genetic FTD Initiative (GENFI). Method: 119 mutation negative controls and 270 mutation carriers (52 MAPT, 107 GRN, 111 C9orf72) from the GENFI cohort completed the FRS at their baseline and follow‐up visits. Participants were grouped by disease severity according to their CDR+NACC FTLD global score at the baseline visit, which generated five mutation groups: asymptomatic (0), prodromal (0.5), mild (1), moderate (2), and severe (3), plus the control group. Annualised FRS change scores were generated for each participant (mean interval between visits = 1.3 years, standard deviation = 0.6). For each of the genetic groups, correlations with annualised change score for the MMSE and the CDR+NACC FTLD SOB were performed. Result: As disease becomes more severe, the annualised change in FRS was larger, peaking at the moderate stage: asymptomatic 0.6 (8.0), prodromal ‐5.1, (23.2), mild ‐7.2 (24.8), moderate ‐7.8 (11.6), severe ‐1.8 (8.2). The moderate group was significantly different from controls (p = 0.018) and the asymptomatic group (p = 0.030). The annualised change in FRS negatively correlated with the annualised change in CDR+NACC FTLD Sum of Boxes (Rho = ‐0.4, p<0.001) and positively correlated with the annualised change in MMSE (Rho = 0.3, p = 0.001) in GRN MCs but not in MAPT or C9orf72. Conclusion: The FRS shows promise as a sensitive clinical outcome measure, but only at certain stages of the disease. More sophisticated modelling utilising the wider GENFI cohort will help to establish the real potential for use in clinical settings. [ABSTRACT FROM AUTHOR] |
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