| Title: |
Comparison of clinical rating scales in genetic frontotemporal dementia within the GENFI cohort |
| Authors: |
Peakman, G; Russell, LL; Convery, RS; Nicholas, JM; Van Swieten, JC; Jiskoot, LC; Moreno, F; Sanchez-Valle, R; Laforce, R; Graff, C; Masellis, M; Tartaglia, MC; Rowe, JB; Borroni, B; Finger, E; Synofzik, M; Galimberti, D; Vandenberghe, R; de Mendonça, A; Butler, CR; Gerhard, A; Ducharme, S; Le Ber, I; Tagliavini, F; Santana, I |
| Publisher Information: |
BMJ Publishing Group |
| Publication Year: |
2026 |
| Collection: |
Oxford University Research Archive (ORA) |
| Description: |
BACKGROUND: Therapeutic trials are now underway in genetic forms of frontotemporal dementia (FTD) but clinical outcome measures are limited. The two most commonly used measures, the Clinical Dementia Rating (CDR)+National Alzheimer’s Disease Coordinating Center (NACC) Frontotemporal Lobar Degeneration (FTLD) and the FTD Rating Scale (FRS), have yet to be compared in detail in the genetic forms of FTD. METHODS: The CDR+NACC FTLD and FRS were assessed cross-sectionally in 725 consecutively recruited participants from the Genetic FTD Initiative: 457 mutation carriers (77 microtubule-associated protein tau (MAPT), 187 GRN, 193 C9orf72) and 268 family members without mutations (non-carrier control group). 231 mutation carriers (51 MAPT, 92 GRN, 88 C9orf72) and 145 non-carriers had available longitudinal data at a follow-up time point. RESULTS: Cross-sectionally, the mean FRS score was lower in all genetic groups compared with controls: GRN mutation carriers mean 83.4 (SD 27.0), MAPT mutation carriers 78.2 (28.8), C9orf72 mutation carriers 71.0 (34.0), controls 96.2 (7.7), p |
| Document Type: |
article in journal/newspaper |
| Language: |
English |
| DOI: |
10.1136/jnnp-2021-326868 |
| Availability: |
https://doi.org/10.1136/jnnp-2021-326868; https://ora.ox.ac.uk/objects/uuid:d64766b7-d1cb-437a-9105-9b845a2b3ca7 |
| Rights: |
info:eu-repo/semantics/openAccess ; CC Attribution (CC BY) |
| Accession Number: |
edsbas.E104A86F |
| Database: |
BASE |