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@article{Peakman:2022:10.1136/jnnp-2021-326868,
author = {Peakman, G and Russell, LL and Convery, RS and Nicholas, JM and Van, Swieten JC and Jiskoot, LC and Moreno, F and Sanchez-Valle, R and Laforce, R and Graff, C and Masellis, M and Tartaglia, MC and Rowe, JB and Borroni, B and Finger, E and Synofzik, M and Galimberti, D and Vandenberghe, R and de, Mendonça A and Butler, CR and Gerhard, A and Ducharme, S and Le, Ber I and Tagliavini, F and Santana, I and Pasquier, F and Levin, J and Danek, A and Otto, M and Sorbi, S and Rohrer, JD and Genetic, FTD Initiative GENFI},
doi = {10.1136/jnnp-2021-326868},
journal = {Journal of Neurology, Neurosurgery and Psychiatry},
pages = {158--168},
title = {Comparison of clinical rating scales in genetic frontotemporal dementia within the GENFI cohort.},
url = {http://dx.doi.org/10.1136/jnnp-2021-326868},
volume = {93},
year = {2022}
}

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TY  - JOUR
AB  - 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<0.001 for all comparisons, while the mean CDR+NACC FTLD Sum of Boxes was significantly higher in all genetic groups: GRN mutation carriers mean 2.6 (5.2), MAPT mutation carriers 3.2 (5.6), C9orf72 mutation carriers 4.2 (6.2), controls 0.2 (0.6), p<0.001 for all comparisons. Mean FRS score decreased and CDR+NACC FTLD Sum of Boxes increased with increasing disease severity within each individual genetic group. FRS and CDR+NACC FTLD Sum of Boxes scores were strongly negatively correlated across all mutation carriers (rs=-0.77, p<0.001) and within each genetic group (rs=-0.67 to -0.81, p<0.001 in each group). Nonetheless, discrepancies in disease staging were seen between the scales, and with each scale and clinician-judged symptomatic status. Longitudinally, annualised change in both FRS and CDR+NACC FTLD Sum of Boxes scores initially increased
AU  - Peakman,G
AU  - Russell,LL
AU  - Convery,RS
AU  - Nicholas,JM
AU  - Van,Swieten JC
AU  - Jiskoot,LC
AU  - Moreno,F
AU  - Sanchez-Valle,R
AU  - Laforce,R
AU  - Graff,C
AU  - Masellis,M
AU  - Tartaglia,MC
AU  - Rowe,JB
AU  - Borroni,B
AU  - Finger,E
AU  - Synofzik,M
AU  - Galimberti,D
AU  - Vandenberghe,R
AU  - de,Mendonça A
AU  - Butler,CR
AU  - Gerhard,A
AU  - Ducharme,S
AU  - Le,Ber I
AU  - Tagliavini,F
AU  - Santana,I
AU  - Pasquier,F
AU  - Levin,J
AU  - Danek,A
AU  - Otto,M
AU  - Sorbi,S
AU  - Rohrer,JD
AU  - Genetic,FTD Initiative GENFI
DO  - 10.1136/jnnp-2021-326868
EP  - 168
PY  - 2022///
SN  - 0022-3050
SP  - 158
TI  - Comparison of clinical rating scales in genetic frontotemporal dementia within the GENFI cohort.
T2  - Journal of Neurology, Neurosurgery and Psychiatry
UR  - http://dx.doi.org/10.1136/jnnp-2021-326868
UR  - https://www.ncbi.nlm.nih.gov/pubmed/34353857
UR  - https://jnnp.bmj.com/content/early/2021/08/04/jnnp-2021-326868
UR  - http://hdl.handle.net/10044/1/91151
VL  - 93
ER  -

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