SwePub
Sök i LIBRIS databas

  Utökad sökning

WFRF:(Ducharme F)
 

Sökning: WFRF:(Ducharme F) > (2020-2024) > Comparison of clini...

LIBRIS Formathandbok  (Information om MARC21)
FältnamnIndikatorerMetadata
00004997naa a2200625 4500
001oai:prod.swepub.kib.ki.se:148249261
003SwePub
008240701s2022 | |||||||||||000 ||eng|
024a http://kipublications.ki.se/Default.aspx?queryparsed=id:1482492612 URI
024a https://doi.org/10.1136/jnnp-2021-3268682 DOI
040 a (SwePub)ki
041 a engb eng
042 9 SwePub
072 7a ref2 swepub-contenttype
072 7a art2 swepub-publicationtype
100a Peakman, G4 aut
2451 0a Comparison of clinical rating scales in genetic frontotemporal dementia within the GENFI cohort
264 c 2021-08-05
264 1b BMJ,c 2022
520 a 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.MethodsThe 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.ResultsCross-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 with disease severity level before decreasing in those with the most severe disease: controls −0.1 (6.0) for FRS, −0.1 (0.4) for CDR+NACC FTLD Sum of Boxes, asymptomatic mutation carriers −0.5 (8.2), 0.2 (0.9), prodromal disease −2.3 (9.9), 0.6 (2.7), mild disease −10.2 (18.6), 3.0 (4.1), moderate disease −9.6 (16.6), 4.4 (4.0), severe disease −2.7 (8.3), 1.7 (3.3). Sample sizes were calculated for a trial of prodromal mutation carriers: over 180 participants per arm would be needed to detect a moderate sized effect (30%) for both outcome measures, with sample sizes lower for the FRS.ConclusionsBoth the FRS and CDR+NACC FTLD measure disease severity in genetic FTD mutation carriers throughout the timeline of their disease, although the FRS may be preferable as an outcome measure. However, neither address a number of key symptoms in the FTD spectrum, for example, motor and neuropsychiatric deficits, which future scales will need to incorporate.
700a Russell, LL4 aut
700a Convery, RS4 aut
700a Nicholas, JM4 aut
700a Van Swieten, JC4 aut
700a Jiskoot, LC4 aut
700a Moreno, F4 aut
700a Sanchez-Valle, R4 aut
700a Laforce, R4 aut
700a Graff, Cu Karolinska Institutet4 aut
700a Masellis, M4 aut
700a Tartaglia, MC4 aut
700a Rowe, JB4 aut
700a Borroni, B4 aut
700a Finger, E4 aut
700a Synofzik, M4 aut
700a Galimberti, D4 aut
700a Vandenberghe, R4 aut
700a de Mendonca, A4 aut
700a Butler, CR4 aut
700a Gerhard, A4 aut
700a Ducharme, S4 aut
700a Le Ber, I4 aut
700a Tagliavini, F4 aut
700a Santana, I4 aut
700a Pasquier, F4 aut
700a Levin, J4 aut
700a Danek, A4 aut
700a Otto, M4 aut
700a Sorbi, S4 aut
700a Rohrer, JD4 aut
710a Karolinska Institutet4 org
773t Journal of neurology, neurosurgery, and psychiatryd : BMJg 93:2, s. 158-168q 93:2<158-168x 1468-330Xx 0022-3050
856u https://jnnp.bmj.com/content/jnnp/early/2021/08/04/jnnp-2021-326868.full.pdf
8564 8u http://kipublications.ki.se/Default.aspx?queryparsed=id:148249261
8564 8u https://doi.org/10.1136/jnnp-2021-326868

Hitta via bibliotek

Till lärosätets databas

Kungliga biblioteket hanterar dina personuppgifter i enlighet med EU:s dataskyddsförordning (2018), GDPR. Läs mer om hur det funkar här.
Så här hanterar KB dina uppgifter vid användning av denna tjänst.

 
pil uppåt Stäng

Kopiera och spara länken för att återkomma till aktuell vy