Comparison of clinical rating scales in genetic frontotemporal dementia within the GENFI cohort.
frontotemporal dementia
Journal
Journal of neurology, neurosurgery, and psychiatry
ISSN: 1468-330X
Titre abrégé: J Neurol Neurosurg Psychiatry
Pays: England
ID NLM: 2985191R
Informations de publication
Date de publication:
02 2022
02 2022
Historique:
received:
13
04
2021
accepted:
30
06
2021
pubmed:
7
8
2021
medline:
17
2
2022
entrez:
6
8
2021
Statut:
ppublish
Résumé
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. 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 ( Cross-sectionally, the mean FRS score was lower in all genetic groups compared with controls: Both 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.
Sections du résumé
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 (
RESULTS
Cross-sectionally, the mean FRS score was lower in all genetic groups compared with controls:
CONCLUSIONS
Both 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.
Identifiants
pubmed: 34353857
pii: jnnp-2021-326868
doi: 10.1136/jnnp-2021-326868
pmc: PMC8785074
doi:
Substances chimiques
C9orf72 Protein
0
MAPT protein, human
0
tau Proteins
0
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
158-168Subventions
Organisme : NIA NIH HHS
ID : U24 AG072122
Pays : United States
Organisme : Medical Research Council
ID : MR/M008525/1
Pays : United Kingdom
Organisme : Medical Research Council
ID : MR/M023664/1
Pays : United Kingdom
Organisme : Medical Research Council
ID : MR/T046015/1
Pays : United Kingdom
Organisme : Medical Research Council
ID : MR/J009482/1
Pays : United Kingdom
Organisme : Medical Research Council
ID : MC_UU_00024/1
Pays : United Kingdom
Organisme : NIA NIH HHS
ID : U01 AG016976
Pays : United States
Investigateurs
Sónia Afonso
(S)
Maria Rosario Almeida
(MR)
Sarah Anderl-Straub
(S)
Christin Andersson
(C)
Anna Antonell
(A)
Silvana Archetti
(S)
Andrea Arighi
(A)
Mircea Balasa
(M)
Myriam Barandiaran
(M)
Nuria Bargalló
(N)
Robart Bartha
(R)
Benjamin Bender
(B)
Alberto Benussi
(A)
Maxime Bertoux
(M)
Anne Bertrand
(A)
Valentina Bessi
(V)
Sandra Black
(S)
Martina Bocchetta
(M)
Sergi Borrego-Ecija
(S)
Jose Bras
(J)
Alexis Brice
(A)
Rose Bruffaerts
(R)
Agnès Camuzat
(A)
Marta Cañada
(M)
Valentina Cantoni
(V)
Paola Caroppo
(P)
David Cash
(D)
Miguel Castelo-Branco
(M)
Olivier Colliot
(O)
Thomas Cope
(T)
Vincent Deramecourt
(V)
María de Arriba
(M)
Giuseppe Di Fede
(GD)
Alina Díez
(A)
Diana Duro
(D)
Chiara Fenoglio
(C)
Camilla Ferrari
(C)
Catarina B Ferreira
(CB)
Nick Fox
(N)
Morris Freedman
(M)
Giorgio Fumagalli
(G)
Aurélie Funkiewiez
(A)
Alazne Gabilondo
(A)
Roberto Gasparotti
(R)
Serge Gauthier
(S)
Stefano Gazzina
(S)
Giorgio Giaccone
(G)
Ana Gorostidi
(A)
Caroline Greaves
(C)
Rita Guerreiro
(R)
Carolin Heller
(C)
Tobias Hoegen
(T)
Begoña Indakoetxea
(B)
Vesna Jelic
(V)
Hans-Otto Karnath
(HO)
Ron Keren
(R)
Tobias Langheinrich
(T)
Thibaud Lebouvier
(T)
Maria João Leitão
(MJ)
Albert Lladó
(A)
Gemma Lombardi
(G)
Sandra Loosli
(S)
Carolina Maruta
(C)
Simon Mead
(S)
Lieke Meeter
(L)
Gabriel Miltenberger
(G)
Rick van Minkelen
(RV)
Sara Mitchell
(S)
Katrina Moore
(K)
Benedetta Nacmias
(B)
Annabel Nelson
(A)
Linn Öijerstedt
(L)
Jaume Olives
(J)
Sebastien Ourselin
(S)
Alessandro Padovani
(A)
Jessica Panman
(J)
Janne M Papma
(JM)
Yolande Pijnenburg
(Y)
Cristina Polito
(C)
Enrico Premi
(E)
Sara Prioni
(S)
Catharina Prix
(C)
Rosa Rademakers
(R)
Veronica Redaelli
(V)
Daisy Rinaldi
(D)
Tim Rittman
(T)
Ekaterina Rogaeva
(E)
Adeline Rollin
(A)
Pedro Rosa-Neto
(P)
Giacomina Rossi
(G)
Martin Rossor
(M)
Beatriz Santiago
(B)
Dario Saracino
(D)
Sabrina Sayah
(S)
Elio Scarpini
(E)
Sonja Schönecker
(S)
Harro Seelaar
(H)
Elisa Semler
(E)
Rachelle Shafei
(R)
Christen Shoesmith
(C)
Imogen Swift
(I)
Miguel Tábuas-Pereira
(M)
Mikel Tainta
(M)
Ricardo Taipa
(R)
David Tang-Wai
(D)
David L Thomas
(DL)
Paul Thompson
(P)
Hakan Thonberg
(H)
Carolyn Timberlake
(C)
Pietro Tiraboschi
(P)
Emily Todd
(E)
Philip Van Damme
(PV)
Mathieu Vandenbulcke
(M)
Michele Veldsman
(M)
Ana Verdelho
(A)
Jorge Villanua
(J)
Jason Warren
(J)
Carlo Wilke
(C)
Ione Woollacott
(I)
Elisabeth Wlasich
(E)
Henrik Zetterberg
(H)
Miren Zulaica
(M)
Informations de copyright
© Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY. Published by BMJ.
Déclaration de conflit d'intérêts
Competing interests: None declared.
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