Title :

Language impairment in the genetic forms of behavioural variant frontotemporal dementia.

Author(s) :

Samra, K. [Auteur]
Macdougall, A. M. [Auteur]
Bouzigues, A. [Auteur]
Bocchetta, M. [Auteur]
Cash, D. M. [Auteur]
Greaves, C. V. [Auteur]
Convery, R. S. [Auteur]
Van Swieten, J. C. [Auteur]
Seelaar, H. [Auteur]
Jiskoot, L. [Auteur]
Moreno, F. [Auteur]
Sanchez-Valle, R. [Auteur]
Laforce, R. [Auteur]
Graff, C. [Auteur]
Masellis, M. [Auteur]
Tartaglia, M. C. [Auteur]
Rowe, J. B. [Auteur]
Borroni, B. [Auteur]
Finger, E. [Auteur]
Synofzik, M. [Auteur]
Galimberti, D. [Auteur]
Vandenberghe, R. [Auteur]
De Mendonça, A. [Auteur]
Butler, C. R. [Auteur]
Gerhard, A. [Auteur]
Ducharme, S. [Auteur]
Le Ber, I. [Auteur]
Tiraboschi, P. [Auteur]
Santana, I. [Auteur]
Pasquier, Florence [Auteur]
Lille Neurosciences & Cognition (LilNCog) - U 1172
Levin, J. [Auteur]
Otto, M. [Auteur]
Sorbi, S. [Auteur]
Rohrer, J. D. [Auteur]
University College of London [London] [UCL]
Russell, L. L. [Auteur]
University College of London [London] [UCL]

Journal title :

Journal of Neurology

Volume number :

270

Pages :

1976–1988

Publisher :

Springer Link

Publication date :

2022-12-21

ISSN :

1432-1459

Keyword(s) :

Frontotemporal dementia
Genetics
Language
Tau
Progranulin
C9orf72

HAL domain(s) :

Sciences du Vivant [q-bio]

English abstract : [en]

Background Behavioural variant fronto-temporal dementia (bvFTD) is characterised by a progressive change in personality in association with atrophy of the frontal and temporal lobes. Whilst language impairment has been ...
Show more >Background Behavioural variant fronto-temporal dementia (bvFTD) is characterised by a progressive change in personality in association with atrophy of the frontal and temporal lobes. Whilst language impairment has been described in people with bvFTD, little is currently known about the extent or type of linguistic difficulties that occur, particularly in the genetic forms. Methods Participants with genetic bvFTD along with healthy controls were recruited from the international multicentre Genetic FTD Initiative (GENFI). Linguistic symptoms were assessed using items from the Progressive Aphasia Severity Scale (PASS). Additionally, participants undertook the Boston Naming Test (BNT), modified Camel and Cactus Test (mCCT) and a category fluency test. Participants underwent a 3T volumetric T1-weighted MRI, with language network regional brain volumes measured and compared between the genetic groups and controls. Results 76% of the genetic bvFTD cohort had impairment in at least one language symptom: 83% C9orf72, 80% MAPT and 56% GRN mutation carriers. All three genetic groups had significantly impaired functional communication, decreased fluency, and impaired sentence comprehension. C9orf72 mutation carriers also had significantly impaired articulation and word retrieval as well as dysgraphia whilst the MAPT mutation group also had impaired word retrieval and single word comprehension. All three groups had difficulties with naming, semantic knowledge and verbal fluency. Atrophy in key left perisylvian language regions differed between the groups, with generalised involvement in the C9orf72 group and more focal temporal and insula involvement in the other groups. Correlates of language symptoms and test scores also differed between the groups. Conclusions Language deficits exist in a substantial proportion of people with familial bvFTD across all three genetic groups. Significant atrophy is seen in the dominant perisylvian language areas and correlates with language impairments within each of the genetic groups. Improved understanding of the language phenotype in the main genetic bvFTD subtypes will be helpful in future studies, particularly in clinical trials where accurate stratification and monitoring of disease progression is required.Show less >

Language :

Anglais

Audience :

Internationale

Popular science :

Non

Administrative institution(s) :

Université de Lille
Inserm
CHU Lille

Collections :

• Lille Neurosciences & Cognition (LilNCog) - U 1172

Submission date :

2024-01-16T00:03:23Z
2024-09-18T06:39:32Z