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Evaluation of the effect of transcranial direct current stimulation on language impairments in the behavioural variant of frontotemporal dementia
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In: Brain Commun (2022)
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Primary Progressive Aphasia Associated With GRN Mutations: New Insights Into the Non-amyloid Logopenic Variant
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Saracino, Dario; Ferrieux, Sophie; NOGUES-LASSIAILLE, Marie; Houot, Marion; FUNKIEWIEZ, Aurelie; Sellami, Leila; Deramecourt, Vincent; Pasquier, Florence; Couratier, Philippe; PARIENTE, Jeremie; GERAUDIE, Amandine; Epelbaum, Stephane; Wallon, David; Hannequin, Didier; Martinaud, Olivier; Clot, Fabienne; CAMUZAT, Agnes; Bottani, Simona; Rinaldi, Daisy; Auriacombe, Sophie; Sarazin, Marie; Didic, Mira; Boutoleau-Bretonnière, Claire; Thauvin-Robinet, Christel; Lagarde, Julien; ROUE-JAGOT, Carole; SELLAL, Francois; Gabelle, Audrey; ETCHARRY-BOUYX, Frederique; Morin, Alexandre; COPPOLA, Cinzia; Levy, Richard; Dubois, Bruno; Brice, Alexis; Colliot, Olivier; GORNO-TEMPINI, Maria Luisa; Teichmann, Marc; Migliaccio, Raffaella; Le Ber, Isabelle; FRENCH RESEARCH NETWORK ON, Ftd Ftd-Als
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In: ISSN: 0028-3878 ; EISSN: 1526-632X ; Neurology ; https://hal.archives-ouvertes.fr/hal-03281660 ; Neurology, American Academy of Neurology, 2021, ⟨10.1212/wnl.0000000000012174⟩ (2021)
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Abstract:
International audience ; OBJECTIVE: To determine relative frequencies and linguistic profiles of primary progressive aphasia (PPA) variants associated with progranulin (GRN) mutations, and study their neuroanatomical correlates. METHODS: PPA patients carrying GRN mutations (PPA-GRN) were selected amongst a national prospective research cohort of 1,696 frontotemporal dementia (FTD) patients, including 235 patients with PPA. All PPA patients with amyloid-positive CSF biomarkers were excluded. In this cross-sectional study, speech/language and cognitive profiles were characterized with standardized evaluations, and grey matter (GM) atrophy patterns using voxel-based morphometry. Comparisons were performed with controls, and sporadic PPA patients. RESULTS: Among the overall population of 235 patients, 45 (19%) carried GRN mutations. We studied 32 of these and showed that logopenic PPA (lvPPA) was the most frequent linguistic variant (13, 41%), followed by non-fluent/agrammatic (nfvPPA: 9, 28%) and mixed forms (8, 25%). Semantic variant was rather rare (2, 6%). LvPPA patients, qualified as non-amyloid-lvPPA, presented canonical logopenic deficit. Seven out of 13 had a pure form, six showed subtle additional linguistic deficits not fitting criteria for mixed PPA, hence labelled as "logopenic-spectrum variant". GM atrophy primarily involved left posterior temporal gyrus, mirroring neuroanatomical changes of amyloid-positive-lvPPA. NfvPPA patients presented agrammatism (89%) rather than apraxia of speech (11%). CONCLUSIONS: This study shows that most frequent PPA variant associated with GRN mutations is non-amyloid lvPPA, preceding nfvPPA and mixed forms, and illustrates that language network may be affected at different levels. GRN testing is indicated for PPA patients, whether familial or sporadic. This finding is important for upcoming GRN gene-specific therapies.
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Keyword:
[SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie; Dementia aphasia; Frontotemporal dementia
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URL: https://hal.archives-ouvertes.fr/hal-03281660/document https://hal.archives-ouvertes.fr/hal-03281660/file/NEUROLOGY-2020-153064v3-LeBer.pdf https://hal.archives-ouvertes.fr/hal-03281660 https://doi.org/10.1212/wnl.0000000000012174
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A Reliable and Rapid Language Tool for the Diagnosis, Classification, and Follow-Up of Primary Progressive Aphasia Variants
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In: Front Neurol (2021)
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The White Matter Module-Hub Network of Semantics Revealed by Semantic Dementia
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In: ISSN: 0898-929X ; EISSN: 1530-8898 ; Journal of Cognitive Neuroscience ; https://hal.inria.fr/hal-02554966 ; Journal of Cognitive Neuroscience, Massachusetts Institute of Technology Press (MIT Press), 2020, pp.1-18. ⟨10.1162/jocn_a_01549⟩ (2020)
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Cognitive and behavioural inhibition deficits in neurodegenerative dementias
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In: Cortex (2020)
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Language boosting by transcranial stimulation in progressive supranuclear palsy ; Stimulation du langage par stimulation transcrânienne dans la paralysie supranucléaire progressive
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In: ISSN: 0028-3878 ; EISSN: 1526-632X ; Neurology ; https://hal.archives-ouvertes.fr/hal-02415190 ; Neurology, American Academy of Neurology, 2019, 93 (6), pp.e537-e547. ⟨10.1212/WNL.0000000000007893⟩ (2019)
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Does surface dyslexia/dysgraphia relate to semantic deficits in the semantic variant of primary progressive aphasia?
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In: ISSN: 0028-3932 ; EISSN: 1873-3514 ; Neuropsychologia ; https://hal.archives-ouvertes.fr/hal-03488501 ; Neuropsychologia, Elsevier, 2019, 135, pp.107241 -. ⟨10.1016/j.neuropsychologia.2019.107241⟩ (2019)
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Testing the therapeutic effects of transcranial direct current stimulation (tDCS) in semantic dementia: a double blind, sham controlled, randomized clinical trial
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In: ISSN: 1745-6215 ; Trials ; https://hal.sorbonne-universite.fr/hal-02407657 ; Trials, BioMed Central, 2019, 20 (1), pp.632. ⟨10.1186/s13063-019-3613-z⟩ (2019)
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Alzheimer's disease including focal presentations
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In: ISSN: 0271-8235 ; EISSN: 1098-9021 ; Seminars in Neurology ; https://hal.sorbonne-universite.fr/hal-02271750 ; Seminars in Neurology, Thieme Publishing, 2019, 39 (02), pp.213-226. ⟨10.1055/s-0039-1681041⟩ (2019)
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Testing the therapeutic effects of transcranial direct current stimulation (tDCS) in semantic dementia: a double blind, sham controlled, randomized clinical trial ...
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Testing the therapeutic effects of transcranial direct current stimulation (tDCS) in semantic dementia: a double blind, sham controlled, randomized clinical trial ...
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Testing the therapeutic effects of transcranial direct current stimulation (tDCS) in semantic dementia: a double blind, sham controlled, randomized clinical trial
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Language boosting by transcranial stimulation in progressive supranuclear palsy
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In: Neurology (2019)
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Testing the therapeutic effects of transcranial direct current stimulation (tDCS) in semantic dementia: a double blind, sham controlled, randomized clinical trial
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Structural, Microstructural, and Metabolic Alterations in Primary Progressive Aphasia Variants
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In: ISSN: 1664-2295 ; Frontiers in Neurology ; https://hal.inria.fr/hal-01897015 ; Frontiers in Neurology, Frontiers, 2018, 9, ⟨10.3389/fneur.2018.00766⟩ (2018)
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Structural, microstructural and metabolic alterations in Primary Progressive Aphasia variants
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In: Annual meeting of the Organization for Human Brain Mapping - OHBM 2018 ; https://hal.inria.fr/hal-01764289 ; Annual meeting of the Organization for Human Brain Mapping - OHBM 2018, Jun 2018, Singapore, Singapore (2018)
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Prevalence of amyloid‐β pathology in distinct variants of primary progressive aphasia
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In: ISSN: 0364-5134 ; EISSN: 1531-8249 ; Annals of Neurology ; https://hal-univ-fcomte.archives-ouvertes.fr/hal-03630161 ; Annals of Neurology, Wiley, 2018, 84 (5), pp.729-740. ⟨10.1002/ana.25333⟩ (2018)
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Prevalence of amyloid‐β pathology in distinct variants of primary progressive aphasia
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In: ISSN: 0364-5134 ; EISSN: 1531-8249 ; Annals of Neurology ; https://www.hal.inserm.fr/inserm-02749861 ; Annals of Neurology, Wiley, 2018, 84 (5), pp.729-740. ⟨10.1002/ana.25333⟩ (2018)
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Parietal involvement in the semantic variant of primary progressive aphasia with Alzheimer's disease CSF profile
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In: ISSN: 1387-2877 ; Journal of Alzheimer's Disease ; https://hal.sorbonne-universite.fr/hal-01928504 ; Journal of Alzheimer's Disease, IOS Press, 2018, 66 (1), pp.271 - 280. ⟨10.3233/JAD-180087⟩ (2018)
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Linguistic characteristics of genetic primary progressive aphasias: a retrospective study of 27 cases carrying GRN and c9orf72 mutations
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In: European Journal of Neurology ; the 4th Congress of the European Academy of Neurology ; https://hal-univ-bourgogne.archives-ouvertes.fr/hal-02049007 ; the 4th Congress of the European Academy of Neurology, Jun 2018, Lisbonne, Portugal. pp.451 : EPR2129 ; https://onlinelibrary.wiley.com/toc/14681331/2018/25/S2 (2018)
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