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Cortical microstructure in primary progressive aphasia: a multicenter study.
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In: Alzheimer's research & therapy, vol 14, iss 1 (2022)
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Neuroanatomical correlations of visuospatial processing in primary progressive aphasia
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In: Brain Commun (2022)
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Editorial: The Unusual Suspects: Linguistic Deficits in Non-Language-Dominant Neurodegenerative Diseases
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In: Front Aging Neurosci (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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Uniform data set language measures for bvFTD and PPA diagnosis and monitoring.
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In: Alzheimer's & dementia (Amsterdam, Netherlands), vol 13, iss 1 (2021)
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Uniform data set language measures for bvFTD and PPA diagnosis and monitoring.
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In: Alzheimer's & dementia (Amsterdam, Netherlands), vol 13, iss 1 (2021)
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Speech Metrics and Samples That Differentiate Between Nonfluent/Agrammatic and Logopenic Variants of Primary Progressive Aphasia
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In: J Speech Lang Hear Res (2021)
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Treatment for Anomia in Bilingual Speakers with Progressive Aphasia
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In: Brain Sci (2021)
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Cortical Tracking of the Speech Envelope in Logopenic Variant Primary Progressive Aphasia
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In: Front Hum Neurosci (2021)
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Uniform data set language measures for bvFTD and PPA diagnosis and monitoring
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In: Alzheimers Dement (Amst) (2021)
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Association of APOE4 and Clinical Variability in Alzheimer Disease With the Pattern of Tau- and Amyloid-PET
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In: Neurology (2021)
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Connected speech markers of amyloid burden in primary progressive aphasia
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Diagnostic Assessment in Primary Progressive Aphasia: An Illustrative Case Example
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Deformation-based shape analysis of the hippocampus in the semantic variant of primary progressive aphasia and Alzheimer's disease.
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Deformation-based shape analysis of the hippocampus in the semantic variant of primary progressive aphasia and Alzheimer's disease.
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Data-Driven, Visual Framework for the Characterization of Aphasias Across Stroke, Post-resective, and Neurodegenerative Disorders Over Time.
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Speech and language therapy approaches to managing primary progressive aphasia
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Primary progressive aphasia and the FTD-MND spectrum disorders: clinical, pathological, and neuroimaging correlates ...
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