scholarly journals Neural substrates of verbal repetition deficits in primary progressive aphasia

2020 ◽  
Author(s):  
Hilary E. Miller ◽  
Claire Cordella ◽  
Jessica A. Collins ◽  
Rania Ezzo ◽  
Megan Quimby ◽  
...  
Keyword(s):  
Cortical Thickness ◽  
Digit Span ◽  
Primary Progressive Aphasia ◽  
Span Task ◽  
Average Score ◽  
Cross Sectional ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Correlation Analyses ◽  
Verbal Repetition

AbstractIn this cross-sectional study, we examined the relationship between cortical thickness and performance on several verbal repetition tasks in a cohort of patients with primary progressive aphasia (PPA) in order to test predictions generated by theoretical accounts of phonological working memory (PWM) that predict phonological content buffers in left posterior inferior frontal sulcus (pIFS) and supramarginal gyrus (SMG). Cortical surfaces were reconstructed from magnetic resonance imaging scans from 42 participants diagnosed with PPA. Cortical thickness was measured in a set of anatomical regions spanning the entire cerebral cortex. Correlation analyses were performed between cortical thickness and average score across three PWM related tasks: the Repetition subtest from the Western Aphasia Battery, a forward digit span task, and a backward digit span task. Significant correlations were found between average PWM score across tasks and cortical thickness in left SMG and left pIFS, in support of prior theoretical accounts of PWM. Exploratory whole-brain correlation analyses performed for each of the three behavioral tasks individually revealed a distinct set of positively-correlated regions for each task. Comparison of cortical thickness measures from different PPA subtypes to cortical thickness in age-matched controls further revealed unique patterns of atrophy in the different PPA subtypes.

scholarly journals Neural substrates of verbal repetition deficits in primary progressive aphasia

2021 ◽  
Author(s):  
Hilary E Miller ◽  
Claire Cordella ◽  
Jessica A Collins ◽  
Rania Ezzo ◽  
Megan Quimby ◽  
...  
Keyword(s):  
Working Memory ◽  
Cortical Thickness ◽  
Digit Span ◽  
Primary Progressive Aphasia ◽  
Supramarginal Gyrus ◽  
Phonological Working Memory ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Left Posterior ◽  
Verbal Repetition

Abstract In this cross-sectional study, we examined the relationship between cortical thickness and performance on several verbal repetition tasks in a cohort of patients with primary progressive aphasia in order to test predictions generated by theoretical accounts of phonological working memory that predict phonological content buffers in left posterior inferior frontal sulcus and supramarginal gyrus. Cortical surfaces were reconstructed from magnetic resonance imaging scans from 42 participants diagnosed with primary progressive aphasia. Cortical thickness was measured in a set of anatomical regions spanning the entire cerebral cortex. Correlation analyses were performed between cortical thickness and average score across three phonological working memory related tasks: the Repetition subtest from the Western Aphasia Battery, a forward digit span task, and a backward digit span task. Significant correlations were found between average working memory score across tasks and cortical thickness in left supramarginal gyrus and left posterior inferior frontal sulcus, in support of prior theoretical accounts of phonological working memory. Exploratory whole-brain correlation analyses performed for each of the three behavioral tasks individually revealed a distinct set of positively-correlated regions for each task. Comparison of cortical thickness measures from different primary progressive aphasia subtypes to cortical thickness in age-matched controls further revealed unique patterns of atrophy in the different subtypes.

scholarly journals Infratentorial pathology in frontotemporal dementia: cerebellar grey and white matter alterations in FTD phenotypes

2021 ◽  
Author(s):  
Mary Clare McKenna ◽  
Rangariroyashe H. Chipika ◽  
Stacey Li Hi Shing ◽  
Foteini Christidi ◽  
Jasmin Lope ◽  
...  
Keyword(s):  
White Matter ◽  
Fractional Anisotropy ◽  
Cortical Thickness ◽  
Cerebellar Atrophy ◽  
Primary Progressive Aphasia ◽  
Radial Diffusivity ◽  
Axial Diffusivity ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Crus I

AbstractThe contribution of cerebellar pathology to cognitive and behavioural manifestations is increasingly recognised, but the cerebellar profiles of FTD phenotypes are relatively poorly characterised. A prospective, single-centre imaging study has been undertaken with a high-resolution structural and diffusion tensor protocol to systematically evaluate cerebellar grey and white matter alterations in behavioural-variant FTD(bvFTD), non-fluent variant primary progressive aphasia(nfvPPA), semantic-variant primary progressive aphasia(svPPA), C9orf72-positive ALS-FTD(C9 + ALSFTD) and C9orf72-negative ALS-FTD(C9-ALSFTD). Cerebellar cortical thickness and complementary morphometric analyses were carried out to appraise atrophy patterns controlling for demographic variables. White matter integrity was assessed in a study-specific white matter skeleton, evaluating three diffusivity metrics: fractional anisotropy (FA), axial diffusivity (AD) and radial diffusivity (RD). Significant cortical thickness reductions were identified in: lobule VII and crus I in bvFTD; lobule VI VII, crus I and II in nfvPPA; and lobule VII, crus I and II in svPPA; lobule IV, VI, VII and Crus I and II in C9 + ALSFTD. Morphometry revealed volume reductions in lobule V in all groups; in addition to lobule VIII in C9 + ALSFTD; lobule VI, VIII and vermis in C9-ALSFTD; lobule V, VII and vermis in bvFTD; and lobule V, VI, VIII and vermis in nfvPPA. Widespread white matter alterations were demonstrated by significant fractional anisotropy, axial diffusivity and radial diffusivity changes in each FTD phenotype that were more focal in those with C9 + ALSFTD and svPPA. Our findings indicate that FTD subtypes are associated with phenotype-specific cerebellar signatures with the selective involvement of specific lobules instead of global cerebellar atrophy.

Spanish Version of the Mini-Linguistic State Examination for the Diagnosis of Primary Progressive Aphasia

2021 ◽  
pp. 1-8
Author(s):  
Jordi A. Matias-Guiu ◽  
Vanesa Pytel ◽  
Laura Hernández-Lorenzo ◽  
Nikil Patel ◽  
Katie A. Peterson ◽  
...  
Keyword(s):  
Primary Progressive Aphasia ◽  
Spanish Version ◽  
Classification Model ◽  
Healthy Controls ◽  
Language Test ◽  
Cross Sectional ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Clinical Variants ◽  
State Examination

Background: Primary progressive aphasia (PPA) is a neurodegenerative syndrome with three main clinical variants: non-fluent, semantic, and logopenic. Clinical diagnosis and accurate classification are challenging and often time-consuming. The Mini-Linguistic State Examination (MLSE) has been recently developed as a short language test to specifically assess language in neurodegenerative disorders. Objective: Our aim was to adapt and validate the Spanish version of MLSE for PPA diagnosis. Methods: Cross-sectional study involving 70 patients with PPA and 42 healthy controls evaluated with the MLSE. Patients were independently diagnosed and classified according to comprehensive cognitive evaluation and advanced neuroimaging. Results: Internal consistency was 0.758. The influence of age and education was very low. The area under the curve for discriminating PPA patients and healthy controls was 0.99. Effect sizes were moderate-large for the discrimination between PPA and healthy controls. Motor speech, phonology, and semantic subscores discriminated between the three clinical variants. A random forest classification model obtained an F1-score of 81%for the three PPA variants. Conclusion: Our study provides a brief and useful language test for PPA diagnosis, with excellent properties for both clinical routine assessment and research purposes.

scholarly journals Differentiation between Subtypes of Primary Progressive Aphasia by Using Cortical Thickness and Diffusion-Tensor MR Imaging Measures

Radiology ◽  
2015 ◽  
Vol 276 (1) ◽  
pp. 219-227 ◽  
Author(s):  
Federica Agosta ◽  
Pilar M. Ferraro ◽  
Elisa Canu ◽  
Massimiliano Copetti ◽  
Sebastiano Galantucci ◽  
...  
Keyword(s):  
Mr Imaging ◽  
Cortical Thickness ◽  
Diffusion Tensor ◽  
Primary Progressive Aphasia ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
And Diffusion ◽  
Diffusion Tensor Mr Imaging

Verbal Repetition in Primary Progressive Aphasia and Alzheimer's Disease

2014 ◽  
Vol 41 (2) ◽  
pp. 575-585 ◽  
Author(s):  
Cristian E. Leyton ◽  
Sharon Savage ◽  
Muireann Irish ◽  
Samantha Schubert ◽  
Olivier Piguet ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Primary Progressive Aphasia ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Verbal Repetition

scholarly journals Speech production differences in English and Italian speakers with nonfluent variant PPA

Neurology ◽  
2020 ◽  
Vol 94 (10) ◽  
pp. e1062-e1072 ◽  
Author(s):  
Elisa Canu ◽  
Federica Agosta ◽  
Giovanni Battistella ◽  
Edoardo G. Spinelli ◽  
Jessica DeLeon ◽  
...  
Keyword(s):  
Gray Matter Volume ◽  
Primary Progressive Aphasia ◽  
Cross Sectional Study ◽  
Speech Impairment ◽  
Neuropsychological Battery ◽  
Connected Speech ◽  
Cross Sectional ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Language Measures

ObjectiveTo understand whether the clinical phenotype of nonfluent/agrammatic primary progressive aphasia (nfvPPA) could present differences depending on the patient’s native language.MethodsIn this cross-sectional study, we analyzed connected speech samples in monolingual English (nfvPPA-E) and Italian speakers (nfvPPA-I) who were diagnosed with nfvPPA and matched for age, sex, and Mini-Mental State Examination scores. Patients also received a comprehensive neuropsychological battery. All patients and 2 groups of age-matched healthy controls underwent an MRI scan with 3D T1-weighted sequences. Connected speech measures and the other cognitive features were compared between patient groups. MRI variables, in terms of gray matter volume, were compared between each patient group and the corresponding controls.ResultsCompared to nfvPPA-E, nfvPPA-I had fewer years of education and shorter reported disease duration. The 2 groups showed similar regional atrophy compatible with clinical diagnosis. Patients did not differ in nonlanguage domains, comprising executive scores. Connected speech sample analysis showed that nfvPPA-E had significantly more distortions than nfvPPA-I, while nfvPPA-I showed reduced scores in some measures of syntactic complexity. On language measures, Italian speakers performed more poorly on syntactic comprehension.ConclusionsnfvPPA-E showed greater motor speech impairment than nfvPPA-I despite higher level of education and comparable disease severity and atrophy changes. The data also suggest greater grammatical impairment in nfvPPA-I. This study illustrates the need to take into account the possible effect of the individual's spoken language on the phenotype and clinical presentation of primary progressive aphasia variants.

scholarly journals The Course of Primary Progressive Aphasia Diagnosis : A Cross-Sectional Study.

2021 ◽  
Author(s):  
Aurélie Mouton ◽  
Alexandra PLONKA ◽  
Roxane FABRE ◽  
Maï Tran ◽  
Philippe ROBERT ◽  
...  
Keyword(s):  
Primary Progressive Aphasia ◽  
Data Bank ◽  
Cross Sectional Study ◽  
Diagnosis Delay ◽  
Sectional Study ◽  
Pharmacological Treatments ◽  
Cross Sectional ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
First Diagnosis

Abstract BackgroundThe Primary Progressive Aphasia (PPA) diagnosis trajectory is debated, as several changes in diagnosis occur during PPA course, due to phenotype evolution from isolated language alterations to global cognitive impairment.The goal of the present study, based on a French cohort was to describe the demographics and the evolution of subjects with (PPA) in comparison with Alzheimer Disease (AD) on a period of seven years. MethodsWe conducted a repeated cross-sectional study. The study population comprised individuals with PPA and AD diagnosis (N=167191) from 2010 to 2016 in the French National data Bank (BNA). Demographic variables, MMSE scores, diagnosis status at each visit and prescribed treatments were considered.ResultsFrom 2010 to 2016, 5186 patients were initially diagnosed with PPA, 162005 with AD. Compared to AD subjects, significant differences were found concerning: age (younger at first diagnosis for PPA), gender (more balanced in PPA), education level (higher in PPA) and MMSE score (higher in PPA). Percentage of pending diagnosis, delay between first consultation and first diagnosis and the number of different diagnoses before the diagnosis of interest were significantly higher in PPA group compared to AD group. Pharmacological and non-pharmacological treatments were significatively more recommended following PPA than AD diagnosis.ConclusionThis study improves the knowledge of PPA epidemiology and has the potential to help adopting appropriate public health service policies. It supports the hypothesis that PPA is diagnosed later than AD. The PPA diagnosis increases the prescription of non-pharmacological treatments, especially speech and language therapy (SLT) that is the main treatment available and most effective when at the initial stage.Trial registrationClinicalTrials.gov identifier NCT03687112

Primary Progressive Aphasia Associated With GRN Mutations: New Insights Into the Non-amyloid Logopenic Variant

Neurology ◽  
2021 ◽  
pp. 10.1212/WNL.0000000000012174
Author(s):  
Dario Saracino ◽  
Sophie Ferrieux ◽  
Marie Noguès-Lassiaille ◽  
Marion Houot ◽  
Aurélie Funkiewiez ◽  
...  
Keyword(s):  
Primary Progressive Aphasia ◽  
Cross Sectional Study ◽  
Csf Biomarkers ◽  
Cognitive Profiles ◽  
Apraxia Of Speech ◽  
Cross Sectional ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Left Posterior ◽  
Language Network

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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