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

Author(s):  
Hilary E Miller ◽  
Claire Cordella ◽  
Jessica A Collins ◽  
Rania Ezzo ◽  
Megan Quimby ◽  
...  

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.

2020 ◽  
Author(s):  
Hilary E. Miller ◽  
Claire Cordella ◽  
Jessica A. Collins ◽  
Rania Ezzo ◽  
Megan Quimby ◽  
...  

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.


Author(s):  
Mary Clare McKenna ◽  
Rangariroyashe H. Chipika ◽  
Stacey Li Hi Shing ◽  
Foteini Christidi ◽  
Jasmin Lope ◽  
...  

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.


2019 ◽  
Vol 34 (7) ◽  
pp. 1290-1290
Author(s):  
L Perez

Abstract Objective Often, individuals with lower educational attainment and limited proficiency in the English language get misdiagnosed and/or undertreated, which can impact their quality of life and other outcomes. The present case study intends to review and discuss the presentation of a monolingual, Spanish-speaking woman with Semantic Variant Primary Progressive Aphasia (svPPA), who was originally referred for a neuropsychological evaluation to determine the severity of her existing Alzheimer’s Disease (AD) diagnosis. Case Description Ms. X, is a 64-year-old, right-handed Hispanic woman with 6 years of education. Symptoms included forgetfulness, restlessness, and insomnia. Her family reported that she was repeating her ideas frequently during conversations and failing to recognize previously acquainted people, including her own relatives. A recent MRI of the brain showed anterior temporal lobe atrophy. Diagnostic Impressions and Outcomes Overall, she showed naming deficits (anomia), impaired verbal fluency, surface dyslexia, and significant problems with comprehension. Executive functioning, sentence repetition, working memory, and attention were generally intact. Qualitatively, her speech was apparently fluent and automatic, yet clearly empty in meaning. In Ms. X’s case, collateral reports of word-finding difficulties, tendency to repeat her thoughts incessantly, associative agnosia and prosopagnosia, and spared repetition and motor speech are strongly indicative of svPPA. Discussion svPPA primarily impacts language production and comprehension, and is characterized by severe anomia, word-finding difficulties, impaired single word comprehension, and in some cases, defective recognition of familiar faces. On testing, impairments can be observed in confrontation naming, with motor speech and repetition, working memory, episodic memory, visuospatial skills, and problem-solving skills relatively intact. Language symptoms are thought to stem from deficits of the semantic system.


Radiology ◽  
2015 ◽  
Vol 276 (1) ◽  
pp. 219-227 ◽  
Author(s):  
Federica Agosta ◽  
Pilar M. Ferraro ◽  
Elisa Canu ◽  
Massimiliano Copetti ◽  
Sebastiano Galantucci ◽  
...  

2014 ◽  
Vol 41 (2) ◽  
pp. 575-585 ◽  
Author(s):  
Cristian E. Leyton ◽  
Sharon Savage ◽  
Muireann Irish ◽  
Samantha Schubert ◽  
Olivier Piguet ◽  
...  

2020 ◽  
Vol 16 (S6) ◽  
Author(s):  
Seckin Arslan ◽  
Alexandra Plonka ◽  
Magali Payne Cogordan ◽  
Valeria Manera ◽  
Auriane Gros ◽  
...  

2021 ◽  
Vol 15 (3) ◽  
pp. 405-412
Author(s):  
Joel Macoir ◽  
Vicent Martel-Sauvageau ◽  
Liziane Bouvier ◽  
Robert Laforce ◽  
Laura Monetta

ABSTRACT. The differential diagnosis of primary progressive aphasia (PPA) is challenging due to overlapping clinical manifestations of the different variants of the disease. This is particularly true for the logopenic variant of PPA (lvPPA), in which such overlap was reported with regard to impairments in repetition abilities. In this study, four individuals with lvPPA underwent standard neuropsychological and language assessments. The influence of psycholinguistic variables on their performance of in word, nonword and sentence repetition tasks was also specifically explored. Some level of heterogeneity was found in cognitive functions and in language. The four participants showed impairment in sentence repetition in which their performance was negatively affected by semantic reversibility and syntactic complexity. This study supports the heterogeneity of lvPPA with respect to the cognitive and linguistic status of participants. It also shows that sentence repetition is influenced not only by length, but also by semantic reversibility and syntactic complexity, two psycholinguistic variables known to place additional demands on phonological working memory.


2018 ◽  
Vol 92 ◽  
pp. 243-254 ◽  
Author(s):  
Willem S. Eikelboom ◽  
Nikki Janssen ◽  
Lize C. Jiskoot ◽  
Esther van den Berg ◽  
Ardi Roelofs ◽  
...  

Neurology ◽  
2021 ◽  
pp. 10.1212/WNL.0000000000012174
Author(s):  
Dario Saracino ◽  
Sophie Ferrieux ◽  
Marie Noguès-Lassiaille ◽  
Marion Houot ◽  
Aurélie Funkiewiez ◽  
...  

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.


Sign in / Sign up

Export Citation Format

Share Document