scholarly journals Quantification of motor speech impairment and its anatomic basis in primary progressive aphasia

Neurology ◽  
2019 ◽  
Vol 92 (17) ◽  
pp. e1992-e2004 ◽  
Author(s):  
Claire Cordella ◽  
Megan Quimby ◽  
Alexandra Touroutoglou ◽  
Michael Brickhouse ◽  
Bradford C. Dickerson ◽  
...  
Keyword(s):  
Quantitative Measure ◽  
Primary Progressive Aphasia ◽  
Cortical Atrophy ◽  
Speech Impairment ◽  
Motor Speech ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Mild Disease ◽  
Rate Of Decline ◽  
Evaluation And Monitoring

ObjectiveTo evaluate whether a quantitative speech measure is effective in identifying and monitoring motor speech impairment (MSI) in patients with primary progressive aphasia (PPA) and to investigate the neuroanatomical basis of MSI in PPA.MethodsSixty-four patients with PPA were evaluated at baseline, with a subset (n = 39) evaluated longitudinally. Articulation rate (AR), a quantitative measure derived from spontaneous speech, was measured at each time point. MRI was collected at baseline. Differences in baseline AR were assessed across PPA subtypes, separated by severity level. Linear mixed-effects models were conducted to assess groups differences across PPA subtypes in rate of decline in AR over a 1-year period. Cortical thickness measured from baseline MRIs was used to test hypotheses about the relationship between cortical atrophy and MSI.ResultsBaseline AR was reduced for patients with nonfluent variant PPA (nfvPPA) compared to other PPA subtypes and controls, even in mild stages of disease. Longitudinal results showed a greater rate of decline in AR for the nfvPPA group over 1 year compared to the logopenic and semantic variant subgroups. Reduced baseline AR was associated with cortical atrophy in left-hemisphere premotor and supplementary motor cortices.ConclusionsThe AR measure is an effective quantitative index of MSI that detects MSI in mild disease stages and tracks decline in MSI longitudinally. The AR measure also demonstrates anatomic localization to motor speech–specific cortical regions. Our findings suggest that this quantitative measure of MSI might have utility in diagnostic evaluation and monitoring of MSI in PPA.

Motor Speech Phenotypes of Frontotemporal Dementia, Primary Progressive Aphasia, and Progressive Apraxia of Speech

2017 ◽  
Vol 60 (4) ◽  
pp. 897-911 ◽  
Author(s):  
Matthew L. Poole ◽  
Amy Brodtmann ◽  
David Darby ◽  
Adam P. Vogel
Keyword(s):  
Frontotemporal Dementia ◽  
Meta Analysis ◽  
Primary Progressive Aphasia ◽  
Control Group ◽  
Apraxia Of Speech ◽  
Speech Impairment ◽  
Motor Speech ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Neuroimaging Data

Purpose Our purpose was to create a comprehensive review of speech impairment in frontotemporal dementia (FTD), primary progressive aphasia (PPA), and progressive apraxia of speech in order to identify the most effective measures for diagnosis and monitoring, and to elucidate associations between speech and neuroimaging. Method Speech and neuroimaging data described in studies of FTD and PPA were systematically reviewed. A meta-analysis was conducted for speech measures that were used consistently in multiple studies. Results The methods and nomenclature used to describe speech in these disorders varied between studies. Our meta-analysis identified 3 speech measures which differentiate variants or healthy control-group participants (e.g., nonfluent and logopenic variants of PPA from all other groups, behavioral-variant FTD from a control group). Deficits within the frontal-lobe speech networks are linked to motor speech profiles of the nonfluent variant of PPA and progressive apraxia of speech. Motor speech impairment is rarely reported in semantic and logopenic variants of PPA. Limited data are available on motor speech impairment in the behavioral variant of FTD. Conclusions Our review identified several measures of speech which may assist with diagnosis and classification, and consolidated the brain–behavior associations relating to speech in FTD, PPA, and progressive apraxia of speech.

Clinical Characteristic in Primary Progressive Aphasia in Relation to Alzheimer’s Disease Biomarkers

2021 ◽  
pp. 1-13
Author(s):  
Sung Hoon Kang ◽  
Hanna Cho ◽  
Jiho Shin ◽  
Hang-Rai Kim ◽  
Young Noh ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Language Impairment ◽  
Amyloid Β ◽  
Primary Progressive Aphasia ◽  
Cortical Atrophy ◽  
Clinical Feature ◽  
Progressive Aphasia ◽  
Disease Biomarkers ◽  
Primary Progressive

Background: Primary progressive aphasia (PPA) is associated with amyloid-β (Aβ) pathology. However, clinical feature of PPA based on Aβ positivity remains unclear. Objective: We aimed to assess the prevalence of Aβ positivity in patients with PPA and compare the clinical characteristics of patients with Aβ-positive (A+) and Aβ-negative (A–) PPA. Further, we applied Aβ and tau classification system (AT system) in patients with PPA for whom additional information of in vivo tau biomarker was available. Methods: We recruited 110 patients with PPA (41 semantic [svPPA], 27 non-fluent [nfvPPA], 32 logopenic [lvPPA], and 10 unclassified [ucPPA]) who underwent Aβ-PET imaging at multi centers. The extent of language impairment and cortical atrophy were compared between the A+ and A–PPA subgroups using general linear models. Results: The prevalence of Aβ positivity was highest in patients with lvPPA (81.3%), followed by ucPPA (60.0%), nfvPPA (18.5%), and svPPA (9.8%). The A+ PPA subgroup manifested cortical atrophy mainly in the left superior temporal/inferior parietal regions and had lower repetition scores compared to the A–PPA subgroup. Further, we observed that more than 90%(13/14) of the patients with A+ PPA had tau deposition. Conclusion: Our findings will help clinicians understand the patterns of language impairment and cortical atrophy in patients with PPA based on Aβ deposition. Considering that most of the A+ PPA patents are tau positive, understanding the influence of Alzheimer’s disease biomarkers on PPA might provide an opportunity for these patients to participate in clinical trials aimed for treating atypical Alzheimer’s disease.

scholarly journals A Case of a Monolingual Spanish-Speaking Latina with Semantic Variant Primary Progressive Aphasia (svPPA)

2019 ◽  
Vol 34 (7) ◽  
pp. 1290-1290
Author(s):  
L Perez
Keyword(s):  
Working Memory ◽  
Language Production ◽  
Primary Progressive Aphasia ◽  
Motor Speech ◽  
Problem Solving Skills ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Spanish Speaking ◽  
Word Finding ◽  
Semantic Variant

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.

scholarly journals Asymmetric TDP pathology in primary progressive aphasia with right hemisphere language dominance

Neurology ◽  
2018 ◽  
Vol 90 (5) ◽  
pp. e396-e403 ◽  
Author(s):  
Garam Kim ◽  
Shahrooz Vahedi ◽  
Tamar Gefen ◽  
Sandra Weintraub ◽  
Eileen H. Bigio ◽  
...  
Keyword(s):  
Right Hemisphere ◽  
Primary Progressive Aphasia ◽  
Cortical Atrophy ◽  
Progressive Aphasia ◽  
Language Dominance ◽  
Primary Progressive ◽  
Activated Microglia ◽  
Unbiased Stereology ◽  
Cortical Regions ◽  
The Right

ObjectiveTo quantitatively examine the regional densities and hemispheric distribution of the 43-kDa transactive response DNA-binding protein (TDP-43) inclusions, neurons, and activated microglia in a left-handed patient with right hemisphere language dominance and logopenic-variant primary progressive aphasia (PPA).MethodsPhosphorylated TDP-43 inclusions, neurons, and activated microglia were visualized with immunohistochemical and histologic methods. Markers were quantified bilaterally with unbiased stereology in language- and memory-related cortical regions.ResultsClinical MRI indicated cortical atrophy in the right hemisphere, mostly in the temporal lobe. Significantly higher densities of TDP-43 inclusions were present in right language-related temporal regions compared to the left or to other right hemisphere regions. The memory-related entorhinal cortex (ERC) and language regions without significant atrophy showed no asymmetry. Activated microglia displayed extensive asymmetry (R > L). A substantial density of neurons remained in all areas and showed no hemispheric asymmetry. However, perikaryal size was significantly smaller in the right hemisphere across all regions except the ERC. To demonstrate the specificity of this finding, sizes of residual neurons were measured in a right-handed case with PPA and were found to be smaller in the language-dominant left hemisphere.ConclusionsThe distribution of TDP-43 inclusions and microglial activation in right temporal language regions showed concordance with anatomic distribution of cortical atrophy and clinical presentation. The results revealed no direct relationship between density of TDP-43 inclusions and activated microglia. Reduced size of the remaining neurons is likely to contribute to cortical atrophy detected by MRI. These findings support the conclusion that there is no obligatory relationship between logopenic PPA and Alzheimer pathology.

scholarly journals Leukoaraiosis severity predicts rate of decline in primary progressive aphasia

Aphasiology ◽  
2019 ◽  
Vol 34 (3) ◽  
pp. 365-375 ◽  
Author(s):  
Adam Odolil ◽  
Amy E. Wright ◽  
Lynsey M. Keator ◽  
Shannon M. Sheppard ◽  
Bonnie Breining ◽  
...  
Keyword(s):  
Primary Progressive Aphasia ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Rate Of Decline

PROMINENT MICROGLIAL ACTIVATION IN CORTICAL WHITE MATTER IS SELECTIVELY ASSOCIATED WITH CORTICAL ATROPHY IN PRIMARY PROGRESSIVE APHASIA

2017 ◽  
Vol 13 (7) ◽  
pp. P1499
Author(s):  
Daniel T. Ohm ◽  
Garam Kim ◽  
Tamar Gefen ◽  
Alfred Rademaker ◽  
Sandra Weintraub ◽  
...  
Keyword(s):  
White Matter ◽  
Microglial Activation ◽  
Primary Progressive Aphasia ◽  
Cortical Atrophy ◽  
Progressive Aphasia ◽  
Primary Progressive

scholarly journals Lack of Frank Agrammatism in the Nonfluent Agrammatic Variant of Primary Progressive Aphasia

2016 ◽  
Vol 6 (3) ◽  
pp. 407-423 ◽  
Author(s):  
Naida L. Graham ◽  
Carol Leonard ◽  
David F. Tang-Wai ◽  
Sandra Black ◽  
Tiffany W. Chow ◽  
...  
Keyword(s):  
Primary Progressive Aphasia ◽  
Diagnostic Feature ◽  
Apraxia Of Speech ◽  
Volumetric Mri ◽  
Motor Speech ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Grammatical Morphemes ◽  
Grammatical Errors ◽  
Diagnosis Method

Background/Aims: Frank agrammatism, defined as the omission and/or substitution of grammatical morphemes with associated grammatical errors, is variably reported in patients with nonfluent variant primary progressive aphasia (nfPPA). This study addressed whether frank agrammatism is typical in agrammatic nfPPA patients when this feature is not required for diagnosis. Method: We assessed grammatical production in 9 patients who satisfied current diagnostic criteria. Although the focus was agrammatism, motor speech skills were also evaluated to determine whether dysfluency arose primarily from apraxia of speech (AOS), instead of, or in addition to, agrammatism. Volumetric MRI analyses provided impartial imaging-supported diagnosis. Results: The majority of cases exhibited neither frank agrammatism nor AOS. Conclusion: There are nfPPA patients with imaging-supported diagnosis and preserved motor speech skills who do not exhibit frank agrammatism, and this may persist beyond the earliest stages of the illness. Because absence of frank agrammatism is a subsidiary diagnostic feature in the logopenic variant of PPA, this result has implications for differentiation of the nonfluent and logopenic variants, and indicates that PPA patients with nonfluent speech in the absence of frank agrammatism or AOS do not necessarily have the logopenic variant.

scholarly journals Western Aphasia Battery–Revised Profiles in Primary Progressive Aphasia and Primary Progressive Apraxia of Speech

2020 ◽  
Vol 29 (1S) ◽  
pp. 498-510 ◽  
Author(s):  
Heather M. Clark ◽  
Rene L. Utianski ◽  
Joseph R. Duffy ◽  
Edythe A. Strand ◽  
Hugo Botha ◽  
...  
Keyword(s):  
Primary Progressive Aphasia ◽  
Relative Performance ◽  
Apraxia Of Speech ◽  
Motor Speech ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
R Classification ◽  
The Mean ◽  
Fluent Aphasia ◽  
Speech And Language Disorders

Purpose The primary aim was to examine the utility of the Western Aphasia Battery–Revised (WAB-R; Kertesz, 2007 ) for classifying variants of primary progressive aphasia (PPA). Traditional WAB-R metrics of Aphasia Quotient (AQ), subtest scores, WAB-R classification, and several novel metrics were examined. A secondary aim was to examine these same WAB-R metrics in individuals with primary progressive apraxia of speech (PPAOS). Method A retrospective analysis of WAB-R records from 169 participants enrolled in a study of neurodegenerative speech and language disorders was conducted. PPA/PPAOS classification was determined by consensus review of speech, language, and cognitive profiles. Scores on each of the WAB-R subtests were obtained to derive AQ, WAB-R aphasia profile, and 3 ratios reflecting relative performance on subtests. Results Mean AQ was significantly higher in the PPAOS group compared to all PPA variants except primary fluent aphasia. AQ above the normal cutoff was observed for 20% of participants with PPA. Significant main effects of group were noted for each of the subtests. Follow-up comparisons most frequently discriminated PPAOS, primary agrammatic aphasia (PAA), and logopenic progressive aphasia. Primary fluent aphasia and semantic dementia (SD) subtest scores were less distinctive, with the exception of Naming for SD, which was significantly lower than for PAA and PPAOS. When the WAB-R AQ detected aphasia, a classification of anomic aphasia was most frequently observed; this pattern held true for each of the PPA variants. The mean Information Content:Naming ratio was highest for SD, and the mean Comprehension:Fluency ratio was highest for PAA. Conclusions In the current study, AQ underestimated the presence of PPA and WAB-R classification did not distinguish among PPA classification determined by consensus. Performance on individual subtests and relative performance across subtests demonstrated inconsistent alignment with PPA classification. We conclude the WAB-R in isolation is inadequate to detect or characterize PPA. We instead suggest utilizing the WAB-R as 1 component of a comprehensive language and motor speech assessment when PPA is suspected.

scholarly journals Semantic and logopenic primary progressive aphasia differ in pitch variation during a motor speech task

2020 ◽  
Vol 16 (S6) ◽  
Author(s):  
Peter S. Pressman ◽  
Eric Lemieux ◽  
Gordon Matthewson ◽  
Chris O'Neill ◽  
Elliott D. Ross
Keyword(s):  
Primary Progressive Aphasia ◽  
Motor Speech ◽  
Progressive Aphasia ◽  
Speech Task ◽  
Primary Progressive
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