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.

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 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.

scholarly journals Dominant Frontotemporal Dementia Mutations in 140 Cases of Primary Progressive Aphasia and Speech Apraxia

2015 ◽  
Vol 39 (5-6) ◽  
pp. 281-286 ◽  
Author(s):  
Eoin P. Flanagan ◽  
Matthew C. Baker ◽  
Ralph B. Perkerson ◽  
Joseph R. Duffy ◽  
Edythe A. Strand ◽  
...  
Keyword(s):  
Frontotemporal Dementia ◽  
Age At Onset ◽  
Primary Progressive Aphasia ◽  
Genetic Mutation ◽  
Chromosome 9 ◽  
Apraxia Of Speech ◽  
Degree Relative ◽  
Reading Frame ◽  
Progressive Aphasia ◽  
Primary Progressive

Background: Mutations in three genes [chromosome 9 open-reading-frame 72 (C9ORF72); microtubule-associated protein tau (MAPT) and progranulin (GRN)] account for the vast majority of familial, and a proportion of sporadic, frontotemporal dementia (FTD) cases. Progressive apraxia of speech (PAOS) is a type of FTD characterized by speech production deficits without a known cause. Methods: We therefore assessed for genetic mutations in C9ORF72, MAPT and GRN in 40 prospectively recruited PAOS patients. For comparison, we also assessed these mutations in 100 patients with primary progressive aphasia (PPA), including logopenic PPA (n = 54), nonfluent/agrammatic PPA (n = 17), semantic PPA (n = 16), and unclassifiable PPA (n = 13). Results: The mean age at onset of PAOS patients was 66.7 years (± 9.3); 50% were women. Ten patients (25%) had ≥1 first-degree relative with a neurodegenerative disease. No mutations were found in any PAOS patient. In comparison, 36% of the PPA patients had a family history and 5 (5%) had a genetic mutation detected: MAPT (n = 0), GRN (n = 3) and C9ORF72 (n = 2). Conclusions: Although limited by an overrepresentation of logopenic PPA, which frequently predicts Alzheimer's disease pathology, this study suggests that mutations in the three genes most commonly associated with FTD are not associated with PAOS and are not commonly associated with PPA.

Word Fluency Test Performance in Primary Progressive Aphasia and Primary Progressive Apraxia of Speech

2021 ◽  
pp. 1-8
Author(s):  
Lucia Scheffel ◽  
Joseph R. Duffy ◽  
Edythe A. Strand ◽  
Keith A. Josephs
Keyword(s):  
Test Performance ◽  
Primary Progressive Aphasia ◽  
High Sensitivity ◽  
Clinical Group ◽  
Apraxia Of Speech ◽  
Word Fluency ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
The Relationship ◽  
Fluency Task

Purpose This study compared performance on three-word fluency measures among individuals with primary progressive aphasia (PPA) and primary progressive apraxia of speech (PPAOS), and examined the relationship between word fluency and other measures of language and speech. Method This study included 106 adults with PPA and 30 adults with PPAOS. PPA participants were divided into three clinical subgroups: semantic (svPPA), logopenic (lvPPA), and nonfluent/agrammatic with or without apraxia of speech (nfPPA). Category fluency, letter fluency, and action/verb fluency tasks were administered to all participants. Results The four clinical groups performed abnormally on the word fluency measures, although not to a degree that represented high sensitivity to their PPA or PPAOS diagnosis. All PPA subgroups produced fewer words compared to individuals with PPAOS on all word fluency measures. Moderate correlations were found between word fluency and aphasia severity and naming performance in some of the clinical groups. Conclusions Word fluency measures are often challenging for individuals with PPA and PPAOS, but they are not of equal difficulty, with letter fluency being the most difficult. Differences among word fluency tests also vary to some degree as a function of the clinical group in question, with least impairment in PPAOS. However, the findings of this study do not support statistically significant differences in word fluency task performance among the PPA subgroups. Correlations suggest that word fluency performance in PPA is at least partly related to aphasia severity.

scholarly journals Electroencephalography in primary progressive aphasia and apraxia of speech

Aphasiology ◽  
2018 ◽  
Vol 33 (11) ◽  
pp. 1410-1417 ◽  
Author(s):  
Rene L. Utianski ◽  
John N. Caviness ◽  
Gregory A. Worrell ◽  
Joseph R. Duffy ◽  
Heather M. Clark ◽  
...  
Keyword(s):  
Primary Progressive Aphasia ◽  
Apraxia Of Speech ◽  
Progressive Aphasia ◽  
Primary Progressive

scholarly journals Primary progressive aphasia patients evaluated using diffusion tensor imaging and voxel based volumetry-preliminary results

2011 ◽  
Vol 69 (3) ◽  
pp. 446-451 ◽  
Author(s):  
Fábio Pascotto de Oliveira ◽  
Jaderson Costa da Costa ◽  
Sabine Possa Marroni ◽  
Ana Maria Marques da Silva ◽  
Sthefanie Hansen Barreiro ◽  
...  
Keyword(s):  
Diffusion Tensor Imaging ◽  
Cognitive Deficits ◽  
Diffusion Tensor ◽  
Primary Progressive Aphasia ◽  
Control Group ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Language Deficit ◽  
Volume Method ◽  
Diffusion Tensor Imaging Dti

There are individuals who have a progressive language deficit without presenting cognitive deficits in other areas. One of the diseases related to this presentation is primary progressive aphasia (PPA). OBJECTIVE: Identify by means of diffusion tensor imaging (DTI) and measurements of cortical volume, brain areas that lead to dysphasia when presenting signs of impaired connectivity or reduced volume. METHOD: Four patients with PPA were evaluated using DTI, and measurements of cortical volumes in temporal areas. These patients were compared with two normal volunteers. RESULTS: There is a trend to a difference in the number and volume of related fibers between control group and patients with PPA. Comparing cortical volumes in temporal areas between groups yielded a trend to a smaller volume in PPA patients. CONCLUSION: Patients with PPA have a trend to impairment in cortical and subcortical levels regarding relevant areas.

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.

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