scholarly journals Coming to Terms with a Conundrum: A Case of Primary Progressive Apraxia of Speech due to Corticobasal Degeneration?

2021 ◽  
pp. 483-489
Author(s):  
Aristotelis Karantzoulis ◽  
Emanuela Susani ◽  
Carlo Ferrarese ◽  
Ildebrando Appollonio ◽  
Lucio Tremolizzo
Keyword(s):  
Natural History ◽  
Single Case ◽  
Corticobasal Degeneration ◽  
Clinical Syndrome ◽  
Apraxia Of Speech ◽  
Motor Speech ◽  
Primary Progressive ◽  
Prominent Symptom ◽  
Cerebrospinal Fluid Biomarkers ◽  
Extrapyramidal Signs

Primary progressive apraxia of speech (PPAOS) is a progressive disorder impairing the motor speech act leaving linguistic function unattained. Although apraxia of speech frequently co-occurs with other neurodegenerative conditions, PPAOS defines a clinical syndrome where apraxia of speech is the sole or prominent symptom for much of the disease’s natural history. Mounting evidence is beginning to fully define this disease as the epiphenomenon of 4-repeat (4R) tau pathology although other pathologic signatures have been reported. Indeed, PPAOS patients generally present a parkinsonian syndrome late into their natural history mostly qualifying for either corticobasal syndrome (CBS) or progressive supranuclear palsy (PSP). This is starting to be reflected in diagnostic criteria for PSP, namely, in the PSP speech and language (SL) subcategory; however, this inclusion is not reflected for CBS. Here, we present a single case of a patient with PPAOS and her clinical follow-up lasting 6 years, from the time she sought our attention to her death which occurred 8 years into the disease. PPAOS was the only and prominent symptom for most of the illness with extrapyramidal signs overtly presenting in the last months of its course. Clinical evaluation, imaging, genetic, and cerebrospinal fluid biomarkers all pointed toward an underlying CBD pathology, albeit the eventual anatomopathological confirmation was not performed. Had her clinical course been more suggestive of PSP, she would have qualified for criteria as PSP-SL. Our case therefore suggests the hypothetic need to discuss the broadening of the existing CBS criteria to encompass isolated PPAOS.

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.

A Preliminary Look Into the Clinical Evolution of Motor Speech Characteristics in Primary Progressive Apraxia of Speech in Québec French

2021 ◽  
pp. 1-18
Author(s):  
Liziane Bouvier ◽  
Laura Monetta ◽  
Paolo Vitali ◽  
Robert Laforce ◽  
Vincent Martel-Sauvageau
Keyword(s):  
Multiple Testing ◽  
Case Series ◽  
Cognitive Screening ◽  
Apraxia Of Speech ◽  
Motor Speech ◽  
Primary Progressive ◽  
Speech Characteristics ◽  
Quebec French ◽  
Language Assessments ◽  
Almost All

Purpose This study aimed to track changes in acoustical and perceptual features of motor speech in patients with phonetic and prosodic primary progressive apraxia of speech (PPAOS) in Québec French over an 18-month period. Method A prospective multiple-case series with multiple testing periods, including four participants with a diagnosis of PPAOS, was conducted. Participants were 0.5–4 years postonset of disease at baseline. They underwent comprehensive motor speech and language assessments and cognitive screening every 6 months for up to 18 months. Acoustical and perceptual analyses of motor speech were conducted. Results Results showed a considerable impairment in motor speech abilities for patients with PPAOS at all time points and a significant decrease in performance for almost all articulatory and prosodic measures over time. Passage reading and diadochokinesis seemed particularly promising for the tracking of changes in PPAOS motor speech characteristics and PPAOS classification. Quantifying length of speech runs made it possible to distinguish phonetic from prosodic PPAOS. Finally, the patients who evolved to phonetic PPAOS developed aphasia, and the two with prosodic PPAOS showed greater motor symptoms such as unequivocal dysarthria. Conclusion This study extends the growing literature on PPAOS and its subtypes by describing specific changes in articulatory and prosodic abilities over a period of at least 6 months, which are important for the diagnosis and management of PPAOS.

scholarly journals Clinical and imaging progression over 10 years in a patient with primary progressive apraxia of speech and autopsy-confirmed corticobasal degeneration

Neurocase ◽  
2018 ◽  
Vol 24 (2) ◽  
pp. 111-120 ◽  
Author(s):  
Katerina A. Tetzloff ◽  
Joseph R. Duffy ◽  
Edythe A. Strand ◽  
Mary M. Machulda ◽  
Sarah M. Boland ◽  
...  
Keyword(s):  
Corticobasal Degeneration ◽  
Apraxia Of Speech ◽  
Primary Progressive

Rapid Syllable Transition Treatment—A Treatment for Childhood Apraxia of Speech and Other Pediatric Motor Speech Disorders

2020 ◽  
Vol 5 (4) ◽  
pp. 821-830 ◽  
Author(s):  
Patricia McCabe ◽  
Donna Claire Thomas ◽  
Elizabeth Murray
Keyword(s):  
Ad Hoc ◽  
Single Case ◽  
Controlled Trial ◽  
Speech Disorders ◽  
Apraxia Of Speech ◽  
Childhood Apraxia Of Speech ◽  
Motor Speech ◽  
Motor Speech Disorders ◽  
Treatment Data ◽  
Core Features

Purpose Childhood apraxia of speech (CAS) is a speech disorder that many generalist speech-language pathologists feel underqualified to treat. For children with CAS, this may result in ad hoc interventions resulting in slower progress. Research evidence for various CAS treatments is primarily limited to single-case experimental design studies; however, two treatments (Rapid Syllable Transition Treatment [ReST] and the Nuffield Dyspraxia Programme, Third Edition) have been compared in a single randomized controlled trial. Conclusion This tutorial describes one of those treatments: ReST which was designed to address the three consensus core features of CAS simultaneously: consistency and accuracy of sounds, sequencing, and prosody. The treatment uses nonwords to help children build and store accurate motor plans and programs using principles of motor learning. Treatment data are described, and commonly reported clinical issues are discussed. Recommendations for which children may be suitable for ReST and for evidence-based practice are described.

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.

Bang for Your Buck: A Single-Case Experimental Design Study of Practice Amount and Distribution in Treatment for Childhood Apraxia of Speech

2019 ◽  
Vol 62 (9) ◽  
pp. 3160-3182 ◽  
Author(s):  
Edwin Maas ◽  
Christina Gildersleeve-Neumann ◽  
Kathy Jakielski ◽  
Nicolette Kovacs ◽  
Ruth Stoeckel ◽  
...  
Keyword(s):  
Single Case ◽  
Evidence Base ◽  
Distributed Practice ◽  
Single Subject ◽  
Single Subject Design ◽  
Apraxia Of Speech ◽  
Childhood Apraxia Of Speech ◽  
Opposite Pattern ◽  
Massed Practice ◽  
Treatment Conditions

Purpose The aim of this study was to examine 2 aspects of treatment intensity in treatment for childhood apraxia of speech (CAS): practice amount and practice distribution. Method Using an alternating-treatments single-subject design with multiple baselines, we compared high versus low amount of practice, and massed versus distributed practice, in 6 children with CAS. Conditions were manipulated in the context of integral stimulation treatment. Changes in perceptual accuracy, scored by blinded analysts, were quantified with effect sizes. Results Four children showed an advantage for high amount of practice, 1 showed an opposite effect, and 1 showed no condition difference. For distribution, 4 children showed a clear advantage for massed over distributed practice post treatment; 1 showed an opposite pattern, and 1 showed no clear difference. Follow-up revealed a similar pattern. All children demonstrated treatment effects (larger gains for treated than untreated items). Conclusions High practice amount and massed practice were associated with more robust speech motor learning in most children with CAS, compared to low amount and distributed practice, respectively. Variation in effects across children warrants further research to determine factors that predict optimal treatment conditions. Finally, this study adds to the evidence base supporting the efficacy of integral stimulation treatment for CAS. Supplemental Material https://doi.org/10.23641/asha.9630599

A Phonomotor Approach to Apraxia of Speech Treatment

2020 ◽  
Vol 29 (4) ◽  
pp. 2109-2130
Author(s):  
Lauren Bislick
Keyword(s):  
Phase I ◽  
Treatment Effects ◽  
Single Case ◽  
Motor Planning ◽  
The Other ◽  
Multimodal Approach ◽  
Apraxia Of Speech ◽  
Duration Of Treatment ◽  
Treatment Gains ◽  
Future Work

Purpose This study continued Phase I investigation of a modified Phonomotor Treatment (PMT) Program on motor planning in two individuals with apraxia of speech (AOS) and aphasia and, with support from prior work, refined Phase I methodology for treatment intensity and duration, a measure of communicative participation, and the use of effect size benchmarks specific to AOS. Method A single-case experimental design with multiple baselines across behaviors and participants was used to examine acquisition, generalization, and maintenance of treatment effects 8–10 weeks posttreatment. Treatment was distributed 3 days a week, and duration of treatment was specific to each participant (criterion based). Experimental stimuli consisted of target sounds or clusters embedded nonwords and real words, specific to each participants' deficit. Results Findings show improved repetition accuracy for targets in trained nonwords, generalization to targets in untrained nonwords and real words, and maintenance of treatment effects at 10 weeks posttreatment for one participant and more variable outcomes for the other participant. Conclusions Results indicate that a modified version of PMT can promote generalization and maintenance of treatment gains for trained speech targets via a multimodal approach emphasizing repeated exposure and practice. While these results are promising, the frequent co-occurrence of AOS and aphasia warrants a treatment that addresses both motor planning and linguistic deficits. Thus, the application of traditional PMT with participant-specific modifications for AOS embedded into the treatment program may be a more effective approach. Future work will continue to examine and maximize improvements in motor planning, while also treating anomia in aphasia.

The Labeling Problem in Aphasia

1986 ◽  
Vol 51 (2) ◽  
pp. 176-180 ◽  
Author(s):  
Audrey L. Holland ◽  
Davida Fromm ◽  
Carol S. Swindell
Keyword(s):  
Speech Disorders ◽  
Case Description ◽  
Test Results ◽  
Apraxia Of Speech ◽  
Poor Agreement ◽  
Motor Speech ◽  
Motor Speech Disorders ◽  
Communication Difficulties ◽  
Token Test ◽  
Primary Problem

Twenty-five "experts" on neurogenic motor speech disorders participated in a tutorial exercise. Each was given information on M, a patient who had communication difficulties as the result of stroke, and asked to complete a questionnaire about his problem. The information included a detailed case description, an audiotape of M's speech obtained at 4, 9, 13, and 17 days post-stroke, and test results from the Western Aphasia Battery, the Token Test, and a battery for apraxia of speech. The experts were in excellent agreement on M's primary problem, although it was called by seven different names. The experts were in poor agreement on his secondary problem(s), e.g., the presence and type of aphasia and dysarthria. The results suggest that labeling is difficult, even for "experts." Furthermore, the practicing clinician needs to be sensitive to the likelihood of more than one coexisting problem.

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