Apraxia of speech (AOS) can be caused by neurodegenerative disease and sometimes is its presenting sign (i.e., primary progressive apraxia of speech, PPAOS). During the last several decades, our ...understanding of PPAOS has evolved from clinical recognition to a fuller understanding of its core and associated clinical features, its distinction from but relationship with primary progressive aphasia, its temporal course and eventual progression to include other neurological deficits, and its neuroimaging correlates and underlying pathology.
This paper provides a comprehensive summary of the literature that has built the current knowledge base about PPAOS and progressive AOS as it co-occurs with progressive aphasia. It reviews the history of its emergence as a recognized syndrome; its relationship with the agrammatic/nonfluent variant of primary progressive aphasia; its salient perceptual features and subtypes; the acoustic and structural/physiological imaging measures that index its presence, severity, and distinction from aphasia; and principles and available data regarding its management and care.
A broad summary of what is known about AOS as a manifestation of neurodegenerative disease.
Primary progressive apraxia of speech is a recognizable syndrome that can be distinguished from other neurodegenerative conditions that affect speech and language.
Abbreviations
AAC = augmentative and alternative communication; AES = Articulatory Error Score; ALS = amyotrophic lateral sclerosis; AOS = apraxia of speech; AOS+PAA = AOS plus the agrammatic/nonfluent variant of PPA; ASRS = Apraxia of Speech Rating Scale; CBD = corticobasal degeneration; CBS = corticobasal syndrome; DAOS = dominant AOS - aphasia present but AOS more severe; MSD = motor speech disorders; nfPPA = nonfluent variant of PPA, with or without AOS; NVOA = nonverbal oral apraxia; PAOS = progressive AOS, with or without aphasia; PAA = agrammatic variant of PPA, without AOS; PPA = primary progressive aphasia, with or without AOS; PPAOS = primary progressive AOS - no aphasia; PSP = progressive supranuclear palsy; SMA = supplementary motor area.
•Phonetic PPAOS is characterized predominantly by distorted sound substitutions.•Prosodic PPAOS is characterized predominantly by slow, segmented speech.•The predominant clinical characteristics of ...PPAOS can be quantified reliably.•PPAOS subtypes are associated with distinct imaging patterns on MRI, DTI, and FDG-PET.
Primary progressive apraxia of speech (PPAOS) is a clinical syndrome in which apraxia of speech is the initial indication of neurodegenerative disease. Prior studies of PPAOS have identified hypometabolism, grey matter atrophy, and white matter tract degeneration in the frontal gyri, precentral cortex, and supplementary motor area (SMA). Recent clinical observations suggest two distinct subtypes of PPAOS may exist. Phonetic PPAOS is characterized predominantly by distorted sound substitutions. Prosodic PPAOS is characterized predominantly by slow, segmented speech. Demographic, clinical, and neuroimaging data (MRI, DTI, and FDG-PET) were analyzed to validate these subtypes and explore anatomic correlates. The Phonetic subtype demonstrated bilateral involvement of the SMA, precentral gyrus, and cerebellar crus. The Prosodic subtype demonstrated more focal involvement in the SMA and right superior cerebellar peduncle. The findings provide converging evidence that differences in the reliably determined predominant clinical characteristics of AOS are associated with distinct imaging patterns, independent of severity.
An Update on Apraxia of Speech Utianski, Rene L.; Josephs, Keith A.
Current neurology and neuroscience reports,
07/2023, Letnik:
23, Številka:
7
Journal Article
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Purpose of Review
Apraxia of speech (AOS) is a motor speech disorder that has long been recognized to occur secondary to acute neurologic insults and, more recently, to neurodegenerative diseases as ...a harbinger for progressive supranuclear palsy and corticobasal syndrome. This article reviews recent findings regarding the clinic phenotypes of AOS, neuroimaging correlates, and the underlying disease processes.
Recent Findings
Two clinical subtypes of AOS map onto two underlying 4-repeat tauopathies. New imaging techniques have recently been applied to the study of progressive AOS. There is no data on the impact of behavioral intervention, although studies of nonfluent/agrammatic primary progressive aphasia that include patients with AOS suggest some benefit in speech intelligibility and maintenance.
Summary
While recent findings suggest subtypes of AOS exist that are linked to molecular pathology and have important implications for disease progression, further research is needed to assess outcome of behavioral and other types of intervention.
Objective
Primary progressive apraxia of speech (PPAOS) is associated with imaging abnormalities in the lateral premotor cortex (LPC) and supplementary motor area (SMA). It is not known whether ...greater involvement of these regions in either hemisphere is associated with demographics, presenting, and/or longitudinal features.
Methods
In 51 prospectively recruited PPAOS patients who completed 18F‐fluorodeoxyglucose (FDG) positron emission tomography (PET), we classified patients as left‐dominant, right‐dominant, or symmetric, based on visual assessment of the LPC and SMA on FDG‐PET. SPM and statistical analyses of regional metabolic values were performed. Diagnosis of PPAOS was made if apraxia of speech was present and aphasia absent. Thirteen patients completed ioflupane‐123I (dopamine transporter DAT) scans. We compared cross‐sectional and longitudinal clinicopathological, genetic, and neuroimaging characteristics across the three groups, with area under the receiver‐operating curve (AUROC) determined as a measure of effect size.
Results
In all, 49% of the PPAOS patients were classified as left‐dominant, 31% as right‐dominant, and 20% as symmetric, which was supported by results from the SPM and regional analyses. There were no differences in baseline characteristics. Longitudinally, right‐dominant PPAOS showed faster rates of progression of ideomotor apraxia (AUROC 0.79), behavioral disturbances (AUROC 0.84), including disinhibition symptoms (AUROC 0.82) and negative behaviors (AUROC 0.82), and parkinsonism (AUROC 0.75) compared to left‐dominant PPAOS. Symmetric PPAOS showed faster rates of dysarthria progression compared to left‐dominant (AUROC 0.89) and right‐dominant PPAOS (AUROC 0.79). Five patients showed abnormal DAT uptake. Braak neurofibrillary tangle stage differed across groups (p = 0.01).
Conclusions
Patients with PPAOS and a right‐dominant pattern of hypometabolism on FDG‐PET have the fastest rates of decline of behavioral and motor features.
Automatic assessment of vowel space area Sandoval, Steven; Berisha, Visar; Utianski, Rene L ...
The Journal of the Acoustical Society of America,
11/2013, Letnik:
134, Številka:
5
Journal Article
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Vowel space area (VSA) is an attractive metric for the study of speech production deficits and reductions in intelligibility, in addition to the traditional study of vowel distinctiveness. ...Traditional VSA estimates are not currently sufficiently sensitive to map to production deficits. The present report describes an automated algorithm using healthy, connected speech rather than single syllables and estimates the entire vowel working space rather than corner vowels. Analyses reveal a strong correlation between the traditional VSA and automated estimates. When the two methods diverge, the automated method seems to provide a more accurate area since it accounts for all vowels.
Functional speech disorders (FSDs), a subtype of functional neurological disorders, are distinguishable from neurogenic motor speech disorders based on their clinical features, clinical course, and ...response to treatment. However, their differential diagnosis and management can be challenging. FSDs are not well understood, but growing evidence suggests a biopsychosocial basis distinct from structural lesions that cause neurogenic motor speech disorders.
Following an overview of FSDs, four patients are described to illustrate the range of clinical manifestations, biopsychosocial contexts, and responses to treatment of FSDs. The path to differential diagnosis is discussed, with particular attention to positive features that led to the FSD diagnosis. Approaches to education, counseling, and management are discussed.
This case series demonstrates that FSDs can present with a variety of manifestations including dysfluencies, articulation errors, dysphonia, rate and prosodic abnormalities, and combinations of disruptions in speech subsystems. FSDs may present in the context of known recent or remote physical or psychosocial trauma or, as in many cases, in the absence of an identifiable triggering event. FSDs are recognizable by positive clinical features and should not be considered a diagnosis of exclusion. With appropriate identification, counseling, and treatment, FSDs may resolve, sometimes rapidly; in some cases, treatment may be prolonged or ineffective.
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DOBA, IZUM, KILJ, NUK, OILJ, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK, VSZLJ
Agrammatic aphasia affects grammatical language production and can result from a neurodegenerative disease. Although it typically presents with concomitant apraxia of speech, this is not always the ...case. Little is known about the clinical course and imaging features of patients that present with agrammatism in the absence of apraxia of speech, which we will refer to as progressive agrammatic aphasia. We aimed to make a detailed description of the longitudinal clinical, linguistic, and neuroimaging features of a cohort of 11 patients with progressive agrammatic aphasia to provide a complete picture of this syndrome. All patients underwent detailed speech and language, neurological and neuropsychological assessments, 3 T structural and diffusion tensor imaging MRI, 18F-fluorodeoxyglucose and Pittsburgh compound B PET. The 11 patients were matched by age and gender to 22 patients who had mixed apraxia of speech and agrammatism. The progressive agrammatic aphasia patients performed abnormally on tests of language, general cognition, executive function, and functional ability at baseline and declined in these measures over time. Only two patients eventually developed apraxia of speech, while parkinsonism was absent-to-mild throughout all visits for all patients. When compared to the patients with mixed apraxia of speech and agrammatism, the patients with progressive agrammatic aphasia performed better on tests of motor speech and parkinsonism but more poorly, and declined faster over time, on tests of general aphasia severity, agrammatism, and naming. The patients with progressive agrammatic aphasia also showed different neuroimaging abnormalities, with greater atrophy, hypometabolism and white matter tract degeneration in the prefrontal and anterior temporal lobes compared to patients with mixed apraxia of speech and agrammatism. These differences were more pronounced as the disease progressed. These results demonstrate that progressive agrammatic aphasia has a different clinical disease course and different underlying neuroanatomical abnormalities than patients with the more common syndrome of mixed agrammatism and apraxia of speech. This supports the distinction of progressive agrammatic aphasia and has implications for the classification of patients with agrammatic aphasia.
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.
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DOBA, IZUM, KILJ, NUK, OILJ, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK, VSZLJ
•All PSP variants showed atrophy or flortaucipir uptake in subcortical structures.•Speech/language, frontal and corticobasal variants showed cortical involvement.•Dentatorubrothalamic tract ...involvement was only seen in some variants.•PSP variants show different patterns of damage to subcortical-cortical circuitry.
Progressive supranuclear palsy (PSP) is a neurodegenerative tauopathy that is associated with different clinical variants, including PSP-Richardson's syndrome (PSP-RS), PSP-parkinsonism (PSP-P), PSP-corticobasal syndrome (PSP-CBS), PSP-frontal (PSP-F), PSP-progressive gait freezing (PSP-PGF) and PSP-speech/language (PSP-SL). While PSP-RS has been well-characterized on neuroimaging, the characteristics of the other atypical variants are less well defined and it is unknown how they compare to each other or relate to neuropathology. We aimed to assess and compare regional atrophy on MRI and 18Fflortaucipir uptake on PET across PSP variants.
105 PSP patients (53 PSP-RS, 23 PSP-SL, 12 PSP-P, 8 PSP-CBS, 5 PSP-F and 4 PSP-PGF) underwent volumetric MRI, with 59 of these also undergoing flortaucipir PET. Voxel-level and region-level analyses were performed comparing PSP variants to 30 controls and to each other. Semi-quantitative tau burden measurements were also performed in 21 patients with autopsy-confirmed PSP.
All variants showed evidence for atrophy or increased flortaucipir uptake in striatum, globus pallidus and thalamus. Superior cerebellar peduncle volume loss was only observed in PSP-RS, PSP-CBS and PSP-F. Volume loss in the frontal lobes was observed in PSP-SL, PSP-CBS and PSP-F, with these variants also showing highest cortical tau burden at autopsy. The PSP-P and PSP-PGF variants showed more restricted patterns of neurodegeneration predominantly involving striatum, globus pallidus, subthalamic nucleus and thalamus. The PSP-SL variant showed greater volume loss and flortaucipir uptake in supplementary motor area and motor cortex compared to all other variants, but showed less involvement of subthalamic nucleus and midbrain. Compared to PSP-RS, PSP-P had larger midbrain volume and greater flortaucipir uptake in putamen.
The PSP variants have different patterns of involvement of subcortical circuitry, perhaps suggesting different patterns of disease spread through the brain. These findings will be important in the development of appropriate neuroimaging biomarkers for the different PSP variants.
Apraxia of speech (AOS) is a motor speech disorder affecting articulatory planning and speech programming. When AOS is the sole manifestation of neurodegeneration, it is termed primary progressive ...apraxia of speech (PPAOS). Recent work has shown that there are distinct PPAOS subtypes: phonetic, prosodic, and those that do not clearly align with either (mixed). PPAOS subtypes differ with respect to the predominating motor speech difficulties, as well as disease progression and underlying pathology. Because past studies have determined PPAOS subtype based on clinical impression, the goal of the present study was to quantitatively determine the distribution of speech error types across PPAOS subtypes in a word repetition task and to investigate how word complexity affects the type and number of speech errors across PPAOS subtypes.
Forty-five patients with PPAOS (13 phonetic, 23 prosodic, and nine mixed) and 45 healthy controls produced multiple repetitions of words that varied in phonetic complexity. Sound additions, deletions, and substitutions/distortions (phonetic errors) and within-word segmentations (prosodic errors) were calculated.
All three PPAOS groups produced significantly more errors than controls, but the total number of errors was comparable among subtypes. The phonetic group produced more phonetic-type errors compared to the prosodic group but comparable to the mixed group. The prosodic group produced more segmentations compared to the phonetic and mixed PPAOS groups. As word complexity increased, the total number of errors increased for PPAOS patients. The phonetic and prosodic groups were more likely to produce phonetic- and prosodic-type errors, respectively, as word complexity increased.
This study provides novel quantitative data showing that PPAOS subtype can be supported by the type and distribution of speech errors in a word repetition task. This may facilitate earlier, more reliable differential diagnosis and aid in disease prognosis, as PPAOS subtypes have distinct disease trajectories.
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DOBA, IZUM, KILJ, NUK, OILJ, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK, VSZLJ
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