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

Tetzloff, Katerina A.; Duffy, Joseph R.; Strand, Edythe A.; Machulda, Mary M.; Boland, Sarah; Utianski, Rene L.; Botha, Hugo; Senjem, Matthew L.; Schwarz, Christopher G.; Josephs, Keith A.; Whitwell, Jennifer L.

doi:10.1080/13554794.2018.1477963

Cited by 26 publications

(30 citation statements)

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“…The development of PSP symptoms is, therefore, fairly common in patients with progressive AOS and/or agrammatic aphasia. Although we and others have previously noted the development of PSP in speech/language patients, this is the largest longitudinal cohort reported to date and the first to apply the MDS‐PSP criteria. It is clear that a large proportion of patients who meet possible PSP‐SL criteria will go on to meet criteria for probable PSP‐RS or probable PSP‐P, with most patients meeting the criteria for both diagnoses.…”

Section: Discussionmentioning

confidence: 84%

“…A patient can be diagnosed as PSP with predominant speech/language disorder (PSP‐SL) if he or she has progressive AOS or nonfluent/agrammatic aphasia and display vertical supranuclear gaze palsy or slowing of vertical saccades . Of note, the diagnostic certainty associated with PSP‐SL is only “possible PSP” because other pathologies have been reported in AOS/agrammatic patients . However, a patient could meet a probable PSP diagnosis if he or she developed additional clinical features, such as postural instability/falls (probable PSP‐Richardson's syndrome [PSP‐RS]), bradykinesia and rigidity (probable PSP‐parkinsonism [PSP‐P]), or freezing of gait (probable PSP‐progressive gait freezing [PSP‐PGF]) …”

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confidence: 99%

“…the diagnostic certainty associated with PSP-SL is only "possible PSP" because other pathologies have been reported in AOS/agrammatic patients. [12][13][14][15][16] However, a patient could meet a probable PSP diagnosis if he or she developed additional clinical features, such as postural instability/falls (probable PSP-Richardson's syndrome [PSP-RS]), bradykinesia and rigidity (probable PSPparkinsonism [PSP-P]), or freezing of gait (probable PSP-progressive gait freezing [PSP-PGF]). 11 We applied the MDS-PSP criteria to a cohort of 52 speech/language patients who had been followed longitudinally to determine which PSP symptoms most commonly develop over time and when in the disease course they develop.…”

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confidence: 99%

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An Evaluation of the Progressive Supranuclear Palsy Speech/Language Variant

Whitwell

Stevens

Duffy

et al. 2019

Movement Disord Clin Pract

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Background The Movement Disorder Society clinical criteria for progressive supranuclear palsy (PSP) provide a framework for assessing the presence/severity of clinical symptoms and define a speech/language variant of PSP. Objectives To evaluate the clinical criteria in a cohort of speech/language patients with longitudinal follow‐up. Methods A total of 52 patients presenting with progressive apraxia of speech and/or agrammatic aphasia were followed longitudinally for up to 6 visits with clinical assessments and magnetic resonance imaging. We assessed oculomotor, postural instability, and akinesia diagnostic levels and determined whether patients met criteria for possible PSP‐speech/language or probable PSP at each visit. Kaplan‐Meier curves assessed time‐to‐event probabilities according to age. Statistical parametric mapping and midbrain volume were assessed according to disease progression. Results Few PSP symptoms were observed early in the disease, with oculomotor abnormalities and falls first observed 2 years after onset. Falls were more common than vertical supranuclear gaze palsy. Bradykinesia and rigidity commonly developed but axial was rarely greater than appendicular rigidity. During follow‐up, 54% met criteria for possible PSP‐speech/language, 38% for probable PSP‐Richardson's syndrome, and 38% for probable PSP‐parkinsonism, most commonly 6 to 6.9 years after onset. The probability of developing PSP was greater when onset was at an age older than 70 years. Patients who progressed to probable PSP had more parkinsonism and oculomotor impairment at baseline and greater midbrain atrophy when compared with those who did not develop probable PSP. Conclusions Symptoms typical of PSP commonly develop in patients presenting with a progressive speech/language disorder. Older age appears to be an important prognostic factor in these patients.

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Section: Discussionmentioning

confidence: 84%

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confidence: 99%

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confidence: 99%

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An Evaluation of the Progressive Supranuclear Palsy Speech/Language Variant

Whitwell

Stevens

Duffy

et al. 2019

Movement Disord Clin Pract

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“…This entity was termed primary progressive apraxia of speech (PPAOS) 8 . Patients with both agPPA and PPAOS often develop an atypical parkinsonian syndrome over time, characterized by limb apraxia, postural instability and eye movement abnormalities [9][10][11][12] . The PPA criteria also defined a semantic variant of PPA which specifically classifies patients with anomia and loss of word knowledge, and hence includes some, although not all, patients that were originally classified as SD 6 .…”

Section: Clinical Classification Of Ftdmentioning

confidence: 99%

FTD spectrum: Neuroimaging across the FTD spectrum

Whitwell

2019

Progress in Molecular Biology and Translational Science

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Frontotemporal dementia is a complex and heterogeneous neurodegenerative disease that encompasses many clinical syndromes, pathological diseases, and genetic mutations. Neuroimaging has played a critical role in our understanding of the underlying pathophysiology of frontotemporal dementia and provided biomarkers to aid diagnosis. Early studies defined patterns of neurodegeneration and hypometabolism associated with the clinical, pathological and genetic aspects of frontotemporal dementia, with more recent studies highlighting how the breakdown of structural and functional brain networks define frontotemporal dementia. Molecular positron emission tomography ligands allowing the in vivo imaging of tau proteins have also provided important insights, although more work is needed to understand the biology of the currently available ligands.

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“…The second is a prosodic subtype, when the speech pattern is dominated by slow rate and segmented speech. Use of the term mixed subtype has also been suggested when there is no clear predominance of one type of error (Josephs et al 2013), for example when AOS is either too mild or severe to permit judgment (Utianski et al 2018a).…”

Section: Introductionmentioning

confidence: 99%

Progressive apraxia of speech in Quebec French speakers: A case series

Bouvier

Monetta

Laforce

et al. 2021

Intl J Lang & Comm Disor

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Background:The term progressive apraxia of speech (PAOS) is used to describe speakers presenting with isolated or dominant apraxia of speech in the context of a neurodegenerative syndrome, including primary progressive apraxia of speech (PPAOS) and dominant progressive apraxia of speech (DAOS), respectively. Its motor speech profile has been increasingly explored in the last decade, but description remains vague and very English oriented, although the effect of speakers' language on motor speech phenotypes is increasingly recognized. Although some studies suggest that speakers presenting with isolated PAOS (PPAOS) versus dominant PAOS with concomitant aphasia (DAOS) should be differentiated, distinct characteristics of the two presentations are unclear. Furthermore, a careful description of their clinical presentation in languages other than English is required. Aims: To describe the motor speech characteristics of Quebec French-speaking participants with prominent PAOS and to explore the communication profile of those presenting more specifically with isolated PAOS (PPAOS), and with dominant PAOS and concomitant aphasia (DAOS). Methods & Procedures: A thorough effort to recruit all speakers presenting with PAOS in the larger population areas of the province of Quebec was conducted over a 3-year span. A total of nine participants with PAOS (pwPAOS; PPAOS = 5, DAOS = 4) underwent a comprehensive language and motor speech assessment, and a cognitive screening. Their performance was compared with 30 matched healthy controls.Outcomes & Results: As a group, pwPAOS differed from healthy speakers on all acoustic and perceptual measures. The PPAOS and PAOS subgroups were similar on several measures, but participants from the PPAOS subgroup tended to perform better on articulatory measures and maximum speech rate tasks. Conclusions & Implications:This study provides an in-depth analysis of motor speech characteristics of PAOS in Quebec French speakers and adds further evidence for the differentiation of PPAOS and DAOS. Combining simple perceptual and acoustic analyses represent a promising approach to distinguish the two variants and identify treatment targets.

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

Cited by 26 publications

References 51 publications

An Evaluation of the Progressive Supranuclear Palsy Speech/Language Variant

An Evaluation of the Progressive Supranuclear Palsy Speech/Language Variant

FTD spectrum: Neuroimaging across the FTD spectrum

Progressive apraxia of speech in Quebec French speakers: A case series

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