Syndromes of nonfluent primary progressive aphasia

Rohrer, Jonathan D.; Rossor, Martin N.; Warren, Jason D.

doi:10.1212/wnl.0b013e3181ed9c6b

Cited by 113 publications

(114 citation statements)

References 40 publications

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“…Several reports describe an increased frequency of AoS in patients with naPPA [40][41][42] . AoS is most prominent in conditions with co-occurring involuntary limb movements and poor limb motor control such as progressive supranuclear palsy syndrome (PSPS) and corticobasal syndrome (CBS), although AoS can occur without an accompanying extrapyramidal disorder, and AoS can occur as an isolated entity without other evidence of the language impairments found in naPPA 43,44 . An important problem has been difficulty quantifying AoS.…”

Section: Speech and Language Deficits In Nappamentioning

confidence: 99%

The non-fluent/agrammatic variant of primary progressive aphasia

Grossman¹

2016

The Behavioral Neurology of Dementia

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In an era of disease-modifying treatments, the non-fluent/agrammatic variant of primary progressive aphasia (naPPA) may help screen for a specific cause of neurodegenerative disease. However, there are controversies surrounding the identification of naPPA. This review describes the characteristic features associated with this discrete, young-onset neurodegenerative condition. Patients with naPPA have a distinct limitation in language emphasizingtheir poor grammatical comprehension and expression, as well as a disorder of speech sound production. Imaging studies associate an impairment of this uniquely human language capacity with disruption of a large-scale neural network centered in left inferior frontal and anterior-superior temporal regions. This corresponds to thepathologic burden of disease anatomically focused in left inferior frontal and anterior-superior temporal regions. A review of the histopathology underlying naPPA relates this condition to frontotemporal lobar degeneration spectrum pathology involving the microtubuleassociated protein tau in a majority of cases. While much work remains to be done, these observations point to unique clinical-pathological correlations that can advance care for an important class of diseases while supplementing our knowledge of human cognitive neuroscience.

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Section: Speech and Language Deficits In Nappamentioning

confidence: 99%

The non-fluent/agrammatic variant of primary progressive aphasia

Grossman¹

2016

The Behavioral Neurology of Dementia

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“…Many clinicians and researchers would still categorize AOS as a form of PNFA or as logopenic/phonologic aphasia [7]. Indeed, the features currently required for this diagnostic category appear on first glance very similar to AOS: Slowed, effortful, labored speech, sometimes with sound distortions [8].…”

Section: Discussionmentioning

confidence: 99%

“…In a voxel-based morphometric study correlating brain volume measures and disease severity scales, Rohrer et al found that the severity of AOS correlated with left posterior inferior frontal lobe atrophy, and that orofacial apraxia severity was more associated with left middle frontal, premotor and supplementary motor cortical atrophy [7]. AOS is associated with damage to the left postcentral gyrus in stroke patients.…”

Section: Brain Imaging and Apraxia Of Speechmentioning

confidence: 99%

Diagnostic Distortions: A Case Report of Progressive Apraxia of Speech

Brodtmann

Pemberton

Darby

et al. 2016

JAD

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Abstract. Apraxia of speech (AOS) can be the presenting symptom of neurodegenerative disease. The position of primary progressive AOS in the nosology of the dementias is still controversial. Despite seeing many specialists, patients are often misdiagnosed, in part due to a lack of quantitative measures of speech dysfunction. We present a single case report of a patient presenting with AOS, including acoustic analysis, language assessment, and brain imaging. A 52-year-old woman presenting with AOS had remained undiagnosed for 6 years despite seeing 8 specialists. Results of her MRI scans, genetic testing, and computerized speech analysis are provided. AOS is an underdiagnosed clinical syndrome causing great distress to patients and families. Using acoustic analysis of speech may lead to improved diagnostic accuracy. AOS is a complex entity with an expanding phenotype, and quantitative clinical measures will be critical for detection and to assess progression.

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“…In logopenic PPA cell loss is initially apparent in posterior-superior temporal lobe structures (Wilson et al, 2010). The behavioral profile is considered similar to that of vascular conduction aphasia (Gorno-Tempini, Dronkers, Rankin, Ogar, La Phengrasamy, Rosen, … Miller, 2004), although Rohrer, Rossor and Warren (2010) (Howard, Swinburn, & Porter, 2004)). With regard to sentence comprehension tested by sentence-picture matching tasks, understanding of written sentences was minimally impaired, but there was a marked impairment of spoken sentence comprehension (CAT Comprehension of spoken sentences 22/32; Comprehension of written sentences 30/32).…”

Section: Introductionmentioning

confidence: 99%

Preservation of passive constructions in a patient with primary progressive aphasia

Zimmerer¹,

Dąbrowska²,

Romanowski³

et al. 2014

Cortex

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Research into agrammatic comprehension in English has described a pattern of impaired understanding of passives and retained ability on active constructions. Some accounts of this dissociation predict that patients who are unable to comprehend actives will also be impaired in the comprehension of passives. We report the case of a man with primary progressive aphasia (WR), whose comprehension was at chance on active sentences, but at ceiling on passives. In a series of reversible sentence comprehension tests WR displayed difficulties with active transitives and truncated actives with an auxilliary. In passive sentences, he displayed sensitivity to the agent marker by, as well as the passive morphology of the verb. This pattern of dissociation challenges current theories of agrammatic comprehension. We explore 3 explanations based on the distinction between morphological and configurational cues, as well as on the semantic and discourse related differences between active and passive constructions.

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Syndromes of nonfluent primary progressive aphasia

Abstract: Background: Despite recent work, the nosology of nonfluent primary progressive aphasia (PPA)

Cited by 113 publications

References 40 publications

The non-fluent/agrammatic variant of primary progressive aphasia

The non-fluent/agrammatic variant of primary progressive aphasia

Diagnostic Distortions: A Case Report of Progressive Apraxia of Speech

Preservation of passive constructions in a patient with primary progressive aphasia

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