Clinical and pathological characterization of progressive aphasia

Knibb, Jonathan A.; Xuereb, John H.; Patterson, Karalyn; Hodges, John R.

doi:10.1002/ana.20700

Cited by 321 publications

(290 citation statements)

References 76 publications

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“…However, recent articles have demonstrated that focal, atypical distribution of Alzheimer disease (AD) pathology is responsible for 20% to 30% of cases with various forms of PPA. 2,[33][34][35] Retrospective PET and MRI studies 36,37 demonstrated in PPA patients with AD pathology a pattern of temporoparietal involvement similar to LPA. Furthermore, all US patients described in this article had cortical amyloid binding on PET scans using the Pittsburgh compound B tracer.…”

Section: Verbal Response Pointingmentioning

confidence: 99%

The logopenic/phonological variant of primary progressive aphasia

et al. 2008

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Objective: Primary progressive aphasia (PPA) is characterized by isolated decline in language functions. Semantic dementia and progressive nonfluent aphasia are accepted PPA variants. A "logopenic" variant (LPA) has also been proposed, but its cognitive and anatomic profile is less defined. The aim of this study was to establish the cognitive and anatomic features of LPA.Methods: Six previously unreported LPA cases underwent extensive neuropsychological evaluation and an experimental study of phonological loop functions, including auditory and visual span tasks with digits, letters, and words. For each patient, a voxel-wise, automated analysis of MRI or SPECT data were conducted using SPM2. Results:In LPA, speech rate was slow, with long word-finding pauses. Grammar and articulation were preserved, although phonological paraphasias could be present. Repetition and comprehension were impaired for sentences but preserved for single words, and naming was moderately affected. Investigation of phonological loop functions showed that patients were severely impaired in digit, letter, and word span tasks. Performance did not improve with pointing, was influenced by word length, and did not show the normal phonological similarity effect. Atrophy or decreased blood flow was consistently found in the posterior portion of the left superior and middle temporal gyri and inferior parietal lobule.

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Section: Verbal Response Pointingmentioning

confidence: 99%

The logopenic/phonological variant of primary progressive aphasia

et al. 2008

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“…As many as one third of patients diagnosed clinically as either progressive non-Xuent aphasia (PNFA) or semantic dementia showed this pattern [36]. Some reports have remarked the relative sparing of medial temporal lobe structures (hippocampus and entorhinal cortex) [16,17,56].…”

Section: Introductionmentioning

confidence: 99%

Argyrophilic thorny astrocyte clusters in association with Alzheimer’s disease pathology in possible primary progressive aphasia

2007

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Although most cases of primary progressive aphasia (PPA) have one of the varieties of frontotemporal lobar degeneration (FTLD) as their pathological substrate, a subset shows Alzheimer's disease (AD) pathology. We report that all eight cases in our clinic diagnosed as possible PPA, on account of the presence of episodic memory diYculties in addition to severe language impairment at the onset of disease, showed AD pathology. Neither focal accentuation of AD pathology nor vascular lesions in language-related areas was observed. Seven of these eight patients showed large argyrophilic thorny astrocyte clusters (ATAC) in the fronto-temporo-parietal cortex and subcortical white matter. The intensely tau immunoreactive astrocytes in ATAC were morphologically similar to the perivascular, subpial, and subependymal astrocytes in elderly brains, but ATAC diVer from the latter by the cortical and subcortical location, widespread distribution outside the medial temporal lobe, and intense argyrophilia. The location of ATAC was related to neither local variations in the load of AD pathology, nor the myelin density of white matter. ATAC were not seen in a comparison group of six cases of AD without a prominent aphasia syndrome. Because of the similarity of astrocytes in ATAC to those seen independently of AD pathology in several subtypes of FTLD and two reported cases of PPA we hypothesize that they are a marker of a pathological process concurrent with AD, and related to the focality of the clinical presentation.

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“…There are established associations between semantic-variant PPA (svPPA) and frontotemporal lobar degeneration-TAR DNA binding protein 43 (TDP-43); nonfluentvariant PPA (nfvPPA) and frontotemporal lobar degeneration-tau; and logopenic-variant PPA (lvPPA) and Alzheimer pathology. [1][2][3][4][5] The recommendations on clinical subtyping of PPA have proposed that clinical classification can be supported by imaging according to the pattern of regional atrophy or metabolic impairment. 6 Left posterior frontoinsular atrophy in nfvPPA, anterior temporal atrophy in svPPA, and left posterior peri-Sylvian or parietal atrophy in lvPPA are the recommended atrophy patterns.…”

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confidence: 99%

Can MRI Visual Assessment Differentiate the Variants of Primary-Progressive Aphasia?

Sheikh‐Bahaei

Cross

Gillard

et al. 2017

AJNR Am J Neuroradiol

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BACKGROUND AND PURPOSE:Primary-progressive aphasia is a clinically and pathologically heterogeneous condition. Nonfluent, semantic, and logopenic are the currently recognized clinical variants. The recommendations for the classification of primary-progressive aphasia have advocated variant-specific patterns of atrophy. The aims of the present study were to evaluate the sensitivity and specificity of the proposed imaging criteria and to assess the intra-and interrater reporting agreements.

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Clinical and pathological characterization of progressive aphasia

Abstract: Progressive aphasia is best seen as a composite of two conditions, on both clinical and pathological levels: progressive nonfluent aphasia and semantic dementia.

Cited by 321 publications

References 76 publications

The logopenic/phonological variant of primary progressive aphasia

The logopenic/phonological variant of primary progressive aphasia

Argyrophilic thorny astrocyte clusters in association with Alzheimer’s disease pathology in possible primary progressive aphasia

Can MRI Visual Assessment Differentiate the Variants of Primary-Progressive Aphasia?

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