scholarly journals An ABCA7 partial deletion and a GRN variant in a semantic variant of primary progressive aphasia patient

2020 ◽  
Vol 16 (S3) ◽  
Author(s):  
Anna Antonell ◽  
Oscar Ramos‐Campoy ◽  
Mircea Balasa ◽  
Sergi Borrego‐Écija ◽  
Núria Montagut ◽  
...  
Keyword(s):  
Primary Progressive Aphasia ◽  
Partial Deletion ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Semantic Variant ◽  
Aphasia Patient

scholarly journals Primary Progressive Aphasia: Toward a Pathophysiological Synthesis

2021 ◽  
Vol 21 (3) ◽  
Author(s):  
Justina Ruksenaite ◽  
Anna Volkmer ◽  
Jessica Jiang ◽  
Jeremy CS Johnson ◽  
Charles R Marshall ◽  
...  
Keyword(s):  
Care Management ◽  
Primary Progressive Aphasia ◽  
Disease Stage ◽  
Major Step ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
New Findings ◽  
Critical Issues ◽  
Semantic Variant ◽  
Auditory Impairments

Abstract Purpose of Review The term primary progressive aphasia (PPA) refers to a diverse group of dementias that present with prominent and early problems with speech and language. They present considerable challenges to clinicians and researchers. Recent Findings Here, we review critical issues around diagnosis of the three major PPA variants (semantic variant PPA, nonfluent/agrammatic variant PPA, logopenic variant PPA), as well as considering ‘fragmentary’ syndromes. We next consider issues around assessing disease stage, before discussing physiological phenotyping of proteinopathies across the PPA spectrum. We also review evidence for core central auditory impairments in PPA, outline critical challenges associated with treatment, discuss pathophysiological features of each major PPA variant, and conclude with thoughts on key challenges that remain to be addressed. Summary New findings elucidating the pathophysiology of PPA represent a major step forward in our understanding of these diseases, with implications for diagnosis, care, management, and therapies.

scholarly journals Breakdowns in Informativeness of Naturalistic Speech Production in Primary Progressive Aphasia

2021 ◽  
Vol 11 (2) ◽  
pp. 130
Author(s):  
Jeanne Gallée ◽  
Claire Cordella ◽  
Evelina Fedorenko ◽  
Daisy Hochberg ◽  
Alexandra Touroutoglou ◽  
...  
Keyword(s):  
Primary Progressive Aphasia ◽  
Speech Analysis ◽  
Clinical Settings ◽  
Functional Communication ◽  
Or Efficiency ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Communication Impairment ◽  
Semantic Variant ◽  
Lower Production

“Functional communication” refers to an individual’s ability to communicate effectively in his or her everyday environment, and thus is a paramount skill to monitor and target therapeutically in people with aphasia. However, traditional controlled-paradigm assessments commonly used in both research and clinical settings often fail to adequately capture this ability. In the current study, facets of functional communication were measured from picture-elicited speech samples from 70 individuals with mild primary progressive aphasia (PPA), including the three variants, and 31 age-matched controls. Building upon methods recently used by Berube et al. (2019), we measured the informativeness of speech by quantifying the content of each patient’s description that was relevant to a picture relative to the total amount of speech they produced. Importantly, form-based errors, such as mispronunciations of words, unusual word choices, or grammatical mistakes are not penalized in this approach. We found that the relative informativeness, or efficiency, of speech was preserved in non-fluent variant PPA patients as compared with controls, whereas the logopenic and semantic variant PPA patients produced significantly less informative output. Furthermore, reduced informativeness in the semantic variant is attributable to a lower production of content units and a propensity for self-referential tangents, whereas for the logopenic variant, a lower production of content units and relatively ”empty” speech and false starts contribute to this reduction. These findings demonstrate that functional communication impairment does not uniformly affect all the PPA variants and highlight the utility of naturalistic speech analysis for measuring the breakdown of functional communication in PPA.

scholarly journals Dyslexic Characteristics of Chinese-Speaking Semantic Variant of Primary Progressive Aphasia

2018 ◽  
Vol 30 (1) ◽  
pp. 31-37 ◽  
Author(s):  
Simon Kang Seng Ting ◽  
Heidi Foo ◽  
Pei Shi Chia ◽  
Shahul Hameed ◽  
Kok Pin Ng ◽  
...  
Keyword(s):  
Primary Progressive Aphasia ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Semantic Variant

scholarly journals Rapid rate on quasi-speech tasks in the semantic variant of primary progressive aphasia: A non-motor phenomenon?

2018 ◽  
Vol 144 (6) ◽  
pp. 3364-3370
Author(s):  
Rene L. Utianski ◽  
Hugo Botha ◽  
Joseph R. Duffy ◽  
Heather M. Clark ◽  
Peter R. Martin ◽  
...  
Keyword(s):  
Primary Progressive Aphasia ◽  
Rapid Rate ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Semantic Variant

Improving verb anomia in the semantic variant of primary progressive aphasia: the effectiveness of a semantic-phonological cueing treatment

Neurocase ◽  
2014 ◽  
Vol 21 (4) ◽  
pp. 448-456 ◽  
Author(s):  
J. Macoir ◽  
M. Leroy ◽  
S. Routhier ◽  
N. Auclair-Ouellet ◽  
M. Houde ◽  
...  
Keyword(s):  
Primary Progressive Aphasia ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Semantic Variant

scholarly journals Beyond the temporal pole: limbic memory circuit in the semantic variant of primary progressive aphasia

Brain ◽  
2014 ◽  
Vol 137 (7) ◽  
pp. 2065-2076 ◽  
Author(s):  
Rachel H. Tan ◽  
Stephanie Wong ◽  
Jillian J. Kril ◽  
Olivier Piguet ◽  
Michael Hornberger ◽  
...  
Keyword(s):  
Primary Progressive Aphasia ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Memory Circuit ◽  
Temporal Pole ◽  
Semantic Variant
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