Semantic dementia (semantic variant primary progressive aphasia)

2016 ◽  
pp. 178-193
Author(s):  
John R. Hodges ◽  
Sharon Savage ◽  
Karalyn E. Patterson
2017 ◽  
Vol 89 (10) ◽  
pp. 1032-1037 ◽  
Author(s):  
W R Bevan-Jones ◽  
Thomas E Cope ◽  
P Simon Jones ◽  
Luca Passamonti ◽  
Young T Hong ◽  
...  

IntroductionSemantic dementia, including the semantic variant of primary progressive aphasia (svPPA), is strongly associated with TAR-DNA binding protein 43 (TDP-43) type C pathology. It provides a useful model in which to test the specificity of in vivo binding of the putative tau ligand [18F]AV-1451, which is elevated in frontotemporal lobar degeneration tauopathies.Methods and resultsSeven patients (five with svPPA and two with ‘right’ semantic dementia) and 12 healthy controls underwent positron emission tomography brain imaging with [18F]AV-1451. Two independent preprocessing methods were used. For both methods, all patients had clearly elevated binding potential (BPND (non-displaceable binding potential)) in temporal lobes, lateralising according to their clinical syndrome and evident in raw images. Region of interest analyses confirmed that BPND was significantly increased in temporal regions, insula and fusiform gyrus, consistent with those areas known to be most affected in semantic dementia. Hierarchical cluster analysis, based on the distribution of [18F]AV-1451 binding potential, separated semantic dementia from controls with 86% sensitivity and 100% specificity.Conclusions[18F]AV-1451 binds in vivo regions that are likely to contain TDP-43 and not significant tau pathology. While this suggests a non-tau target for [18F]AV-1451, the pathological regions in semantic dementia do not normally contain significant levels of recently proposed ‘off target’ binding sites for [18F]AV-1451, such as neuronal monoamine oxidase or neuromelanin. Postmortem and longitudinal data will be useful to assess the utility of [18F]AV-1451 to differentiate and track different types of frontotemporal lobar degeneration.


2021 ◽  
Vol 21 (3) ◽  
Author(s):  
Justina Ruksenaite ◽  
Anna Volkmer ◽  
Jessica Jiang ◽  
Jeremy CS Johnson ◽  
Charles R Marshall ◽  
...  

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.


2021 ◽  
Vol 11 (2) ◽  
pp. 130
Author(s):  
Jeanne Gallée ◽  
Claire Cordella ◽  
Evelina Fedorenko ◽  
Daisy Hochberg ◽  
Alexandra Touroutoglou ◽  
...  

“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.


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

2018 ◽  
Vol 144 (6) ◽  
pp. 3364-3370
Author(s):  
Rene L. Utianski ◽  
Hugo Botha ◽  
Joseph R. Duffy ◽  
Heather M. Clark ◽  
Peter R. Martin ◽  
...  

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