Language and Speech Markers of Primary Progressive Aphasia: A Systematic Review

Purpose This systematic review aimed to establish language and speech markers to support the clinical diagnosis of primary progressive aphasia (PPA) and its clinical phenotypes. Our first objective was to identify behavioral language and speech markers of early-stage PPA. Our second objective was to identify the electrophysiological correlates of the language and speech characteristics in PPA. Method The databases MEDLINE, Web of Science, and Embase were searched for relevant articles. To identify behavioral markers, the initial subjective complaints and the language and speech deficits detected during the initial diagnostic evaluation were summarized for PPA in general and each clinical variant according to the 2011 consensus diagnostic criteria (nonfluent variant [NFV], semantic variant, and logopenic variant [LV]). To identify electrophysiological markers, the studies in which event-related potentials (ERPs) were elicited by a language or speech paradigm in patients with PPA were included. Results In total, 114 relevant studies were identified, including 110 behavioral studies and only four electrophysiological studies. This review suggests that patients with the semantic variant could be accurately differentiated from the NFV and LV in the initial stages based on the consensus criteria. Nonetheless, the early differentiation between the NFV and LV is not straightforward. In the four electrophysiological studies, differences in the latency, amplitude, and topographical distribution of the semantic N400 component were found between patients with PPA and healthy controls. Conclusions To accurately differentiate the NFV from the LV, it could be important to assess the language and speech degeneration by more specific assessments and by more objective diagnostic methods that offer insights into the language-related processes. Electrophysiological markers of PPA were not identified in this review due to the low number of studies that investigated language-related ERPs. More controlled ERP studies in larger patient cohorts are needed to investigate the diagnostic applicability of language-related ERPs in PPA. Supplemental Material https://doi.org/10.23641/asha.12798080

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Slowing of Event-Related Potentials in Primary Progressive Aphasia. A case report

The Scientific World JOURNAL ◽

10.1100/tsw.2009.67 ◽

2009 ◽

Vol 9 ◽

pp. 633-638 ◽

Cited By ~ 2

Author(s):

Salvatore Giaquinto ◽

Francesca Ranghi

Keyword(s):

Word Recognition ◽

Cognitive Abilities ◽

Single Case ◽

Electrical Potential ◽

Primary Progressive Aphasia ◽

Event Related Potentials ◽

Control Group ◽

Progressive Aphasia ◽

Primary Progressive ◽

Related Potentials

Primary Progressive Aphasia (PPA) is a rare and insidious language impairment that worsens over time. It belongs to the group of fronto–temporal dementias. This study was aimed at assessing the role of speed of cognitive abilities, such as word recognition, in PPA. The design is a single-case, longitudinal study. A male patient suffering from PPA was enrolled and 15 healthy older adults were the control group. An event-related electrical potential connected with word recognition, namely the N400, was delayed by 200 msec at baseline compared to healthy controls and progressively deteriorated. One year later, the delay was greater and two years later the potential had disappeared. Reduced speed of processing is an early pathological factor that negatively affecting higher cognitive functions in APP. Event–related electrical potentials are recommended in the field of aphasia and cognitive decline. They permit observation of a speed decline in higher cognitive abilities, when pathological changes at a central level begin and language comprehension seems to be unaffected.

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Supplemental Material for The Concept of Regularization: Resolving the Problem of Surface Dyslexia in Semantic Variant Primary Progressive Aphasia Across Different Languages

Neuropsychology ◽

10.1037/neu0000611.supp ◽

2019 ◽

Keyword(s):

Primary Progressive Aphasia ◽

Progressive Aphasia ◽

Primary Progressive ◽

Semantic Variant ◽

Surface Dyslexia

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Primary Progressive Aphasia: Toward a Pathophysiological Synthesis

Current Neurology and Neuroscience Reports ◽

10.1007/s11910-021-01097-z ◽

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.

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Breakdowns in Informativeness of Naturalistic Speech Production in Primary Progressive Aphasia

Brain Sciences ◽

10.3390/brainsci11020130 ◽

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.

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