scholarly journals Diagnostic Assessment in Primary Progressive Aphasia: An Illustrative Case Example

2020 ◽  
Vol 29 (4) ◽  
pp. 1833-1849
Author(s):  
Eduardo Europa ◽  
Leonardo Iaccarino ◽  
David C. Perry ◽  
Elizabeth Weis ◽  
Ariane E. Welch ◽  
...  

Purpose Diagnosis and classification of primary progressive aphasia (PPA) requires confirmation of specific speech and language symptoms, highlighting the important role of speech-language pathologists in the evaluation process. The purpose of this case report is to inform speech-language pathologists regarding current practices for diagnostic assessment in PPA, describing standard approaches as well as complementary, state-of-the-art procedures that may improve diagnostic precision. Method We describe the diagnostic evaluation of a 49-year-old woman with complaints of progressive word-finding difficulty. She completed standard neurological, neuropsychological, and speech-language evaluations, as well as magnetic resonance and positron emission tomography imaging of her brain. In addition, a history of developmental speech, language, and learning abilities was obtained, as well as genetic testing and assessment of cerebrospinal fluid biomarkers. We discuss the evaluation results in the context of the most current research related to PPA diagnosis. Conclusion Detailed behavioral assessment, thorough intake of symptom history and neurodevelopmental differences, multimodal neuroimaging, and comprehensive examination of genes and biomarkers are of paramount importance for detecting and characterizing PPA, with ramifications for early behavioral and/or pharmacological intervention. Supplemental Material https://doi.org/10.23641/asha.12771113

Author(s):  
Jennifer M. Ogar

Abstract Speech-language pathologists are increasingly treating patients with progressive disorders, including primary progressive aphasia (PPA). For many years, two variants of PPA were recognized: a nonfluent type—progressive nonfluent aphasia (PNFA)—and a fluent form—semantic dementia (SD). In 2004, a third variant—logopenic progressive aphasia (LPA)—was described. This article will review clinical symptoms, neuroimaging correlates, and the neuropathologies that are typically associated with PNFA, SD, and LPA. Case studies are included to further illustrate the characteristics of each of these three PPA variants.


2020 ◽  
Vol 29 (1S) ◽  
pp. 485-497 ◽  
Author(s):  
E. Susan Duncan ◽  
Neila J. Donovan ◽  
Seyed Ahmad Sajjadi

Purpose We sought to examine interrater reliability in clinical assessment of apraxia of speech (AOS) in individuals with primary progressive aphasia and to identify speech characteristics predictive of AOS diagnosis. Method Fifty-two individuals with primary progressive aphasia were recorded performing a variety of speech tasks. These recordings were viewed by 2 experienced speech-language pathologists, who independently rated them on the presence and severity of AOS as well as 14 associated speech characteristics. We calculated interrater reliability (percent agreement and Cohen's kappa) for these ratings. For each rater, we used stepwise regression to identify speech characteristics significantly predictive of AOS diagnosis. We used the overlap between raters to create a more parsimonious model, which we evaluated with multiple linear regression. Results Results yielded high agreement on the presence (90%) and severity of AOS (weighted Cohen's κ = .834) but lower agreement for specific speech characteristics (weighted Cohen's κ ranging from .036 to .582). Stepwise regression identified 2 speech characteristics predictive of AOS diagnosis for both raters (articulatory groping and increased errors with increased length/complexity). These alone accounted for ≥ 50% of the variance of AOS severity in the constrained model. Conclusions Our study adds to a growing body of research that highlights the difficulty in objective clinical characterization of AOS and perceptual characterization of speech features. It further supports the need for consensus diagnostic criteria with standardized testing tools and for the identification and validation of objective markers of AOS. Additionally, these findings underscore the need for a training protocol if diagnostic tools are to be effective when shared beyond the research teams that develop and test them and disseminated to practicing speech-language pathologists, in order to ensure consistent application.


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