Poster Session 02 Program Schedule
02/15/2024
08:00 am - 09:15 am
Room: Majestic Complex (Posters 61-120)
Poster Session 02: Aging | MCI | Neurodegenerative Disease - PART 1
Final Abstract #94
Longitudinal Evaluation of Semantic Variant Primary Progressive Aphasia: Case Report
Chaniqua Mazyck, University of Houston, Houston, United States
Samantha Henry, Baylor College of Medicine, Houston, United States
Ana Diaz Santos, Cogstate, Ltd., New Haven, United States
Jennifer Stinson, Baylor College of Medicine, Houston, United States
Category: Language and Speech Functions/Aphasia
Keyword 1: dementia - other cortical
Keyword 2: language: aphasia
Keyword 3: language
Objective:
Although there are distinct clinical profiles established for each of the three main primary progressive aphasias in the advanced phase, there is a dearth of research that identifies the early clinical features of semantic variant primary progressive aphasia (svPPA) during disease onset (Rogalski et al., 2011; Sapolsky et al., 2011; Mesulam et al., 2012). It has been shown that early intervention of neurodegenerative disorders has better outcomes for affected individuals compared to those who obtain later intervention (Mangialasche, et al., 2010; Wattmo & Wallin, 2017; Velayudhan et al., 2020). The purpose of this study is to provide a longitudinal case study which highlights early clinical features of svPPA to assist clinicians with early detection and treatment.
Participants and Methods:
Ms. Doe underwent two comprehensive neurological exams and three annual neuropsychological (NP) evaluations between 2018 to 2020. NP evaluations included a clinical interview and comprehensive psychometric testing. Final diagnoses were obtained through comprehensive review of Ms. Doe’s NP evaluation performance, in conjunction with her neurological and medical history.
Results:
During the initial NP evaluation (2018), Ms. Doe specifically complained of difficulty with reading and comprehension, which began 4-5 years prior with insidious onset and progressive decline. Results of testing demonstrated impaired confrontation naming with an otherwise intact neurocognitive profile. Concurrent neuroimaging was done; an MRI revealed focal atrophy of the anterior temporal lobes, bilaterally, right greater than left. Additionally, FDG-PET scan indicated extensive decrease in metabolic activity in the temporal lobes with relative preservation of activity in the posterior cingulate gyrus and parietal lobes.
Follow-up NP evaluation (2019) revealed deficits in confrontation naming and single-word comprehension with impaired object knowledge and spared sentence repetition and speech production. There was evidence of interim decline in executive functioning and changes in personality and neurobehavioral functioning. Combined with neuroimaging done in 2018, she met consensus diagnostic criteria for imaging-supported semantic variant PPA (Gordo-Tempini et al., 2011). Final NP evaluation (2020) revealed continued decline in object knowledge with intact basic comprehension, repetition, and fluency.
Conclusions:
Efforts have been made to better classify PPA variants to increase diagnostic certainty and better tailor interventions for patients and their families. In the current case, languages changes were not detected via formal testing until her second NP evaluation, approximately 5-6 years following her reported symptom onset. This mimics the pattern seen with detection of Alzheimer’s type dementia, with subjective memory complaints pre-dating the detection of memory changes on formal assessment by several years. The current case study highlights the need to develop more sensitive measures for the assessment of language. This is particularly important as the typical age range of onset of PPA is younger than other types of dementia and many are in a period of their lives with high work and family demands. Earlier detection of changes allows patients to make better informed decisions and engage in early intervention.
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