Intensive treatment of naming in semantic Primary Progressive Aphasia using semantic feature analysis: case report

Background/Objectives: Primary Progressive Aphasia (PPA) is a neurodegenerative disorder characterized by gradual and progressive deterioration of speech and language abilities. Speech and language therapy is considered an important intervention to slow decline and support the recovery of linguistic functions in individuals with PPA. This study aims to examine the effectiveness of an elaborated Semantic Feature Analysis (SFA) approach in enhancing naming abilities and semantic networks in individuals with the logopenic and semantic variants of PPA. Methods: Fourteen participants were recruited, including seven individuals with logopenic PPA and seven with semantic PPA. All participants received an elaborated SFA intervention twice weekly for four weeks. The Aphasia Language Assessment Test (ADD), the Turkish Picture Naming Test (T-RAT), and the SAQOL-39 were conducted at the following three time points: prior to treatment (pre-test), immediately after treatment (post-test), and one month post-treatment (follow-up). Results: Significant improvements were observed in ADD, T-RAT, and SAQOL-39 scores in both logopenic and semantic PPA groups following treatment (p < 0.05). Although follow-up scores declined compared to posttest performance (p < 0.05), several follow-up scores remained higher than pretest levels. Between-group comparisons indicated no significant difference in ADD scores; however, logopenic PPA participants demonstrated higher T-RAT scores (p < 0.05), while semantic PPA participants showed higher SAQOL-39 scores, except at follow-up (p < 0.05). Conclusions: Preliminary results suggest that the elaborated SFA intervention is effective in improving naming skills, language functioning, and quality of life in both logopenic and semantic variants of PPA. Although treatment gains partially decreased after one month, many improvements were maintained above baseline, supporting the clinical value of SFA in managing language decline in PPA.

Background Primary progressive aphasia (PPA) is a neurodegenerative disorder characterized by a progressive decline of language functions. Its symptoms are grouped into three PPA variants: nonfluent PPA, logopenic PPA, and semantic PPA. Grammatical deficiencies differ depending on the PPA variant. Aims This study aims to determine the differences between PPA variants with respect to part of speech (POS) production and to identify morphological markers that classify PPA variants using machine learning. By fulfilling these aims, the overarching goal is to provide objective measures that can facilitate clinical diagnosis, evaluation, and prognosis. Method and Procedure Connected speech productions from PPA patients produced in a picture description task were transcribed, and the POS class of each word was estimated using natural language processing, namely, POS tagging. We then implemented a twofold analysis: (a) linear regression to determine how patients with nonfluent PPA, semantic PPA, and logopenic PPA variants differ in their POS productions and (b) a supervised classification analysis based on POS using machine learning models (i.e., random forests, decision trees, and support vector machines) to subtype PPA variants and generate feature importance (FI). Outcome and Results Using an automated analysis of a short picture description task, this study showed that content versus function words can distinguish patients with nonfluent PPA, semantic PPA, and logopenic PPA variants. Verbs were less important as distinguishing features of patients with different PPA variants than earlier thought. Finally, the study showed that among the most important distinguishing features of PPA variants were elaborative speech elements, such as adjectives and adverbs.

Event Abstract Back to Event Gray matter predictors of treatment outcomes in semantic variant primary progressive aphasia Heather R. Dial1*, Stephanie M. Grasso1, H. I. Hubbard2*, Kristin Schaffer1, Lisa Wauters1, Eduardo Europa3, Lindsey Wineholt4, Maria Luisa Gorno-Tempini3 and Maya Henry1 1 University of Texas at Austin, United States 2 University of Kentucky, United States 3 University of California, San Francisco, United States 4 Dell Medical School, The University of Texas at Austin, United States Introduction/Method. Semantic variant primary progressive aphasia (svPPA) is a neurodegenerative disorder characterized by anomia and single-word comprehension impairment in the context of anterior temporal lobe atrophy (left > right; Gorno-Tempini et al., 2011). Despite growing evidence supporting anomia treatment in svPPA, there is very little research investigating the neural bases of responsiveness to intervention. Existing research suggests relatively spared regions within the left hemisphere language network and right hemisphere language homologues may support treatment-induced gains (Dressel et al., 2010; Jokel et al., 2016; Meyer et al., 2017). Episodic memory has also been implicated in vocabulary re-learning in svPPA, suggesting a role for medial temporal lobe structures (Snowden & Neary, 2002). In the current study, we examined the relation between pre-treatment gray matter (GM) volumes and treatment outcomes following a lexical retrieval treatment in sixteen individuals with svPPA. Participants completed treatment designed to capitalize on residual semantic, episodic, orthographic and phonological processes to support word retrieval (e.g., Henry et al., in press). Voxel-based morphometry (VBM) was used to a) determine regions of significant atrophy in svPPA participants relative to controls and b) examine the relation between treatment outcomes (effect sizes for trained and untrained items) and pre-treatment GM volumes using both whole-brain and region of interest (ROI) approaches. Based on previous research, bilateral hippocampi as well as bilateral parahippocampal; superior, middle and inferior temporal; inferior and middle frontal; and inferior parietal gyri were selected as ROIs and examined relative to treatment outcomes via Pearson’s correlation. To determine whether regional GM volumes provide predictive value beyond overall disease severity and behavioral assessments, all analyses included total GM volume, Mini Mental State Exam and Boston Naming Test scores as covariates. Results/Conclusion. Following treatment, participants showed improvement on trained items (M d = 7.85, range = 1.28 to 19.36), with generalization to untrained items for some participants (M d = 1.27, range = -0.58 to 4.55). SvPPA participants showed left greater than right anterior temporal atrophy relative to controls (Figure 1a). Gains in naming for trained items were predicted by GM volumes in left inferior and middle frontal gyri and right inferior parietal cortex; generalization to untrained items was predicted by left superior frontal and parietal regions and left hippocampus and parahippocampal gyrus (Figure 1b-c). ROI analyses confirmed the relation between GM volumes in left hippocampus and gains for untrained items (Figure 1d, r = 0.79, FDR-corrected p < 0.01), but no relation was observed for other ROIs for trained or untrained items. Thus, the structural integrity of anatomically preserved left hemisphere language network regions and homologous right hemisphere regions is predictive of improvement for trained and untrained items, suggesting that relatively spared linguistic processes support treatment-induced gains. Generalization to untrained items was also related to left hemisphere medial temporal lobe structures. Improved performance on untrained items may reflect the use of trained strategies to assist word retrieval, capitalizing on spared episodic memory for strategic components of the intervention. Future research should explore the relation between episodic memory and treatment outcomes in svPPA. Figure 1 Acknowledgements This research was supported by NIDCD Grant R01NS050915 (Maria Luisa Gorno-Tempini); NIDCD Grant R01DC016291 (Maya L. Henry); NIDCD Grant R03DC013403 (Maya L. Henry); NIDCD Grant F31DC016229 (Stephanie M. Grasso); and NIDCD Grant F32DC016812 (Heather R. Dial). References Dressel, K., Huber, W., Frings, L., Kummerer, D., Saur, D., Mader, I., … Abel, S. (2010). Model-oriented naming therapy in semantic dementia: A single-case fMRI study. Aphasiology, 24, 1537-1558. Gorno-Tempini, M., Hillis, A., Weintraub, S., Kertesz, A., Mendez, M., Cappa, S., …Grossman, M. (2011). Classification of primary progressive aphasia and its variants. Neurology, 76, 1006-1014. Henry, M., Hubbard, H.I., Grasso, S., Dial, H., Beeson, P., Miller, B., & Gorno-Tempini, M.L. (in press). Treatment for word retrieval in semantic and logopenic variants of primary progressive aphasia: Immediate and long-term outcomes. Journal of Speech, Language, and Hearing Research. 1-27. Jokel, R., Kielar, A., Anderson, N., Black, S., Rochon, E., Graham, S., … Tang-Wai, D. (2016). Behavioral and neuroimaging changes after naming therapy for semantic variant primary progressive aphasia. Neuropsychologia, 89, 191-216. Meyer, A., Faria, A., Tippett, D., Hillis, A., & Friedman, R. (2017). The relationship between baseline volume in temporal areas and post-treatment naming accuracy in primary progressive aphasia. Aphasiology, 31, 1059-1077. Snowden, J., & Neary, D. (2002). Relearning of verbal labels in semantic dementia. Neuropsychologia, 40, 1715-1728. Keywords: semantic variant (svPPA), primary progressive aphasia, speech-language intervention, voxel-based morphometry, VBM, neural bases of treatment outcomes Conference: Academy of Aphasia 57th Annual Meeting, Macau, Macao, SAR China, 27 Oct - 29 Oct, 2019. Presentation Type: Poster presentation Topic: Not eligible for student award Citation: Dial HR, Grasso SM, Hubbard HI, Schaffer K, Wauters L, Europa E, Wineholt L, Gorno-Tempini M and Henry M (2019). Gray matter predictors of treatment outcomes in semantic variant primary progressive aphasia. Front. Hum. Neurosci. Conference Abstract: Academy of Aphasia 57th Annual Meeting. doi: 10.3389/conf.fnhum.2019.01.00118 Copyright: The abstracts in this collection have not been subject to any Frontiers peer review or checks, and are not endorsed by Frontiers. They are made available through the Frontiers publishing platform as a service to conference organizers and presenters. The copyright in the individual abstracts is owned by the author of each abstract or his/her employer unless otherwise stated. Each abstract, as well as the collection of abstracts, are published under a Creative Commons CC-BY 4.0 (attribution) licence (https://creativecommons.org/licenses/by/4.0/) and may thus be reproduced, translated, adapted and be the subject of derivative works provided the authors and Frontiers are attributed. For Frontiers’ terms and conditions please see https://www.frontiersin.org/legal/terms-and-conditions. Received: 06 May 2019; Published Online: 09 Oct 2019. * Correspondence: PhD. Heather R Dial, University of Texas at Austin, Austin, Texas, 78705, United States, heather.raye.dial@gmail.com PhD. H. I Hubbard, University of Kentucky, Lexington, Kentucky, 40506, United States, isabelhubbard@uky.edu Login Required This action requires you to be registered with Frontiers and logged in. To register or login click here. Abstract Info Abstract The Authors in Frontiers Heather R Dial Stephanie M Grasso H. I Hubbard Kristin Schaffer Lisa Wauters Eduardo Europa Lindsey Wineholt Maria Luisa Gorno-Tempini Maya Henry Google Heather R Dial Stephanie M Grasso H. I Hubbard Kristin Schaffer Lisa Wauters Eduardo Europa Lindsey Wineholt Maria Luisa Gorno-Tempini Maya Henry Google Scholar Heather R Dial Stephanie M Grasso H. I Hubbard Kristin Schaffer Lisa Wauters Eduardo Europa Lindsey Wineholt Maria Luisa Gorno-Tempini Maya Henry PubMed Heather R Dial Stephanie M Grasso H. I Hubbard Kristin Schaffer Lisa Wauters Eduardo Europa Lindsey Wineholt Maria Luisa Gorno-Tempini Maya Henry Related Article in Frontiers Google Scholar PubMed Abstract Close Back to top Javascript is disabled. Please enable Javascript in your browser settings in order to see all the content on this page.

Purpose Recent studies confirm the utility of speech-language intervention in primary progressive aphasia (PPA); however, long-term outcomes, ideal dosage parameters, and relative benefits of intervention across clinical variants warrant additional investigation. The purpose of this study was to determine whether naming treatment affords significant, lasting, and generalized improvement for individuals with semantic and logopenic PPA and whether dosage manipulations significantly affect treatment outcomes. Method Eighteen individuals with PPA (9 semantic and 9 logopenic variant) underwent lexical retrieval treatment designed to leverage spared cognitive-linguistic domains and develop self-cueing strategies to promote naming. One group (n = 10) underwent once-weekly treatment sessions, and the other group (n = 8) received the same treatment with 2 sessions per week and an additional "booster" treatment phase at 3 months post-treatment. Performance on trained and untrained targets/tasks was measured immediately after treatment and at 3, 6, and 12 months post-treatment. Results Outcomes from the full cohort of individuals with PPA showed significantly improved naming of trained items immediately post-treatment and at all follow-up assessments through 1 year. Generalized improvement on untrained items was significant up to 6 months post-treatment. The positive response to treatment was comparable regardless of session frequency or inclusion of a booster phase. Outcomes were comparable across PPA subtypes, as was maintenance of gains over the post-treatment period. Conclusion This study documents positive naming treatment outcomes for a group of individuals with PPA, demonstrating strong direct treatment effects, maintenance of gains up to 1 year post-treatment, and generalization to untrained items. Lexical retrieval treatment, in conjunction with daily home practice, had a strong positive effect that did not require more than 1 clinician-directed treatment session per week. Findings confirm that strategic training designed to capitalize on spared cognitive-linguistic abilities results in significant and lasting improvement, despite ongoing disease progression, in PPA.

Utilization of resting-state electroencephalography spectral power in convolutional neural networks for classification of primary progressive aphasia

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Objective: Our aim was to report diffusion tensor imaging (DTI) patterns in three patients, each with a different primary progressive aphasia (PPA) variant. Design: One agrammatic PPA, one semantic PPA, and one logopenic PPA subject underwent a magnetic resonance imaging examination including DTI sequences. The fractional anisotropy (FA) value was calculated in regions of interest (ROIs) involved in these three variants (perisylvian region, temporal pole, and parietotemporal junction) for each patient. Left-right FA ratios in each ROI were compared between PPA subjects and a group of three amnestic mild cognitive impairment patients with a cerebrospinal fluid biomarker profile of the Alzheimer type. Results: The FA values were lower in the left hemisphere (p = 0.03). The lowest FA values were observed in the left perisylvian region for the non-fluent/agrammatic subtype PPA patient, in the left anterior temporal lobe for the semantic subtype PPA patient, and in the left parietotemporal junction for the logopenic patient (p = 0.028). The left-right FA ratio in these specific ROIs for each PPA variant was significantly lower than in the amnestic mild cognitive impairment group (p = 0.009). Conclusion: DTI patterns could be an effective new tool for diagnosing PPA and classifying the three variants.

BackgroundHearing loss has emerged as a marker of cognitive decline and potential treatment target in dementia, however the symptom profiles that characterise hearing impairment in different dementia syndromes are poorly characterised. Beyond sound detection, these diseases impair aspects of auditory perception that are crucial for communication in daily life. Here we addressed changes in everyday hearing function in Alzheimer’s disease and frontotemporal dementia syndromes, using standardised auditory symptom questionnaires completed by patients’ caregivers.MethodPrimary caregivers for patients with Alzheimer’s disease (n = 20), logopenic variant primary progressive aphasia (n = 8), nonfluent variant primary progressive aphasia (n = 13), semantic variant primary progressive aphasia (n = 13) and behavioural variant frontotemporal dementia (n = 18) completed the Hearing Impairment Impact – Significant Other Profile, adapted Amsterdam Inventory for Auditory Disability and Khalfa Hyperacusis questionnaires. The Amsterdam Inventory for Auditory Disability was also completed by 15 healthy age‐matched individuals. No participant had a past history of significant otological disease.ResultScores on the Hearing Impairment Impact – Significant Other Profile and Khalfa Hyperacusis Questionnaire respectively demonstrated greater carer burden and increased sensitivity to sound in the behavioural variant frontotemporal dementia group compared with the Alzheimer’s disease group. On the Amsterdam Inventory for Auditory Disability, multi‐domain hearing difficulties were endorsed for the behavioural variant frontotemporal dementia and primary progressive aphasia groups relative to healthy controls, and total scores in the logopenic and semantic variant primary progressive aphasia groups were significantly worse than both the healthy control and Alzheimer’s disease groups. Syndromic groups showed differentiable hearing profiles across hearing domains.ConclusionMajor dementia syndromes have distinct profiles of everyday hearing difficulty, and patients with frontotemporal dementia and progressive aphasia syndromes may have particularly marked (albeit often unrecognised) changes in their hearing. These findings should motivate the development of bespoke auditory tests to assess central hearing function and personalised hearing management strategies in these diseases.

Semantic Feature Analysis (SFA) is a treatment technique designed to improve lexical retrieval by increasing the level of activation within a semantic network. The purpose of this study was to replicate the Boyle and Coelho (1995) study in which SFA was applied with a mild non-fluent aphasic individual resulting in improved confrontation naming of trained and untrained items but no generalization to connected speech. The present study investigated whether a comparable treatment effect could be demonstrated, and to what extent severity and type aphasia might impact overall outcome. SFA was applied to an individual with a moderate fluent aphasia secondary to a closed head injury. Gains in confrontation naming of both trained and untrained stimulus pictures were noted as well as measures of connected speech. Potential explanations for these findings are discussed.

Am I looking at a cat or a dog? Gaze in the semantic variant of primary progressive aphasia is subject to excessive taxonomic capture.

Primary progressive aphasia, a neurodegenerative syndrome, presents mainly with language impairment. Both semantic and logopenic variants are fluent variants of primary progressive aphasia. Before the research criteria of primary progressive aphasia were proposed, progressive fluent aphasias, such as progressive anomic aphasia, transcortical sensory aphasia and Wernicke’s aphasia, were reported as classical progressive fluent aphasias seen in Alzheimer’s disease. However, since the research criteria of primary progressive aphasia were established, classical fluent variants (other than semantic and logopenic variants) have been neglected and have not been included in the current classification of primary progressive aphasia. This study aimed to determine whether unclassified fluent variants (other than semantic and logopenic variants) can be manifestations of primary progressive aphasia. This study also reconfirmed the characteristics of classical progressive fluent aphasia, such as progressive anomic aphasia, progressive transcortical sensory aphasia and progressive Wernicke’s aphasia as unclassified fluent variants of primary progressive aphasia, using comparison with the current model of primary progressive aphasia. Twelve consecutive patients with an unclassified fluent variant other than semantic or logopenic variant underwent language, neurological, neuropsychological and neuroimaging (MRI and single-photon emission computed tomography) testing. Based on comprehensive language tests, we redefined the diagnoses as primary progressive anomic aphasia (n = 8), primary progressive transcortical sensory aphasia (n = 3) and primary progressive Wernicke’s aphasia (n = 1). Anomic aphasia was characterized by anomia but preserved repetition and comprehension; transcortical sensory aphasia by relatively preserved repetition but poor word comprehension; and Wernicke’s aphasia by poor repetition and word comprehension. In patients with anomic aphasia, voxel-based morphometry of MRI data revealed cortical atrophy, which was most prominent in the temporoparietal lobes, with no obvious lateralization; in two-thirds of patients with transcortical sensory aphasia and in one patient with Wernicke’s aphasia, it revealed atrophy, predominantly in the left temporoparietal lobe. Statistical analysis of single-photon emission computed tomography using three-dimensional stereotactic surface projections revealed patterns of left-sided hypoperfusion in the majority of patients. The temporal and parietal lobes were involved in all cases; the degree of hypoperfusion was higher in patients with transcortical sensory aphasia or Wernicke’s aphasia than in patients with anomic aphasia. The present study demonstrated the clinical and imaging features of 12 patients with an unclassified fluent variant of primary progressive aphasia, which we redefined as primary progressive anomic aphasia, primary progressive transcortical sensory aphasia and primary progressive Wernicke’s aphasia. Classical fluent variants other than semantic and logopenic variants can be found in primary progressive aphasia.

Successful communication in daily life depends on accurate decoding of speech signals that are acoustically degraded by challenging listening conditions. This process presents the brain with a demanding computational task that is vulnerable to neurodegenerative pathologies. However, despite recent intense interest in the link between hearing impairment and dementia, comprehension of acoustically degraded speech in these diseases has been little studied. Here we addressed this issue in a cohort of 19 patients with typical Alzheimer’s disease and 30 patients representing the three canonical syndromes of primary progressive aphasia (non-fluent/agrammatic variant primary progressive aphasia; semantic variant primary progressive aphasia; logopenic variant primary progressive aphasia), compared to 25 healthy age-matched controls. As a paradigm for the acoustically degraded speech signals of daily life, we used noise-vocoding: synthetic division of the speech signal into frequency channels constituted from amplitude-modulated white noise, such that fewer channels convey less spectrotemporal detail thereby reducing intelligibility.We investigated the impact of noise-vocoding on recognition of spoken three-digit numbers and used psychometric modelling to ascertain the threshold number of noise-vocoding channels required for 50% intelligibility by each participant. Associations of noise-vocoded speech intelligibility threshold with general demographic, clinical and neuropsychological characteristics and regional grey matter volume (defined by voxel-based morphometry of patients’ brain images) were also assessed. Mean noise-vocoded speech intelligibility threshold was significantly higher in all patient groups than healthy controls, and significantly higher in Alzheimer’s disease and logopenic variant primary progressive aphasia than semantic variant primary progressive aphasia (all P < 0.05). In a receiver operating characteristic analysis, vocoded intelligibility threshold discriminated Alzheimer’s disease, non-fluent variant and logopenic variant primary progressive aphasia patients very well from healthy controls. Further, this central hearing measure correlated with overall disease severity but not with peripheral hearing or clear speech perception. Neuroanatomically, after correcting for multiple voxel-wise comparisons in predefined regions of interest, impaired noise-vocoded speech comprehension across syndromes was significantly associated (P < 0.05) with atrophy of left planum temporale, angular gyrus and anterior cingulate gyrus: a cortical network that has previously been widely implicated in processing degraded speech signals.Our findings suggest that the comprehension of acoustically altered speech captures an auditory brain process relevant to daily hearing and communication in major dementia syndromes, with novel diagnostic and therapeutic implications.

The primary progressive aphasias (PPA) are a heterogeneous group of language-led neurodegenerative diseases resulting from large-scale brain network degeneration. White matter (WM) pathways bind networks together, and might therefore hold information about PPA pathogenesis. Here we used diffusion tensor imaging and tract-based spatial statistics to compare WM tract changes between PPA syndromes and with respect to Alzheimer's disease and healthy controls in 33 patients with PPA (13 nonfluent/agrammatic PPA); 10 logopenic variant PPA; and 10 semantic variant PPA. Nonfluent/agrammatic PPA was associated with predominantly left-sided and anterior tract alterations including uncinate fasciculus (UF) and subcortical projections; semantic variant PPA with bilateral alterations in inferior longitudinal fasciculus and UF; and logopenic variant PPA with bilateral but predominantly left-sided alterations in inferior longitudinal fasciculus, UF, superior longitudinal fasciculus, and subcortical projections. Tract alterations were more extensive than gray matter alterations, and the extent of alteration across tracts and PPA syndromes varied between diffusivity metrics. These WM signatures of PPA syndromes illustrate the selective vulnerability of brain language networks in these diseases and might have some pathologic specificity.

SHORT-TERM REHABILITATION FOR LINGUISTIC IMPAIRMENTS IN PRIMARY PROGRESSIVE APHASIA: A CASE SERIES

BackgroundLanguage assessment is critical in the diagnosis of patients with neurodegenerative diseases, in particular those presenting with progressive speech and language impairment such as the primary progressive aphasias (PPA). Current diagnostic criteria identify three main variants of PPA based on clinical and neuroimaging features: semantic variant PPA ‐ characterised by dramatically reduced ability to understand the meaning of words and objects; nonfluent variant PPA – featuring problems with the grammatical and phonetic aspects of language production; and logopenic variant PPA – characterised by a striking inability to repeat spoken sentences. Most currently utilised assessments of the heterogeneous language profiles of PPA are time‐consuming and not readily comparable across languages. Improved tools to screen, diagnose, and monitor these conditions will be essential to progress in the characterisation and treatments for brain diseases underlying PPAs. The aim of the current project is to develop and fully validate English and Italian versions of an abbreviated language assessment instrument, the Mini Linguistic State Examination (MLSE), which will be able to detect the differing patterns of linguistic deficit that characterise the three variants of PPA with complete equivalence between English and Italian versions.MethodsParticipants completed the MLSE test which included tasks of picture naming, word and sentence repetition, word and sentence comprehension, semantic association, reading, writing, and picture description.ResultsData were used to present contrasting characteristics of the MLSE performance in 61 patients with PPA (20 PNFA, 17 SD, and 24 LPA), and a matched control group (40 controls; aged: 45‐75 years).ConclusionsThe MLSE will provide a much‐needed short clinical tool to classify and monitor PPA. Additionally, the MLSE will be a template for further language‐specific versions.