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Diagnostic Assessment in Primary Progressive Aphasia: An Illustrative Case Example.

ABSTRACT: 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.

SUBMITTER: Europa E

PROVIDER: S-EPMC8740567 | biostudies-literature | 2020 Nov

REPOSITORIES: biostudies-literature

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Diagnostic Assessment in Primary Progressive Aphasia: An Illustrative Case Example.

Europa Eduardo E Iaccarino Leonardo L Perry David C DC Weis Elizabeth E Welch Ariane E AE Rabinovici Gil D GD Miller Bruce L BL Gorno-Tempini Maria Luisa ML Henry Maya L ML

American journal of speech-language pathology 20200910 4

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 d ...[more]

PMID: 32910678

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Project description:Aphasia typically is associated with comparable difficulties in written and spoken modalities of language expression and comprehension. In contrast, auditory verbal agnosia is the disproportionate difficulty comprehending spoken compared to written language, also typically greater than difficulties with spoken and written language expression, in the absence of a primary sensory deficit. The terms pure word deafness and auditory verbal agnosia are often used synonymously. However, the broader term of auditory agnosia more accurately reflects difficulty processing both speech and non-speech sounds whereas individuals with auditory verbal agnosia (pure word deafness) have preserved processing of environmental sounds. Auditory agnosia is reported in the stroke literature, but rarely reported in progressive neurologic disorders. Here, we report a case of a woman who presented with what is best described as a prominent auditory deficit in the context of an initially unclassifiable, or mixed, primary progressive aphasia (PPA) with accompanying apraxia of speech. Her clinical presentation shared features with auditory agnosia, although sensory functioning was not formally assessed. We report clinical and neuroimaging data spanning 6 years and subsequent autopsy results. She presented at 65 years of age, 5 years post onset of symptoms that included insidious and progressive difficulties thinking of words, constructing sentences, pronouncing words, and understanding instructions. She had disproportionate difficulty with comprehension of spoken compared to written language. She eventually developed features of the nonfluent/agrammatic variant of PPA, as well as an apraxia of speech. Imaging with [18F]-fluorodeoxyglucose (FDG)-PET revealed progression of bilateral (left greater than right) hypometabolism involving the frontal, temporal (predominantly the lateral superior gyrus), and parietal lobes, that eventually included the supplementary motor area, anterior cingulate, and caudate. Autopsy revealed pathological lesions consistent with corticobasal degeneration.

Project description:Background and purposePrimary-progressive aphasia is a clinically and pathologically heterogeneous condition. Nonfluent, semantic, and logopenic are the currently recognized clinical variants. The recommendations for the classification of primary-progressive aphasia have advocated variant-specific patterns of atrophy. The aims of the present study were to evaluate the sensitivity and specificity of the proposed imaging criteria and to assess the intra- and interrater reporting agreements.Materials and methodsThe cohort comprised 51 patients with a root diagnosis of primary-progressive aphasia, 25 patients with typical Alzheimer disease, and 26 matched control participants. Group-level analysis (voxel-based morphometry) confirmed the proposed atrophy patterns for the 3 syndromes. The individual T1-weighted anatomic images were reported by 3 senior neuroradiologists.ResultsWe observed a dichotomized pattern of high sensitivity (92%) and specificity (93%) for the proposed atrophy pattern of semantic-variant primary-progressive aphasia and low sensitivity (21% for nonfluent-variant primary-progressive aphasia and 43% for logopenic-variant primary-progressive aphasia) but high specificity (91% for nonfluent-variant primary-progressive aphasia and 95% for logopenic-variant primary-progressive aphasia) in other primary-progressive aphasia variants and Alzheimer disease (sensitivity 43%, specificity 92%). MR imaging was least sensitive for the diagnosis of nonfluent-variant primary-progressive aphasia. Intrarater agreement analysis showed mean κ values above the widely accepted threshold of 0.6 (mean, 0.63 ± 0.16). Pair-wise interobserver agreement outcomes, however, were well below this threshold in 5 of the 6 possible interrater contrasts (mean, 0.41 ± 0.09).ConclusionsWhile the group-level results were in precise agreement with the recommendations, semantic-variant primary-progressive aphasia was the only subtype for which the proposed recommendations were both sensitive and specific at an individual level.

Project description:IntroductionPrimary progressive aphasia (PPA) is divided into three prototypical subtypes that are all characterized by their single core symptom of aphasia. Although later in their course, other cognitive, behavioral, and motor domains may become involved, little is known about the progression profile of each subtype relative to the other subtypes.MethodsIn this longitudinal retrospective cohort study, based on the recent biomarker-supported diagnostic criteria, 24 subjects diagnosed with semantic variant (svPPA), 22 with non-fluent variant (nfvPPA), and 18 with logopenic variant (lvPPA) were collected and followed up for 1-6 years. Symptom distribution, cognitive test and neuropsychiatric inventory scores, and progression into another syndrome were assessed.ResultsOver time, lvPPA progressed with broader language problems (PPA-extended) and nfvPPA progressed to mutism, whereas semantic impairment remained the major problem in svPPA. Apart from linguistic problems, svPPA developed pronounced behavioral disturbances, whereas lvPPA exhibited a greater cognitive decline. By contrast, in nfvPPA motor deficits were more common. Furthermore, within 5 years (IQR = 2.5) after clinical onset, 65.6% of the patients additionally fulfilled the clinical criteria for another neurodegenerative syndrome (PPA-plus). Fourteen out of 24 (58%) svPPA patients additionally met the diagnostic criteria of behavioral variant frontotemporal dementia (5.1 years, IQR = 1.1), whereas the clinical features of 15/18 (83%) lvPPA patients were consistent with Alzheimer disease dementia (4.5 years IQR = 3.4). Furthermore, 12/22 (54%) of the subjects with the nfvPPA progressed to meet the diagnostic criteria of corticobasal syndrome, progressive supranuclear palsy, or motor neuron disease (5.1 years IQR = 3.4).DiscussionDespite aphasia being the initial and unique hallmark of the syndrome, our longitudinal results showed that PPA is not a language limited disorder and progression differs widely for each subtype, both with respect to the nature of symptoms and disease duration.

Project description:Purpose This study sought to determine the initial feasibility and benefit of a novel intervention that combines speech-language treatment with counseling treatment for an individual with the nonfluent/agrammatic variant of primary progressive aphasia (PPA). Method Using a single-case experimental design, we evaluated the utility of modified script training paired with aphasia-modified cognitive behavioral therapy. The study employed a multiple baseline design across scripts for the primary linguistic outcome measure and a mixed methods approach for analyzing counseling outcomes. Psychosocial and communicative functioning scales were administered in conjunction with a phenomenological analysis of semi-structured interviews. Results The participant completed all study phases and participated in all treatment components. She met the criterion of 90% correct, intelligible scripted words on all trained scripts through 12 months post-treatment. Treatment outcomes were comparable to a comparison cohort that received script training without counseling (Henry et al., 2018). At post-treatment, the participant demonstrated stability or improvement on all measures of psychosocial and communicative functioning, with stability documented on seven out of 11 scales at follow-ups through 12 months post-treatment. A phenomenological analysis revealed pervasive themes of loss and resilience at both time points, and emerging themes of positive self-perception, sense of agency, and emotional attunement following treatment. Conclusions Results indicate that script training with aphasia-modified cognitive behavioral therapy is a feasible treatment for an individual with the nonfluent/agrammatic variant of PPA, with immediate and lasting benefits to speech-language production and psychosocial functioning. These findings are the first to support the integration of personal adjustment counseling techniques within a speech-language treatment paradigm for PPA. Supplemental Material https://doi.org/10.23641/asha.14925330.

Dataset Information

Diagnostic Assessment in Primary Progressive Aphasia: An Illustrative Case Example.

Publications

Diagnostic Assessment in Primary Progressive Aphasia: An Illustrative Case Example.

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