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Prominent auditory deficits in primary progressive aphasia: A case study.

ABSTRACT: 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.

SUBMITTER: Utianski RL

PROVIDER: S-EPMC6650352 | biostudies-literature | 2019 Aug

REPOSITORIES: biostudies-literature

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Prominent auditory deficits in primary progressive aphasia: A case study.

Utianski Rene L RL Duffy Joseph R JR Clark Heather M HM Machulda Mary M MM Dickson Dennis W DW Whitwell Jennifer L JL Josephs Keith A KA

Cortex; a journal devoted to the study of the nervous system and behavior 20190221

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 ter ...[more]

PMID: 30878181

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Project description:BackgroundA subset of patients with the nonfluent variant of primary progressive aphasia (PPA) exhibit concomitant single-word comprehension problems, constituting a 'mixed variant' phenotype. This phenotype is rare and currently not fully characterized. The aim of this study was twofold: to assess the prevalence and nature of single-word comprehension problems in the nonfluent variant and to study multimodal imaging characteristics of atrophy, tau, and amyloid burden associated with this mixed phenotype.MethodsA consecutive memory-clinic recruited series of 20 PPA patients (12 nonfluent, five semantic, and three logopenic variants) were studied on neurolinguistic and neuropsychological domains relative to 64 cognitively intact healthy older control subjects. The neuroimaging battery included high-resolution volumetric magnetic resonance imaging processed with voxel-based morphometry, and positron emission tomography with the tau-tracer [18F]-THK5351 and amyloid-tracer [11C]-Pittsburgh Compound B.ResultsSeven out of 12 subjects who had been classified a priori with nonfluent variant PPA showed deficits on conventional single-word comprehension tasks along with speech apraxia and agrammatism, corresponding to a mixed variant phenotype. These mixed variant cases included three females and four males, with a mean age at onset of 65 years (range 44-77 years). Object knowledge and object recognition were additionally affected, although less severely compared with the semantic variant. The mixed variant was characterized by a distributed atrophy pattern in frontal and temporoparietal regions. A more focal pattern of elevated [18F]-THK5351 binding was present in the supplementary motor area, the left premotor cortex, midbrain, and basal ganglia. This pattern was closely similar to that seen in pure nonfluent variant PPA. At the individual patient level, elevated [18F]-THK5351 binding in the supplementary motor area and premotor cortex was present in six out of seven mixed variant cases and in five and four of these cases, respectively, in the thalamus and midbrain. Amyloid biomarker positivity was present in two out of seven mixed variant cases, compared with none of the five pure nonfluent cases.ConclusionsA substantial proportion of PPA patients with speech apraxia and agrammatism also have single-word comprehension deficits. At the neurobiological level, the mixed variant shows a high degree of similarity with the pure nonfluent variant of PPA.Trial registrationEudraCT, 2014-002976-10 . Registered on 13-01-2015.

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

Prominent auditory deficits in primary progressive aphasia: A case study.

Publications

Prominent auditory deficits in primary progressive aphasia: A case study.

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