Parietal Involvement in the Semantic Variant of Primary Progressive Aphasia with Alzheimer’s Disease Cerebrospinal Fluid Profile

Bera, G.; Migliaccio, Raffaella; Michelin, T.; Lamari, Foudil; Ferrieux, Sophie; Nogues, Marie; Bertín, Hugo Darío; Habert, Marie Odile; Dubois, Bruno; Teichmann, Marc; Kas, Aurélie

doi:10.3233/jad-180087

Cited by 6 publications

(4 citation statements)

References 42 publications

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“…Patients with svPPA and AD pathology demonstrated a more widespread cerebral hypoperfusion than patients with svPPA and TDP43 pathology. Hypoperfusion areas encompass the inferior parietal lobule, posterior cingulate cortex, and precuneus, similar to patients with typical AD ( 47 – 49 ). Brain atrophy extended to the inferior parietal lobule, and increased p-tau levels in the CSF suggested AD pathology in the present case.…”

Section: Discussionmentioning

confidence: 78%

Case Report: Semantic Variant Primary Progressive Aphasia With Impaired Verbal Word Discrimination

et al. 2022

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Some patients with primary progressive aphasia (PPA) present with various types of hearing deficits. Research on the auditory function and speech sounds in PPA, including temporal, phonemic, and prosodic processing, revealed impairment in some of these auditory processes. Many patients with PPA who present with impaired word recognition subsequently developed non-fluent variant PPA. Herein, we present a patient with semantic variant PPA (svPPA) who demonstrated impaired verbal word discrimination. Audiological examinations revealed normal auditory brainstem responses and slightly impaired pure-tone perception. By contrast, verbal word discrimination and monosyllable identification were impaired, and temporal auditory acuity deteriorated. Analyses of brain magnetic resonance images revealed a significant decrease in the gray matter volume in bilateral superior temporal areas, predominantly on the left, compared with those of patients with typical svPPA, which appeared to be associated with impaired word recognition in our patient.

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Section: Discussionmentioning

confidence: 78%

Case Report: Semantic Variant Primary Progressive Aphasia With Impaired Verbal Word Discrimination

et al. 2022

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“…It is also important to further investigate the similarities and differences between bvAD and dAD, which were initially considered a single subtype. Additionally, AD pathology can cause other phenotypes not described in this review, such as motor variant of AD or even svPPA, which have been previously described as clinically indistinguishable from patients with non-AD svPPA but have a parietal involvement that is absent in the others [31]. While progress has been made in diagnosing atypical AD, more longitudinal studies are needed to understand the disease progression.…”

Section: Discussionmentioning

confidence: 86%

Atypical forms of Alzheimer's disease: patients not to forget

Montembeault

Migliaccio

2023

Current Opinion in Neurology

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Purpose of review The aim of this paper is to summarize the latest work on neuroimaging in atypical Alzheimer's disease (AD) patients and to emphasize innovative aspects in the clinic and research. The paper will mostly cover language (logopenic variant of primary progressive aphasia; lvPPA), visual (posterior cortical atrophy; PCA), behavioral (bvAD) and dysexecutive (dAD) variants of AD. Recent findings MRI and PET can detect and differentiate typical and atypical AD variants, and novel imaging markers like brain iron deposition, white matter hyperintensities (WMH), cortical mean diffusivity, and brain total creatine can also contribute. Together, these approaches have helped to characterize variant-specific distinct imaging profiles. Even within each variant, various subtypes that capture the heterogeneity of cases have been revealed. Finally, in-vivo pathology markers have led to significant advances in the atypical AD neuroimaging field. Summary Overall, the recent neuroimaging literature on atypical AD variants contribute to increase knowledge of these lesser-known AD variants and are key to generate atypical variant-specific clinical trial endpoints, which are required for inclusion of these patients in clinical trials assessing treatments. In return, studying these patients can inform the neurobiology of various cognitive functions, such as language, executive, memory, and visuospatial abilities.

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“…In the latest updated version [5], the diagnosis of atypical Alzheimer's disease requires the presence of (1) a clinical phenotype consistent with one of the atypical presentations (visual/posterior variant, logopenic variant of primary progressive aphasia, and frontal variant) and (2) biological, genetic, and/or in vivo molecular imaging signs supporting the diagnosis of Alzheimer's disease. However, more new clinical phenotypes have recently been identified as clinical variants of Alzheimer's disease, including patients with semantic variant of primary progressive aphasia [6] or with corticobasal syndrome [7]. Those new clinical variants add to the taxonomy of Alzheimer's disease, highlight a large variability among patients, and importantly pose major challenges for the diagnosis of early-onset Alzheimer variants [8], emphasizing the importance of in vivo biomarkers for diagnosis.…”

Section: The Complexity Of Early-onset Alzheimer's Diseasementioning

confidence: 99%