Logopenic Variant Primary Progressive Aphasia

Roytman, Michelle; Chiang, Gloria

doi:10.1007/978-3-030-82367-2_27

Cited by 3 publications

(3 citation statements)

References 19 publications

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“…(1) PPA phenotypes, such an estimate was higher for PNFA (10%) than for SD (3%) -this being explainable according to a corticofugal model of TDP-43-mediated neurodegeneration: indeed, PNFA primarily affects frontal-insular areas contiguous to motor regions, whereas SD mostly involves anterior temporal lobes, which are topographically distant from motor cortices (Braak et al, 2013;Brettschneider et al, 2013). By contrast, the finding of MND/MN dysfunction never cooccurring with lvPPA was in line with the latter being commonly underpinned by a non-FTLD neuropathology (Roytman and Chiang, 2022).…”

Section: Clinical Phenotypesmentioning

confidence: 61%

“…By contrast, the finding of MND/MN dysfunction never co-occurring with lvPPA was in line with the latter being commonly underpinned by a non-FTLD neuropathology ( Roytman and Chiang, 2022 ).…”

Section: Discussionmentioning

confidence: 77%

“…Records were searched for in Scopus and PubMed on August 8th, 2021 through the following string: (“motor neuron disease” OR “amyotrophic lateral sclerosis”) AND (“primary progressive aphasia” OR “progressive aphasia” OR “progressive non-fluent aphasia” OR “progressive non-fluent aphasia” OR “semantic dementia” OR “semantic variant” OR “agrammatic variant” OR “non-fluent variant” OR “non-fluent variant”). The string did not referred to logopenic-variant PPA (lvPPA), as being commonly underpinned by a neuropathology different from frontotemporal lobar degeneration (FTLD), i.e., that of Alzheimer’s disease (AD) ( Roytman and Chiang, 2022 ).…”

Section: Methodsmentioning

confidence: 99%

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Primary progressive aphasia and motor neuron disease: A review

Aiello

Feroldi

Luca

et al. 2022

Front. Aging Neurosci.

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BackgroundThis study aims at reviewing, within the framework of motor neuron disease-frontotemporal degeneration (MND-FTD)-spectrum disorders, evidence on the co-occurrence between primary progressive aphasia (PPA) and MND in order to profile such a complex at pathological, genetic and clinical levels.MethodsThis review was pre-registered (osf.io/ds8m4) and performed in accordance with the 2020 PRISMA guidelines. Case reports/series and group studies were included if addressing (1) progressive non-fluent aphasia (PNFA) or semantic dementia (SD) with MND or (2) MND patients with co-morbid PNFA/SD.ResultsOut of 546 initial records, 56 studies were included. As to case reports/series (N = 35), which included 61 PPA-MND patients, the following findings yielded: (1) PNFA is more frequent than SD in PPA-MND; (2) in PPA-MND, the most prevalent motor phenotypes are amyotrophic lateral sclerosis and predominant-upper MND, with bulbar involvement being ubiquitous; (3) extrapyramidal features are moderately frequent in PPA-MND; (4) PPA-MND patients usually display frontotemporal, left-greater-than-right involvement; (5) TDP-43-B is the typical pathological substrate of PPA-MND; (6) TBK1 mutations represent the most frequent genetic risk factors for PPA-MND.As to group studies, including 121 patients, proportional meta-analytic procedures revealed that: (1) the lifetime prevalence of MND in PPA is 6%; (2) PPA occurs in 19% of patients with co-morbid MND and FTD; (3) MND is more frequent in PNFA (10%) than in SD patients (3%).DiscussionInsights herewith delivered into the clinical, neuropathological and genetic features of PPA-MND patients prompt further investigations aimed at improving clinical practice within the MND-FTD spectrum.

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Section: Clinical Phenotypesmentioning

confidence: 61%

Section: Discussionmentioning

confidence: 77%

Section: Methodsmentioning

confidence: 99%

See 1 more Smart Citation

Primary progressive aphasia and motor neuron disease: A review

Aiello

Feroldi

Luca

et al. 2022

Front. Aging Neurosci.

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Logopenic Variant Primary Progressive Aphasia

Song

2022

The Palgrave Encyclopedia of Critical Perspectives on Mental Health

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Purpose-The aim of this review is to explore the evolution of the logopenic variant of primary progressive aphasia as a distinct clinical entity and to outline recent advances that have clarified its clinical characteristics, neural underpinnings and potential genetic and pathological bases. This is particularly relevant as researchers attempt to identify clinico-pathological relationships in subtypes of primary progressive aphasia in hopes of utilizing language phenotype as a marker of underlying disease.Recent findings-Recent work has served to refine and expand upon the clinical phenotype of the logopenic variant. Logopenic patients show a unique pattern of spared and impaired language processes that reliably distinguish this syndrome from other variants of progressive aphasia. Specifically, they exhibit deficits in naming and repetition in the context of spared semantic, syntactic and motor speech abilities. Further, there is a growing body of evidence indicating a possible link between the logopenic variant phenotype and specific pathological and genetic correlates.Summary-Findings indicate that the logopenic variant is a distinct subtype of progressive aphasia that may hold value as a predictor of underlying pathology. Additional research, however, is warranted in order to further clarify the cognitive-linguistic profile and to confirm its relation to certain pathological and genetic processes.

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Suspected dementia, the eye and the ‘mind’s eye’: How primary care eye specialists could support early identification of Alzheimer’s Disease and its variants

Rowley¹,

Cook²

2022

bpsneur

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The current article outlines the nature of visuoperceptual deficits in Alzheimer’s Disease (AD) and its ‘atypical’ variants. Evidence to highlight that visual and visuoperceptual difficulties are common in dementias driven by Alzheimer’s-based pathology will be discussed. Following this, the ways in which early identification of visual and lower-level visuoperceptual symptoms of dementia may be facilitated within primary care optical health settings will be considered. It is hoped that this may inspire work to support opticians and optometrists to identify where timely signposting to dementia services may be beneficial for people with suspected dementia who are experiencing visuoperceptual deficits. This is particularly important given that visual and visuoperceptual deficits can be early signs of dementia and often have a significant impact on activities of daily living (e.g. dressing, increased falls risk, accidents whilst driving, difficulties navigating familiar environments; Tippett et al., 2019). An illustrative case example is provided, outlining how timely detection of suspected dementia could have enabled early provision of appropriate treatment and support to enhance quality of life (e.g. medication for symptom management, provision of cognitive strategies and functional aids; Armstrong & Kergoat, 2015).

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Logopenic Variant Primary Progressive Aphasia

Cited by 3 publications

References 19 publications

Primary progressive aphasia and motor neuron disease: A review

Primary progressive aphasia and motor neuron disease: A review

Logopenic Variant Primary Progressive Aphasia

Suspected dementia, the eye and the ‘mind’s eye’: How primary care eye specialists could support early identification of Alzheimer’s Disease and its variants

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