Survival in the Three Common Variants of Primary Progressive Aphasia: A Retrospective Study in a Tertiary Memory Clinic

Tastevin, Maud; Lavoie, Monica; Sablonnière, Justine De La; Carrier-Auclair, Julie; Laforce, Robert

doi:10.3390/brainsci11091113

Cited by 18 publications

(14 citation statements)

References 26 publications

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“…As to the median survival of PPA-MND patients – i.e., ≈3 years –, it was slightly lower than that of patients with PPA only ( Montembeault et al, 2018 ), this agreeing with the fact that MN involvement represents a risk factor for a shorter survival in PPA patients ( De La Sablonnière et al, 2021 ; El-Wahsh et al, 2021 ). Its high heterogeneity herewith found might be accounted for by the diversities across both PPA and MND phenotypes – since survival is longer in SD than in PNFA ( Tastevin et al, 2021 ), and shorter in classical ALS as compared to atypical MND variants – such as predominant-UMN phenotypes ( Turner and Talbot, 2020 ), which were relatively highly represented (25.5%) among PPA-MND patients.…”

Section: Discussionmentioning

confidence: 85%

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: Discussionmentioning

confidence: 85%

Primary progressive aphasia and motor neuron disease: A review

Aiello

Feroldi

Luca

et al. 2022

Front. Aging Neurosci.

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“…In this special issue, our group has provided recent insights into survival in the three PPAs. Indeed, significant differences in survival were found with svPPA showing the longest and nfvPPA showing more neurologically-related causes of death [70].…”

Section: Survival Datamentioning

confidence: 99%

Longitudinal Changes in Cognition, Behaviours, and Functional Abilities in the Three Main Variants of Primary Progressive Aphasia: A Literature Review

et al. 2021

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Primary progressive aphasias (PPAs) are a group of neurodegenerative diseases presenting with insidious and relentless language impairment. Three main PPA variants have been described: the non-fluent/agrammatic variant (nfvPPA), the semantic variant (svPPA), and the logopenic variant (lvPPA). At the time of diagnosis, patients and their families’ main question pertains to prognosis and evolution, but very few data exist to support clinicians’ claims. The objective of this study was to review the current literature on the longitudinal changes in cognition, behaviours, and functional abilities in the three main PPA variants. A comprehensive review was undertaken via a search on PUBMED and EMBASE. Two authors independently reviewed a total of 65 full-text records for eligibility. A total of 14 group studies and one meta-analysis were included. Among these, eight studies included all three PPA variants. Eight studies were prospective, and the follow-up duration was between one and five years. Overall, svPPA patients showed more behavioural disturbances both at baseline and over the course of the disease. Patients with lvPPA showed a worse cognitive decline, especially in episodic memory, and faster progression to dementia. Finally, patients with nfvPPA showed the most significant losses in language production and functional abilities. Data regarding the prodromal and last stages of PPA are still missing and studies with a longer follow-up observation period are needed.

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“…Notably, sv-PPA patients attended more clinical visits and sv-PPA carers completed more CBI-R questionnaires than their PPA variant counterparts. Although the varying rates of attrition may reflect each variant's trajectory to incapacity and death (Tastevin et al, 2021), we acknowledge that these high and varying rates of nonparticipation at follow up visit (and completion of behavioural measures) likely contributed to the data modelling. Accordingly, more research is needed using larger groups with higher participation rates to verify our findings.…”

Section: Discussionmentioning

confidence: 99%

Longitudinal changes in behaviour, mood and functional capacity in the primary progressive aphasia variants

Foxe

Irish

Ramanan

et al. 2021

Eur J of Neuroscience

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Primary progressive aphasia (PPA) is a neurodegenerative clinical syndrome characterised by a progressive decline in speech and language functions. Deficits in behaviour, mood and functional capacity are reported in PPA but are less well understood. This study examined the PPA variants' profiles on these domains at initial presentation and over time and evaluated their relations to overall cognitive ability. Behaviour, mood and functional capacity were measured annually (over $6 years) in 145 individuals diagnosed with PPA (41 logopenic [lv-PPA], 44 non-fluent [nfv-PPA] and 60 semantic variants [sv-PPA]) using the Cambridge Behavioural Inventory-Revised (CBI-R) carer questionnaire. Overall cognition was assessed annually with the Addenbrooke's Cognitive Examination-III. Distinct profiles were observed across PPA syndromes. Notably, sv-PPA carers reported greater behavioural,eating and motivational disturbances than the other PPA variants throughout the disease course. Reported memory problems were also greater in sv-PPA and lv-PPA than in nfv-PPA across all time points. These disturbances occurred in the context of the sv-PPA group demonstrating a slower rate of cognitive decline than the lv-PPA group and a parallel rate to that found in the nfv-PPA group. Associations between overall cognition and the CBI-R domains were trivial at baseline assessment; however, distinct profiles emerged when mapping each syndrome's overall cognitive decline with their behavioural, mood and functional trajectories. Our findings demonstrate that the evolving behaviour, mood and functional capacity profiles of the PPA variants are distinct and extend beyond the primary disorder of language. These findings have important implications for clinical management and caregiver education in PPA.

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Survival in the Three Common Variants of Primary Progressive Aphasia: A Retrospective Study in a Tertiary Memory Clinic

Cited by 18 publications

References 26 publications

Primary progressive aphasia and motor neuron disease: A review

Primary progressive aphasia and motor neuron disease: A review

Longitudinal Changes in Cognition, Behaviours, and Functional Abilities in the Three Main Variants of Primary Progressive Aphasia: A Literature Review

Longitudinal changes in behaviour, mood and functional capacity in the primary progressive aphasia variants

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