Abstract
Knowledge on the natural history of the three main variants of primary progressive aphasia (PPA) is lacking, particularly regarding mortality. Moreover, advanced stages and end of life issues are rarely discussed with caregivers and families at diagnosis, which can cause more psychological distress. We analyzed data from 83 deceased patients with a diagnosis of PPA. We studied survival in patients with a diagnosis of logopenic variant (lvPPA), semantic variant (svPPA), or non-fluent variant (nfvPPA) and examined causes of death. From medical records, we retrospectively collected data for each patient at several time points spanning five years before the first visit to death. When possible, interviews were performed with proxies of patients to complete missing data. Results showed that survival from symptom onset and diagnosis was significantly longer in svPPA than in lvPPA (p = 0.002) and nfvPPA (p < 0.001). No relevant confounders were associated with survival. Mean survival from symptom onset was 7.6 years for lvPPA, 7.1 years for nfvPPA, and 12 years for svPPA. The most common causes of death were natural cardio-pulmonary arrest and pneumonia. Aspiration pneumonia represented 23% of deaths in nfvPPA. In conclusion, this pilot study found significant differences in survival between the three variants of PPA with svPPA showing the longest and nfvPPA showing more neurologically-related causes of death.
Highlights
Primary progressive aphasias (PPAs) are a group of neurodegenerative diseases characterized by a predominant and progressive deterioration of language, with relative preservation of other cognitive functions over at least two years after the onset of the disease [1].Since 2011, PPAs have been classified into three variants based on their clinical manifestations: the semantic variant, the non-fluent/agrammatic variant, and the logopenic variant [2].Demographic and epidemiological data regarding PPAs are lacking and most estimations are based on Frontotemporal Lobar Degeneration (FTLD) studies
While nfvPPA and svPPA are commonly considered as clinical presentations of FTLD with a predominant FTLD-tau pathology for nfvPPA (64% of cases) and FTLDTDP-43 for svPPA (80% of cases), it is estimated that 86% of lvPPA are associated with
Eighty-three patients were included in the analyses: 35 lvPPA, 18 svPPA, and 30 nfvPPA
Summary
Since 2011, PPAs have been classified into three variants based on their clinical manifestations: the semantic variant (svPPA), the non-fluent/agrammatic variant (nfvPPA), and the logopenic variant (lvPPA) [2]. Demographic and epidemiological data regarding PPAs are lacking and most estimations are based on Frontotemporal Lobar Degeneration (FTLD) studies. PPAs represent 20–40% of FTLD cases [3] with an estimated prevalence between 3.6 and 8.1/100,000 inhabitants [4,5,6]. A recent study suggested a prevalence of 3.1/100,000 (95% confidence interval [2.96–3.23]) from a French database including 2035 PPAs patients followed in tertiary centers [7]. While nfvPPA and svPPA are commonly considered as clinical presentations of FTLD with a predominant FTLD-tau pathology for nfvPPA (64% of cases) and FTLDTDP-43 for svPPA (80% of cases), it is estimated that 86% of lvPPA are associated with
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