Survival in a German Population with Frontotemporal Lobar Degeneration

Nunnemann, Sabine; Schuster, Tibor; Förstl, Hans; Kurz, Alexander; Diehl‐Schmid, Janine

doi:10.1159/000331485

Cited by 38 publications

(33 citation statements)

References 50 publications

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“…Eleven studies were North American [3,7,19,31,32,33,34,35,36,37,38], eleven were Western European [8,10,39,40,41,42,43,44,45,46,47] and four were from Southeast and East Asia [5,48,49,50]. One was multi-continental [4].…”

Section: Resultsmentioning

confidence: 99%

“…References were scoured for more studies fitting the criteria. For two studies [8,10], the authors provided original data. When papers shared samples, the larger studies were selected.…”

Section: Methodsmentioning

confidence: 99%

“…How other phenotypes compare to one another is not clear, as there have been few relevant analyses. Two survival studies suggested that SD has longer median survival than bvFTD [5,7], but direct comparisons of survival curves yielded mixed results [4,8]. Also, we do not know how demographic characteristics, clinical features complicating the illness (e.g., parkinsonism and dysphagia) and comorbid conditions (e.g., heart disease, cancer, diabetes mellitus) shape FTD survival.…”

Section: Introductionmentioning

confidence: 97%

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Survival in Frontotemporal Dementia Phenotypes: A Meta-Analysis

Kansal

Mareddy

Sloane

et al. 2016

Dement Geriatr Cogn Disord

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Background: Survival in frontotemporal dementia (FTD) is not well understood. We conducted a mixed effects meta-analysis of survival in FTD to examine phenotype differences and contributory factors. Methods: The PubMed, Medline, EMBASE, CINAHL, PsycINFO and Cochrane databases were searched for studies describing survival or natural history of behavioral variant FTD (bvFTD), progressive non-fluent aphasia (PNFA), semantic dementia (SD), FTD with amyotrophic lateral sclerosis (FTD-ALS), progressive supranuclear palsy and corticobasal degeneration. There were no language restrictions. Results: We included 27 studies (2,462 subjects). Aggregate mean and median survival were derived for each phenotype and, for comparison, Alzheimer's disease (AD) (using data from the selected studies). Survival was shortest in FTD-ALS (2.5 years). Mean survival was longest in bvFTD and PNFA (8 years) and median survival in SD (12 years). AD was comparable in survival to all except FTD-ALS. Age and sex did not affect survival; the education effect was equivocal. Heterogeneity in FTD survival was largely, but not wholly, explained by phenotypes. Conclusions: Survival differs for FTD phenotypes but, except for FTD-ALS, compares well to AD survival. Elucidating the potential causes of within-phenotype heterogeneity in survival (such as complicating features and comorbidities) may open up opportunities for tailored interventions.

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Section: Resultsmentioning

confidence: 99%

“…References were scoured for more studies fitting the criteria. For two studies [8,10], the authors provided original data. When papers shared samples, the larger studies were selected.…”

Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 97%

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Survival in Frontotemporal Dementia Phenotypes: A Meta-Analysis

Kansal

Mareddy

Sloane

et al. 2016

Dement Geriatr Cogn Disord

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“…The common answer will probably be quite vague, explaining that it differs per person and that dementia is typically known for its insidious onset and slowly progressive course. In general, dementia has a reduced life expectancy [1,2], but survival is highly variable with reported median survival times ranging from 3-13 years after onset or diagnosis [1][2][3][4][5][6]. However, a subgroup deteriorates faster and dies within years or even months [5,7,8].…”

Section: Introductionmentioning

confidence: 99%

Dementia and Rapid Mortality: Who is at Risk?

Staekenborg

Pijnenburg

Lemstra

et al. 2016

JAD

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Abstract.Background: Dementia is typically known for its insidious onset and slowly progressive course, but a subgroup deteriorates fast and dies within years or even months. Objective: The purpose of this study was to characterize dementia patients with a rapidly progressive course to death and evaluate their cause of death. Methods: We retrospectively included all patients from the Amsterdam Dementia Cohort who died within two years after diagnosis. We evaluated the characteristics of these rapid progressors and compared them to patients known to be alive two years after diagnosis ('non-rapid mortality'). Results: We included 129 dementia patients (13% of our total cohort with known follow-up) with rapid mortality (age 72 ± 10 y [29% <65 y], 70[55%]M, MMSE 20 ± 5). Mean(SD) survival was 12 ± 7 months. Compared to non-rapid mortality patients (n = 892; age 68 ± 9, 503(56%)M, MMSE 22 ± 5), patients with rapid mortality were slightly older at time of diagnosis, had lower MMSE scores, more depressive symptoms and higher prevalence of a cardiovascular history (all p < 0.05). Alzheimer's disease (AD, 43%) was most frequent in patients with rapid mortality, but the occurrence was much lower compared to non-rapid mortality patients (71%), while all other dementia diagnoses, especially Creutzfeldt-Jakob disease (CJD), vascular dementia (VaD), and frontotemporal dementia (FTD), were more frequent (p < 0.001). There were no specific characteristics for AD patients with rapid versus non-rapid mortality, especially APOE genotypes and CSF-profiles were comparable (p > 0.70). Cause of death was highly variable without a clear relation to dementia diagnosis, with exception of dementia itself in CJD, intracerebral hematoma in VaD, and motor neuron disease in FTD. Conclusions: Short survival is relatively common (∼13% in our cohort) and occurs in all different types of dementia, with overrepresentation of non-AD dementias like CJD, VaD, and FTD.

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“…7,8 FTD is typically caused by a spectrum of pathologies known as frontotemporal lobar degeneration (FTLD), most commonly including FTLD-tau and FTLD-TDP. 9 Survival in pathologically confirmed FTLD ranges from 72 months to 126 months from onset to death, [10][11][12][13] but little is known about factors that contribute to this large range. The possible role of CR in FTLD survival has been investigated in a small number of studies, but these are restricted to neuroimaging studies in patients without autopsy confirmation.…”

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confidence: 99%

Occupational attainment influences survival in autopsy-confirmed frontotemporal degeneration

et al. 2015

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Objective: To examine the influence of occupational attainment and education on survival in autopsyconfirmed cases of frontotemporal lobar degeneration (FTLD) and Alzheimer disease (AD).Methods: We performed a retrospective chart review of 83 demographically matched, autopsyconfirmed FTLD (n 5 34) and AD (n 5 49) cases. Each patient's primary occupation was classified and ranked. Level of education was recorded in years. Survival was defined as time from symptom onset until death. Linear regression was used to test for associations among occupational attainment, education, and patient survival.Results: Median survival was 81 months for FTLD and 95 months for AD. Years of education and occupational attainment were similar for both groups. We found that higher occupational attainment was associated with longer survival in FTLD but not AD. Cognitive reserve (CR) posits that a person develops cognitive strategies and neural connections over the course of a lifetime through experiences such as education, occupation, and mental engagement. Conclusions:

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Survival in a German Population with Frontotemporal Lobar Degeneration

Cited by 38 publications

References 50 publications

Survival in Frontotemporal Dementia Phenotypes: A Meta-Analysis

Survival in Frontotemporal Dementia Phenotypes: A Meta-Analysis

Dementia and Rapid Mortality: Who is at Risk?

Occupational attainment influences survival in autopsy-confirmed frontotemporal degeneration

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