Survival in Frontotemporal Dementia Phenotypes: A Meta-Analysis.

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.

Survival in a German population with frontotemporal lobar degeneration.

BACKGROUND: The present study aimed at analysing survival of patients with behavioural-variant frontotemporal dementia (bvFTD), semantic dementia (SD) and progressive non-fluent aphasia (PNFA). Furthermore, the objective of the study was to identify prognostic factors associated with survival and to examine causes of death. METHODS: Interviews were performed with the proxies of 124 patients with frontotemporal lobar degeneration (FTLD). RESULTS: Survival from the onset of first symptoms was significantly longer in SD than in bvFTD (10.5 years). Median survival in PNFA was 12.6 years. Age at onset, gender, education and severity of dementia at diagnosis did not significantly influence survival. We did not identify any phenocopy cases. The most frequent cause of death as reported by caregivers was respiratory system disorder. CONCLUSION: This study adds to the growing literature on survival in patients with FTLD and provides insights into the causes of death.