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Abstract Details

Systematic Review of Illness Duration and Life Expectancy in Frontotemporal Degeneration
Aging and Dementia
P05 - (-)
107
BACKGROUND: Life expectancy is incompletely characterized in FTD, a pathologically heterogeneous disease that causes most primary dementia arising in midlife or earlier.
DESIGN/METHODS: This is a systematic review of literature databases, limited to English-language reports. Studies were grouped by sampling strategy (ante-mortem v post-mortem). Data abstracted include phenotype, pathologic type, age at onset, and mean (and median) illness duration. Comparisons with survival in Alzheimer disease (AD) were analyzed. Summary estimates of survival for FTD phenotypes and AD were computed.
RESULTS: Twenty-six studies (13 ante-mortem, 13 post-mortem) met criteria for inclusion with a total of 1771 subjects and 14010 observed years of illness. Survival in FTD was 8.5 years and in AD 9.4 years. In ante-mortem samples survival in behavioral FTD was 9.1 years and in AD was 9.7 years years. In post mortem samples (the latter comparisons derived from just 3 studies) survival was 8.9 years and 6.0 respectively. Estimated survival was shortest in FTD with motor neuron disease (FTD-MND) 3.2 years, longest in corticobasal degeneration and semantic dementia (10.2 and 11.0 years, respectively), and intermediate in progressive non-fluent aphasia (8.5 years) and progressive supranuclear palsy (7.1 years).
CONCLUSIONS: FTD phenotypes vary in survival. FTD-MND shows short survival, PSP intermediate survival, and other phenotypes longer survival comparable to AD. There is still much to learn, such as factors that shape illness progression and survival in individuals. Future analyses will examine the effects of specific features (such as parkinsonism, anorexia, oral-buccal apraxia) on survival.
Authors/Disclosures
Kelly Sloane, MD
PRESENTER
The institution of Dr. Sloane has received research support from American Heart Association.
Mohamed H. Al Zwahmah, MD, MBBS, FAAN (King Faisal Specialist Hospital and Research Center) Dr. Al Zwahmah has nothing to disclose.
No disclosure on file
No disclosure on file
Edward Huey No disclosure on file
No disclosure on file
Claire Tsao (Abbott) No disclosure on file