title: Rate of progression differs in frontotemporal dementia and Alzheimer disease.
abstract: OBJECTIVE
  To compare survival and rates of cognitive and functional decline in patients with autopsy-confirmed frontotemporal dementia (FTD) and Alzheimer disease (AD) in a large multicenter study.
  
  
  BACKGROUND
  Despite advances in the clinical characterization of FTD, little is known about its rate of progression. Characterizing survival and rate of decline in FTD is important because it can provide prognostic guidelines and benchmarks to use in the evaluation of disease-modifying drugs.
  
  
  METHODS
  Seventy patients with FTD and 70 patients with AD who were followed by seven Alzheimer disease research centers until confirmation of diagnosis at autopsy were matched for overall age, education, and Mini-Mental State Examination (MMSE) score at initial evaluation. Survival and rates of cognitive and functional decline were compared.
  
  
  RESULTS
  Patients with FTD had significantly shorter survival from initial evaluation to death than patients with AD (FTD = 4.2 years, AD = 6.0 years; log-rank test = 5.17, p < 0.05), and they declined significantly faster over one year on the MMSE (mean annual rate of change: -6.7 points for FTD vs -2.3 points for AD). A significantly greater proportion of patients with FTD were impaired in basic activities of daily living (ADLs) at initial evaluation, and lost the capacity for independent or minimally-assisted ADLs over the subsequent year.
  
  
  CONCLUSIONS
  The results are consistent with shorter survival and faster rates of cognitive and functional decline in patients with frontotemporal dementia (FTD) compared to those with Alzheimer disease (AD). This suggests that FTD follows a more malignant disease course than AD once dementia is clinically recognized.
authors.affiliation: University of California, San Diego, La Jolla, CA 92093-0948, USA.
authors.forename: K
authors.initials: K
authors.lastname: Rascovsky
authors.forename: D P
authors.initials: DP
authors.lastname: Salmon
authors.forename: A M
authors.initials: AM
authors.lastname: Lipton
authors.forename: J B
authors.initials: JB
authors.lastname: Leverenz
authors.forename: C
authors.initials: C
authors.lastname: DeCarli
authors.forename: W J
authors.initials: WJ
authors.lastname: Jagust
authors.forename: C M
authors.initials: CM
authors.lastname: Clark
authors.forename: M F
authors.initials: MF
authors.lastname: Mendez
authors.forename: D F
authors.initials: DF
authors.lastname: Tang-Wai
authors.forename: N R
authors.initials: NR
authors.lastname: Graff-Radford
authors.forename: D
authors.initials: D
authors.lastname: Galasko
mesh_terms: D000203:Activities of Daily Living; D000328:Adult; D000367:Age Factors; D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D000544:Alzheimer Disease / Q000473:pathology / Q000503:physiopathology* / Q000523:psychology; D003072:Cognition Disorders / Q000473:pathology / Q000503:physiopathology* / Q000523:psychology; D003704:Dementia / Q000473:pathology / Q000503:physiopathology* / Q000523:psychology; D018450:Disease Progression; D004522:Educational Status; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009483:Neuropsychological Tests; D011446:Prospective Studies; D017678:Sex Distribution; D015996:Survival Rate / Q000639:trends