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From the University of California, San Diego (Drs. Rascovsky, Salmon, and Galasko); The University of Texas Southwestern Medical Center (Dr. Lipton), Dallas; University of Washington (Dr. Leverenz), Seattle; University of California, Davis (Dr. DeCarli); University of California, Berkeley (Dr. Jagust); University of Pennsylvania (Dr. Clark), Philadelphia; University of California, Los Angeles (Dr. Mendez); Mayo Clinic (Drs. Tang-Wai and Graff-Radford), Jacksonville, FL.
Address correspondence and reprint requests to Dr. David Salmon, Alzheimer's Disease Research Center, University of California, San Diego, Mail Code 0948, 9500 Gilman Drive, La Jolla, CA 92093-0948; e-mail: dsalmon{at}ucsd.edu
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.
This study was supported with funding from the National Alzheimer's Coordinating Center and NIA grants P30 AG10129, P30 AG-12300, P50 AG16574, P50 AG10124, P50 AG05131 and P50 AG05136.
Disclosure: The authors report no conflicts of interest.
Received November 3, 2004. Accepted in final form April 18, 2005.
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