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From the Memory and Aging Center and Department of Neurology (Drs. Roberson, Johnson, Yaffe, Kramer, and B.L. Miller, J.H. Hesse, K.D. Rose, and H. Slama), Gladstone Institute of Neurological Disease (Dr. Roberson), and Department of Psychiatry and Epidemiology (Dr. Yaffe), University of California, San Francisco; Center for Neurodegenerative Disease Research (Drs. Forman and Trojanowski), Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia; and Department of Pathology (Dr. C.A. Miller), Keck School of Medicine, University of Southern California, Los Angeles.
Address correspondence and reprint requests to Dr Roberson, Gladstone Institute of Neurological Disease, 1650 Owens St., San Francisco, CA 94158; e-mail: eroberson{at}gladstone.ucsf.edu
Background: Frontotemporal lobar degeneration (FTLD) is a common cause of non-Alzheimer dementia, but its natural history and the factors related to mortality in affected patients are not well understood.
Methods: This retrospective, longitudinal study compared survival in FTLD (n = 177) with Alzheimer disease (AD; n = 395). Hazards analysis investigated the contribution of various demographic, neuropsychiatric, and neuropsychological variables and associated neurologic and neuropathologic findings.
Results: The frontotemporal dementia (FTD) subtype of FTLD progressed faster than AD (median survival from retrospectively determined symptom onset, 8.7 ± 1.2 vs 11.8 ± 0.6 years, p < 0.0001; median survival from initial clinic presentation, 3.0 ± 0.5 vs 5.7 ± 0.1 years, p < 0.0001). Survival was similarly reduced in the related conditions corticobasal degeneration and progressive supranuclear palsy. Survival in the semantic dementia subtype of FTLD (11.9 ± 0.2 years from onset and 5.3 ± 0.4 years from presentation), however, was significantly longer than in FTD and did not differ from AD. Hazards analysis to determine factors affecting survival in FTLD showed no effect of age at onset, sex, education, family history, or neuropsychiatric profile. Among neuropsychological measures examined, impaired letter fluency had a significant association with reduced survival. Associated ALS significantly reduced survival in FTLD. The presence of tau-positive inclusions was associated with the slowest progression.
Conclusions: Frontotemporal lobar degeneration progresses more rapidly than Alzheimer disease, and the fastest-progressing cases are those with the frontotemporal dementia clinical subtype, coexisting motor neuron disease, or tau-negative neuropathology.
Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the September 13 issue to find the title link for this article.
Supported by California State Grant 15945, the DHS/ADP California Research Consortium for the Study of Frontotemporal Dementia, NIH PPG AG19724, NIH ADRCs P50 AG16573 (UCSF) and P50 AG05142 (USC), NIH K08 AG20073 (M.S.F), NIH AG20073 (J.Q.T.), and the Giannini Family Foundation (E.D.R.).
The authors report no conflicts of interest.
Received December 12, 2004. Accepted in final form May 17, 2005.
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