In contrast to the 2010 Dutch study that says those with PSP progress faster than those with FTD, this UCSF study shows that they are about the same.
There’s not much in the UCSF article about PSP and CBD. The article’s focus is on frontotemporal lobar degeneration (FTLD), which is an umbrella term for three separate clinical syndromes — FTD (frontotemporal dementia), semantic dementia (SD), and progressive aphasia (PA). The authors compare the progression and survival time of the three FTD subtypes with AD and CBD/PSP. (Because of the smaller number of CBD and PSP participants, they were combined into one group.) According to this study, FTD progresses faster than AD and about the same as CBD/PSP.
Most interesting to me was the Discussion section of the article. The authors suggest that those disorders where frontal and subcortical areas are affected — FTD and CBD/PSP — have shorter survival than those disorders where the temporal lobe is more affected — AD and SD. “Frontal lobe disease might hasten mortality more than temporal degeneration either because its attendant apathy contributes to the akinetic–mute state that is a common final pathway to death in neurodegenerative disease or because behavioral problems associated with frontal lobe disease lead to overmedication or other differences in the quality of medical care delivered.”
Finally, the authors’ statements with regard to FTD certainly about to PSP and CBD: An “accurate understanding of the natural history of FTLD is important for patient care and counseling about prognosis.” And, “this research has implications for future clinical studies; for example, longitudinal therapeutic trials must consider the more rapid course of FTD, possibly following patients at shorter intervals.”
I’ve copied the abstract below.
Robin
Neurology 2005;65:719-725
Frontotemporal dementia progresses to death faster than Alzheimer disease
E. D. Roberson, MD, PhD, J. H. Hesse, BA, K. D. Rose, BA, H. Slama, BA, J. K. Johnson, PhD, K. Yaffe, MD, M. S. Forman, MD, PhD, C. A. Miller, MD, J. Q. Trojanowski, MD, PhD, J. H. Kramer, PsyD and B. L. Miller, MD
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.
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.
PubMed ID#: 16157905 (see pubmed.gov for this abstract)