Research Article
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Published Online: 10 March 2009

Survival in Frontotemporal Lobar Degeneration and Related Disorders: Latent Class Predictors and Brain Functional Correlates

Publication: Rejuvenation Research
Volume 12, Issue Number 1

Abstract

Background: Establishing survival rate in frontotemporal lobar degeneration (FTLD) is a clinical challenge for defining disease outcomes and monitoring therapeutic interventions. Using the latent profile analysis (LPA) approach, we have previously suggested that FTLD patients can be grouped into specific phenotypes— “pseudomanic behavior” (LC1), “cognitive” (LC2), and “pseudodepressed behavior” (LC3)—on the basis of neuropsychological, functional, and behavioral data.
Objective: The aim of this study was to evaluate the rate of survival in FTLD, to identify predictors of survival, and to determine the likely usefulness of LPA in defining prognosis.
Methods: A total of 252 FTLD patients entered the study. A clinical evaluation and standardized assessment were carried out, as well as a brain imaging study. LPA on neuropsychological, functional, and behavioral data was performed. Each patient was followed up over a 5-year period, and institutionalization or death was considered.
Results: The survival rate was associated neither with demographic characteristics, co-morbidities, family history for dementia, nor clinical diagnosis. The presence of the three LC phenotypes was confirmed by LPA. A different survival rate was predicted by LCs, the worse prognosis being found in LC1 (hazard ratio [HR] = 15.7, 95% confidence interval [CI] = 7.2–34.9, p < 0.001, reference LC3). LC2 had a worse prognosis compared to LC3 (HR = 2.07, 95% CI = 0.98–4.37, p = 0.06). Greater hypoperfusion in the orbitomesial frontal cortex was specifically associated with LC1 compared with the other LCs.
Conclusions: A data-driven approach regarding neuropsychological and behavioral assessment might be useful in clinical practice for defining a FTLD prognosis and hopefully will lead to the possibility of identifying patient groups for the evaluation of treatment response in future trials.

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cover image Rejuvenation Research
Rejuvenation Research
Volume 12Issue Number 1February 2009
Pages: 33 - 44
PubMed: 19236162

History

Published online: 10 March 2009
Published ahead of print: 23 February 2009
Published in print: February 2009

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B. Borroni
Center for Aging Brain and Dementia, Department of Neurology, University of Brescia, Brescia, Italy.
M. Grassi
Department of Health Sciences, Section of Medical Statistics & Epidemiology, University of Pavia, Pavia, Italy.
C. Agosti
Center for Aging Brain and Dementia, Department of Neurology, University of Brescia, Brescia, Italy.
E. Premi
Center for Aging Brain and Dementia, Department of Neurology, University of Brescia, Brescia, Italy.
A. Alberici
Center for Aging Brain and Dementia, Department of Neurology, University of Brescia, Brescia, Italy.
B. Paghera
Nuclear Medicine Unit, Brescia Hospital, Brescia, Italy.
S. Lucchini
Nuclear Medicine Unit, Brescia Hospital, Brescia, Italy.
M. Di Luca
Centre of Excellence for Neurodegenerative Disorders and Department of Pharmacological Sciences, University of Milan, Milan, Italy.
D. Perani
Vita-Salute San Raffaele University, IRCCS San Raffaele, National Institute of Neuroscience (INN) and IBFM-CNR, Milan, Italy.
A. Padovani
Center for Aging Brain and Dementia, Department of Neurology, University of Brescia, Brescia, Italy.

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