Survival in frontotemporal lobar degeneration and related disorders: latent class predictors and brain functional correlates

Rejuvenation Res. 2009 Feb;12(1):33-44. doi: 10.1089/rej.2008.0812.


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.

Publication types

  • Controlled Clinical Trial
  • Research Support, Non-U.S. Gov't
  • Validation Study

MeSH terms

  • Adult
  • Aged
  • Brain / physiopathology*
  • Cognition Disorders / diagnosis
  • Cognition Disorders / mortality*
  • Cysteine / analogs & derivatives
  • Cysteine / pharmacokinetics
  • Dementia / diagnosis
  • Dementia / mortality*
  • Female
  • Follow-Up Studies
  • Humans
  • Male
  • Middle Aged
  • Neuropsychological Tests
  • Organotechnetium Compounds / pharmacokinetics
  • Prognosis
  • Radioactive Tracers
  • Severity of Illness Index*
  • Survival Rate


  • Organotechnetium Compounds
  • Radioactive Tracers
  • technetium Tc 99m bicisate
  • Cysteine