Back to Search
Start Over
Establishing short-term prognosis in Frontotemporal Lobar Degeneration spectrum: role of genetic background and clinical phenotype
- Source :
- Neurobiology of aging. 31(2)
- Publication Year :
- 2007
-
Abstract
- Background: Establishing the short-term prognosis in Frontotemporal Lobar Degeneration (FTLD) is a clinical challenge for defining disease outcomes and monitoring therapeutic interventions. No reliable neuropsychological assessment balancing all FTLD aspects is available yet, thus no clear-cut follow-up study has been performed. Objective: To evaluate the rate of progression and the predictors of worsening in FTLD patients. Methods: One-hundred twenty-seven FTLD patients entered the study and were re-evaluated at 1-year follow-up. A statistical driven approach on wide neuropsychological, behavioral, and functional data was applied to identify homogeneous groups both at baseline and at follow-up within FTLD. Three set of predictors on disease progression were considered: (i) the demographic characteristics, (ii) the genetic background, i.e. Apolipoprotein E (APOE) genotype, Tau haplotype, and functional polymorphisms affecting serotonin and dopamine pathways, and (iii) the clinical phenotype. Results: Among FTLD, two groups of patients were recognized on the basis of the overall assessment, thus termed for different disease severity as "good performers" and "bad performers". At 1-year follow-up, almost 30% of FTLD patients progressed from "good" to "bad" performances, whilst 70% maintained stable "good" performances. APOE ε4 allele, Tau H2 haplotype and behavioral variant FTD phenotype were associated with worse prognosis over time. Conclusions: This preliminary study proposed genetic and clinical predictors in FTLD progression. The identification of disease-modifying predictors of prognosis opens a new avenue in studying FTLD, and may contribute to define outcomes and to monitor pharmacological targets. © 2008 Elsevier Inc. All rights reserved.
- Subjects :
- Apolipoprotein E
Oncology
Male
Aging
Pathology
Time Factors
Apolipoprotein E4
Neuropsychological Tests
Risk Factors
Genotype
Neuropsychological assessment
Serotonin Plasma Membrane Transport Proteins
biology
medicine.diagnostic_test
General Neuroscience
Neuropsychology
Frontotemporal lobar degeneration
Prognosis
Phenotype
Disease Progression
Neuropsychological Test
Female
Psychology
Serotonin Plasma Membrane Transport Protein
Human
medicine.medical_specialty
Time Factor
Prognosi
Tau protein
tau Proteins
Catechol O-Methyltransferase
Follow-Up Studie
Internal medicine
mental disorders
medicine
Humans
Genetic Predisposition to Disease
Allele
Aged
Risk Factor
tau Protein
Haplotype
nutritional and metabolic diseases
medicine.disease
nervous system diseases
biology.protein
Neurology (clinical)
Geriatrics and Gerontology
Frontotemporal Lobar Degeneration
Developmental Biology
Follow-Up Studies
Subjects
Details
- ISSN :
- 15581497
- Volume :
- 31
- Issue :
- 2
- Database :
- OpenAIRE
- Journal :
- Neurobiology of aging
- Accession number :
- edsair.doi.dedup.....00521375f812416bff603654f647ab64