Factors associated with survival probability in autopsy-proven frontotemporal lobar degeneration
Autor: | Jason Karlawish, H. B. Coslett, Christopher M. Clark, Peachie Moore, Vivianna M. Van Deerlin, Jennifer M. Farmer, Steven E. Arnold, Yong Dong Wang, Howard J. Rosen, Xuran Wang, V M-Y Lee, Anjan Chatterjee, Mark S. Forman, Sharon X. Xie, John Q. Trojanowski, Murray Grossman |
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Rok vydání: | 2007 |
Předmět: |
Adult
Male Pathology medicine.medical_specialty Autopsy Kaplan-Meier Estimate Basal Ganglia Cohort Studies Diagnosis Differential Alzheimer Disease Risk Factors Internal medicine mental disorders medicine Dementia Humans Genetic Predisposition to Disease Survival rate Aged Proportional Hazards Models Aged 80 and over Proportional hazards model Hazard ratio Brain Frontotemporal lobar degeneration Middle Aged medicine.disease Temporal Lobe Frontal Lobe Survival Rate Psychiatry and Mental health Tauopathies Cohort Disease Progression Educational Status Surgery Female Neurology (clinical) Psychology Cohort study |
Zdroj: | Journal of neurology, neurosurgery, and psychiatry. 79(2) |
ISSN: | 1468-330X |
Popis: | Objective: To examine the clinical and pathological factors associated with survival in autopsy-confirmed frontotemporal lobar degeneration (FTLD). Methods: The final analysis cohort included 71 patients with pathologically proven FTLD, excluding patients with clinical motor neuron disease (MND), evaluated at the University of Pennsylvania or at the University of California, San Francisco. We assessed clinical and demographic features; cognitive functioning at presentation; genetic markers of disease; and graded anatomical distribution of tau, ubiquitin and amyloid pathology. Results: The tau-negative group (n = 35) had a median survival time of 96 months (95% CI: 72–114 months), whereas the tau-positive group (n = 36) had a median survival time of 72 months (95% CI: 60–84 months). Patients with tau-positive pathology across all brain regions had shorter survival than those with tau-negative pathology in univariate Cox regression analyses (Hazard ratio of dying = 2.003, 95% CI = 1.209–3.318, p = 0.007). Conclusions: Tau-positive pathology represents a significant risk to survival in FTLD, whereas tau-negative pathology is associated with a longer survival time when clinical MND is excluded. |
Databáze: | OpenAIRE |
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