Article Text

other Versions

PDF
Factors associated with survival probability in autopsy-proven frontotemporal lobar degeneration
  1. S X Xie (sxie{at}cceb.med.upenn.edu)
  1. University of Pennsylvania School of Medicine, United States
    1. M S Forman
    1. University of Pennsylvania School of Medicine, United States
      1. J Farmer
      1. University of Pennsylvania School of Medicine, United States
        1. P Moore
        1. University of Pennsylvania School of Medicine, United States
          1. Y Wang
          1. University of Pennsylvania School of Medicine, United States
            1. X Wang
            1. University of Pennsylvania School of Medicine, United States
              1. C M Clark
              1. University of Pennsylvania School of Medicine, United States
                1. H B Coslett
                1. University of Pennsylvania School of Medicine, United States
                  1. A Chatterjee
                  1. University of Pennsylvania School of Medicine, United States
                    1. S E Arnold
                    1. University of Pennsylvania School of Medicine, United States
                      1. H Rosen
                      1. University of California, San Francisco, United States
                        1. J H T Karlawish
                        1. University of Pennsylvania School of Medicine, United States
                          1. V M Van Deerlin
                          1. University of Pennsylvania School of Medicine, United States
                            1. V M-Y Lee
                            1. University of Pennsylvania School of Medicine, United States
                              1. J Q Trojanowski
                              1. University of Pennsylvania School of Medicine, United States
                                1. M Grossman (mgrossma{at}mail.med.upenn.edu)
                                1. University of Pennsylvania School of Medicine, United States

                                  Abstract

                                  Objective: To examine 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 anatomic 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), while 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, while tau-negative pathology is associated with longer survival time when clinical MND is excluded.

                                  • frontotemporal lobar degeneration
                                  • survival
                                  • tau

                                  Statistics from Altmetric.com

                                  Request permissions

                                  If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.