title: Survival in progressive supranuclear palsy and frontotemporal dementia.
abstract: OBJECTIVE
  To compare survival and to identify prognostic predictors for progressive supranuclear palsy and frontotemporal dementia.
  
  
  BACKGROUND
  Progressive supranuclear palsy (PSP) and frontotemporal dementia (FTD) are related disorders. Homozygosity for H1 haplotype is associated with PSP, whereas several MAPT mutations have been identified in FTLD-tau. Survival duration probably reflects underlying pathophysiology or disease.
  
  
  METHODS
  Patients with PSP and FTD were recruited by nationwide referral. Survival of 354 FTD patients was compared with that of 197 PSP patients. Cox regression analysis was performed to identify prognostic predictors. FTLD-tau was defined as Pick disease and FTDP-17 with MAPT mutations. Semiquantitative evaluation of tau-positive pathology was performed on all pathologically proven cases.
  
  
  RESULTS
  The median survival of PSP patients (8.0 years; 95% CI 7.3 to 8.7) was significantly shorter than that of FTD patients (9.9 years; 95% CI 9.2 to 10.6). Corrected for demographic differences, PSP patients were still significantly more at risk of dying than FTD patients. In PSP, male gender, older onset-age and higher PSP Rating Scale score were identified as independent predictors for shorter survival, whereas in FTD a positive family history and an older onset-age were associated with a poor prognosis. The difference in hazard rate was even more pronounced when comparing pathologically proven cases of PSP with FTLD-tau.
  
  
  CONCLUSIONS
  Survival of PSP patients is shorter than that of FTD patients, and probably reflects a more aggressive disease process in PSP. Independent predictors of shorter survival in PSP were male gender, older onset-age and higher PSP rating scale score, whereas in FTD a positive family history and higher onset-age were predictors for worse prognosis.
authors.affiliation: Department of Neurology, Erasmus University Medical Centre Rotterdam, Room Hs 611, 's-Gravendijkwal 230, 3015 CE Rotterdam, The Netherlands.
authors.forename: Wang Zheng
authors.initials: WZ
authors.lastname: Chiu
authors.forename: L D
authors.initials: LD
authors.lastname: Kaat
authors.forename: Harro
authors.initials: H
authors.lastname: Seelaar
authors.forename: Sonia M
authors.initials: SM
authors.lastname: Rosso
authors.forename: Agnita Jw
authors.initials: AJ
authors.lastname: Boon
authors.forename: Wouter
authors.initials: W
authors.lastname: Kamphorst
authors.forename: John C
authors.initials: JC
authors.lastname: van Swieten
mesh_terms: D017668:Age of Onset; D000368:Aged; D001921:Brain / Q000098:blood supply / Q000473:pathology; D003072:Cognition Disorders / Q000175:diagnosis / Q000453:epidemiology; D005260:Female; D057180:Frontotemporal Dementia / Q000175:diagnosis / Q000235:genetics / Q000401:mortality*; D005838:Genotype; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009483:Neuropsychological Tests; D011379:Prognosis; D011446:Prospective Studies; D013494:Supranuclear Palsy, Progressive / Q000175:diagnosis / Q000235:genetics / Q000401:mortality*; D015996:Survival Rate; D013997:Time Factors; D015899:Tomography, Emission-Computed, Single-Photon; D016875:tau Proteins / Q000235:genetics
chemical_list: C054369:MAPT protein, human; D016875:tau Proteins