Survival in progressive supranuclear palsy and frontotemporal dementia.
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.
Chiu
Wang Zheng
WZ
Department of Neurology, Erasmus University Medical Centre Rotterdam, Room Hs 611, 's-Gravendijkwal 230, 3015 CE Rotterdam, The Netherlands.
Kaat
L D
LD
Seelaar
Harro
H
Rosso
Sonia M
SM
Boon
Agnita Jw
AJ
Kamphorst
Wouter
W
van Swieten
John C
JC
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
C054369:MAPT protein, human; D016875:tau Proteins