Survival in progressive supranuclear palsy and frontotemporal dementia
Résumé
Abstract Objective: To compare survival and to identify prognostic predictors for progressive supranuclear palsy and frontotemporal dementia. Background: PSP and 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 nation-wide referral. Survival of 354 FTD patients was compared to that of 197 PSP patients. Cox regression analysis was performed to identify prognostic predictors. FTLD-tau was defined as Pick's disease and FTDP-17 with MAPT mutations. Semiquantitative evaluation of tau-positive pathology was performed on all pathologically proven cases. Results: Median survival of PSP patients (8.0 years; 95% CI 7.3 to 8.7) was significantly shorter than 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. Conclusion: Survival of PSP patients is shorter than 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.
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