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Survival in progressive supranuclear palsy and frontotemporal dementia
  1. Wang Zheng Chiu1,
  2. L D Kaat1,
  3. Harro Seelaar1,
  4. Sonia M Rosso1,
  5. Agnita JW Boon1,
  6. Wouter Kamphorst2,
  7. John C van Swieten
  1. 1Department of Neurology, Erasmus Medical Centre, Rotterdam, The Netherlands
  2. 2Department of Neuropathology, Vrije Universiteit Medical Centre, Amsterdam, The Netherlands
  1. Correspondence to Dr John C. van Swieten, Department of Neurology, Erasmus University Medical Centre Rotterdam, Room Hs 611, ‘s-Gravendijkwal 230, 3015 CE Rotterdam, The Netherlands; j.c.vanswieten{at}erasmusmc.nl

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-τ. 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-τ was defined as Pick disease and FTDP-17 with MAPT mutations. Semiquantitative evaluation of τ-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-τ.

Conclusion 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.

  • Progressive supranuclear palsy
  • frontotemporal dementia
  • frontotemporal lobar degeneration
  • survival
  • PSP rating scale
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Footnotes

  • Funding This study was financially supported by: Prinses Beatrix Fonds (grant no 01-0128) Javastraat 86, 2585 AS Den Haag—the Hersenstichting (project no 13F05(2).14), Koediefstraat 5, 2511 CG Den Haag- stichting Dioraphte (grant no 07 01 05).

  • Competing interests None.

  • Ethics approval Ethics approval was provided by the Erasmus Medical Centre, Rotterdam.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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