Survival in progressive supranuclear palsy and frontotemporal dementia

• Published in: Journal of neurology, neurosurgery and psychiatry Vol. 81; no. 4; pp. 441 - 445
• Main Authors: Chiu, Wang Zheng, Kaat, L D, Seelaar, Harro, Rosso, Sonia M, Boon, Agnita JW, Kamphorst, Wouter, van Swieten, John C
• Format: Journal Article
• Language: English
• Published: London BMJ Publishing Group Ltd 01.04.2010; BMJ Publishing Group; BMJ Publishing Group LTD
• Subjects: Age of Onset; Aged; Biological and medical sciences; Brain - blood supply; Brain - pathology; Cognition Disorders - diagnosis; Cognition Disorders - epidemiology; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Dementia; Female; frontotemporal dementia; Frontotemporal Dementia - diagnosis; Frontotemporal Dementia - genetics; Frontotemporal Dementia - mortality; frontotemporal lobar degeneration; Genotype; Humans; Magnetic Resonance Imaging; Male; Medical sciences; Neurology; Neuropsychological Tests; Prognosis; Progressive supranuclear palsy; Prospective Studies; PSP rating scale; Supranuclear Palsy, Progressive - diagnosis; Supranuclear Palsy, Progressive - genetics; Supranuclear Palsy, Progressive - mortality; survival; Survival Rate; tau Proteins - genetics; Time Factors; Tomography, Emission-Computed, Single-Photon; Eye disease; Nervous system diseases; Central nervous system disease; Supranuclear ophthalmoplegia; Degenerative disease; Oculomotor syndrome; Frontotemporal dementia; Brain stem syndrome; Survival; Cerebral disorder; FRONTAL LOBE; SUPRANUCLEAR PALSY
• ISSN: 0022-3050; 1468-330X
• DOI: 10.1136/jnnp.2009.195719

ObjectiveTo compare survival and to identify prognostic predictors for progressive supranuclear palsy and frontotemporal dementia.BackgroundProgressive 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.MethodsPatients 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.ResultsThe 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.ConclusionSurvival 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.