Survival in progressive supranuclear palsy and frontotemporal dementia
Survival in progressive supranuclear palsy and frontotemporal dementia
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DOI：
10.1136/jnnp.2009.195719
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发表时间：
2010-04-01
影响因子：
11
通讯作者：
van Swieten, John C.
中科院分区：
文献类型：
--
作者：
Chiu, Wang Zheng;Kaat, L. D.;van Swieten, John C.
目的比较进行性核上性麻痹（PSP）和额颞叶痴呆（FTD）的生存率并确定预后预测因子。H1单倍型的纯合性与PSP相关，而在FTLD-tau中已鉴定出几种MAPT突变。生存时间可能反映了潜在的病理生理学或diseases.Methods PSP和FTD患者招募全国范围内的转诊。比较了354例FTD患者和197例PSP患者的生存率。进行考克斯回归分析以确定预后预测因素。FTLD-tau定义为Pick病和具有MAPT突变的FTDP-17。结果PSP患者的中位生存期（8.0年，95%CI 7.3 ~ 8.7）明显短于FTD患者（9.9年，95%CI 9.2 ~ 10.6）。校正人口统计学差异后，PSP患者的死亡风险仍显著高于FTD患者。在PSP中，男性、发病年龄较大和PSP评定量表评分较高被确定为生存期较短的独立预测因素，而在FTD中，阳性家族史和发病年龄较大与预后不良相关。风险率的差异更显着时，比较病理证实的情况下，PSP与FTLD-Tau.Conclusion PSP患者的生存期短于FTD患者，可能反映了一个更积极的疾病过程中PSP。PSP患者生存期较短的独立预测因素为男性、发病年龄较大和PSP评定量表评分较高，而FTD患者阳性家族史和发病年龄较高是预后较差的预测因素。
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.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.