Predictors of survival in a series of clinically diagnosed progressive supranuclear palsy patients
Predictors of survival in a series of clinically diagnosed progressive supranuclear palsy patients
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DOI：
10.1016/j.parkreldis.2013.06.014
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发表时间：
2013-11-01
影响因子：
4.1
通讯作者：
Logroscino, Giancarlo
中科院分区：
文献类型：
--
作者：
dell'Aquila, Claudia;Zoccolella, Stefano;Logroscino, Giancarlo
背景：对进行性核上性麻痹预后因素的研究显示了相互矛盾的结果。我们进行了一项回顾性研究，以确定临床诊断为进行性核上性麻痹患者的生存的临床预测因素。方法：从门诊病历和护理人员电话访谈中收集病史、生存和5个临床残疾里程碑（无法独立行走、言语不清、严重吞咽困难、痴呆和住院）的数据。根据发病前2年的症状将患者细分为理查德森综合征和psp -帕金森症。生存率采用Kaplan Meier法和Cox回归分析。结果：连续纳入43例患者（86%为理查德森综合征）。运动障碍是最常见的发病症状。在随访期间，60.5%的患者在7.1年（2.2-18年）的中位生存期后死亡。发病年龄较大（bb0 63岁）（危险度2.8;95% Cl: 1.3-5.7; p = 0.007），早期吞咽困难(危险度2.3；95% Cl: 1-5.3；p = 0.05)和早期认知缺陷（风险比3.6;95% Cl: 1.6-8.2; p = 0.002）是较短生存期的预测因素。与psp -帕金森患者相比，理查德森综合征患者的生存期更短，死亡风险更高，但无统计学意义（HR 3 95% Cl: 0.9-9.9; p = 0.07）。77%的患者在随访期间出现严重残疾：到达第一个临床残疾里程碑的时间越短，生存期越短（HR 7.8; 95% Cl: 2.3-26; p = 0.0008）。结论：早期吞咽困难、认知障碍、发病年龄和致残时间是较短生存期的预测因素；理查德森综合症的病程不如帕金森综合症。临床里程碑应被视为未来临床试验的可能终点。(C) 2013 Elsevier Ltd.版权所有。
Background: Investigations into prognostic factors in progressive supranuclear palsy have shown conflicting results. We performed a retrospective study in order to identify clinical predictors of survival in clinically diagnosed progressive supranuclear palsy patients referred to our centre.Methods: Data on medical history, survival and five clinical disability milestones (inability to walk unassisted, unintelligible speech, severe dysphagia, dementia and institutionalization) were collected from outpatients' medical records and by a telephone interview to caregivers. Patients were subdivided into Richardson's syndrome and PSP-Parkinsonism according to symptoms during the first 2 years of disease. Survival was analyzed by the Kaplan Meier method and Cox regression analysis.Results: Forty-three consecutive patients were enrolled (86% Richardson's syndrome). Motor disturbances were the most frequent symptoms of onset. During the follow-up, 60.5% of patients died after a median survival of 7.1 years (2.2-18). Older age at onset (> 63) (HR 2.8; 95% Cl: 1.3-5.7; p = 0.007), early dysphagia (HR 2.3;.95% Cl: 1-5.3; p = 0.05) and early cognitive deficits (HR 3.6; 95% Cl: 1.6-8.2; p = 0.002) were predictors of shorter survival. Compared to PSP-Parkinsonism patients, Richardson's syndrome patients had shorter survival and higher mortality risk although not statistically significant (HR 3 95% Cl: 0.9-9.9; p = 0.07). Seventy-seven percent of patients developed severe disability during follow-up: shorter time to the first clinical disability milestone predicted shorter survival (HR 7.8; 95% Cl: 2.3-26; p = 0.0008).Conclusions: early dysphagia, cognitive impairment, older age at onset, and time to disability were predictors of shorter survival; Richardson's syndrome had a less favorable course than PSP-Parkinsonism. Clinical milestones should be considered as possible endpoints in future clinical trials. (C) 2013 Elsevier Ltd. All rights reserved.