Xie Tao, Yuen Carlen A, Kang Wenjun, Padmanaban Mahesh, Hain Timothy C, Nichols Jeffrey
Department of Neurology, University of Chicago Medicine, Chicago, IL, United States.
Department of Neurology, Columbia University Medical Center, New York, NY, United States.
Front Neurol. 2021 Sep 28;12:736784. doi: 10.3389/fneur.2021.736784. eCollection 2021.
It is an unmet need to estimate survival duration for patients with progressive supranuclear palsy (PSP). The objective of this study was to identify factors associated with the survival duration in patients with PSP. We followed up 23 patients with probable PSP-RS (Richardson syndrome) or PSP-P (parkinsonism) in our PSP center until death from 2011 to 2019. We prospectively and quantitatively rated their downgaze palsy whenever first noticed in our clinic. This was utilized along with the disease duration, motor function, medication use for parkinsonism, sex, age at onset of PSP, comorbid pulmonary and cardiovascular diseases, and the total survival duration from the onset of PSP to death for prediction analysis. A well-fitted linear regression model and a multivariant Cox model were applied to identify predicting factors for total survival duration. All patients had the specific hummingbird sign on brain MRI for PSP when downgaze palsy was documented. We found that the severity of downgaze palsy and the disease duration at the assessment were consistently correlated with the total survival duration in both models. The total survival duration could be further estimated by a formed regression equation. We conclude that severity and time to develop downgaze palsy could help to estimate the total survival duration in patients with probable PSP-RS and PSP-P, the major forms of PSP, which has significant clinical applications in clinical counseling and trial enrollment.
估计进行性核上性麻痹（PSP）患者的生存时间是一项尚未满足的需求。本研究的目的是确定与PSP患者生存时间相关的因素。2011年至2019年，我们在PSP中心对23例可能患有PSP-RS（理查森综合征）或PSP-P（帕金森综合征）的患者进行随访直至死亡。我们在诊所首次发现患者下视麻痹时，对其进行前瞻性定量评分。将此评分与病程、运动功能、帕金森病用药情况、性别、PSP发病年龄、合并的肺部和心血管疾病以及从PSP发病到死亡的总生存时间一起用于预测分析。应用拟合良好的线性回归模型和多变量Cox模型来确定总生存时间的预测因素。当记录到下视麻痹时，所有患者脑部MRI均有PSP特有的蜂鸟征。我们发现，在两个模型中，下视麻痹的严重程度和评估时的病程均与总生存时间一致相关。总生存时间可通过一个形成的回归方程进一步估计。我们得出结论，下视麻痹的严重程度和出现时间有助于估计PSP的主要类型PSP-RS和PSP-P患者的总生存时间，这在临床咨询和试验入组方面具有重要的临床应用价值。
