Abstract
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.
Highlights
Downgaze palsy in the first year of disease onset is a very specific feature of progressive supranuclear palsy (PSP)-RS [1]
We collected information on sex, at onset (AO) of PSP, disease duration when the downgaze palsy was first quantitatively documented, and the severity rated at that time, the UPDRS-III (Unified Parkinson’s Disease Rating Sale, motor section) scores, medication use or following a visit for parkinsonism, cardiovascular diseases or risk factors, and pulmonary diseases
AO was 66.7 ± 7.9 years; disease duration when the first downgaze palsy was documented was 4.1 ± 3.4 years; the severity of downgaze palsy was 62.8 ± 31.2% ; the total survival duration from the disease onset to death was 6.6 ± 4.0 years; remaining survival duration after the assessment for the first downgaze palsy was 2.5 ± 1.4 years; cardiovascular disease was present in 17 patients (74%); pulmonary disease was present in 4 patients (17%); medication was tried in 10 patients (43%); UPDRS-III was recorded in all 23 patients, with an average score of 29.3 ± 9.1
Summary
Downgaze palsy in the first year of disease onset is a very specific feature of PSP-RS (progressive supranuclear palsy, Richardson syndrome) [1]. In our previous autopsy study, earlier onset of downgaze palsy (or shorter latency between the disease onset and the occurrence of downgaze palsy) was associated with a shorter survival duration of PSP (-RS, or -P) [5]. This result is consistent with the observation that patients with PSP-RS (with earlier occurrence of downgaze palsy) have a worse prognosis and shorter survival duration than PSP-P (with later occurrence or even no occurrence of downgaze palsy before the death) [6]. We hypothesized that the combination of the disease duration when downgaze palsy was first identified (not necessarily at the onset) with the severity of downgaze palsy might predict prognosis in clinical practice, regardless of when the downgaze palsy first occurs or when the patient is first seen. The potential roles of other factors in the survival duration could be assessed, such as the sex, age at onset (AO) of PSP, medication use for motor symptoms (symptomatic use for parkinsonism), the severity of the motor symptoms, and the presence of pulmonary and cardiovascular diseases (as aspiration pneumonia and its complications are the common causes of death in PSP) [7, 8]
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