Objective: To study whether quantitative downgaze palsy could predict the survival duration in patients with progressive supranuclear palsy (PSP).

Background: No clinical feature has been established to assess the survival duration in patients with PSP. Downgaze palsy is the most specific sign for PSP.

Method: We followed up patients with probable PSP-RS (Richardson syndrome) or PSP-P (parkinsonism) in our PSP center till they died from PSP during 2011 to 2019. We collected information on disease duration when downgaze palsy was first documented, the severity of downgaze palsy prospectively rated then (with 10 the least palsy and 100 the worst palsy in percentage), sex, age at onset (AO) of PSP, motor function and medication use for parkinsonism, pulmonary and cardiovascular diseases, and the total survival duration between the onset of PSP and death. Linear regression and multivariant Cox models were applied to identify predicting factors and form an equation to calculate the total survival duration.

Results: We found 23 patients with probable PSP-RS or PSP-P, all having hummingbird sign on MRI when downgaze palsy was documented. Disease duration and severity of downgaze palsy at the assessment were found to be predicting factors for total survival duration in both models. The total survival duration can be predicted by an equation as 5.76 + (1.11 x disease duration at the assessment)– (0.03 x downgaze palsy severity then, ranging from 10-100) –(0.03 x AO) (adjusted R²=0.92), which yielded a discrepancy of only 0.82 ± 0.67 years compared to the actual total survival duration.

Conclusion: Quantitative downgaze palsy predicts the total survival duration in patients with probable PSP-RS and PSP-P, which fulfills unmet needs to help clinical practice and trial enrollment.

References: 1. Litvan I, Agid Y, Kankovic J, et al. Accuracy of clinical criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome). Neurology 1996;46:922-930. 2. Lee WJ, Kim JH, Shin YU, et al. Differences in eye movement range based on age and gaze direction. Eye 2019:33:1145-1151. 3. Chen A, Riley D, King SA, et al. The disturbance of gaze in progressive supranuclear palsy: implications for pathogenesis. Front Neurol 2010;doi:10.3389/fneur.2010.00147. 4. Williams DR, Holton JL, Strand C, et al. Pathological tau burden and distribution distinguishes progressive supranuclear palsy-parkinsonism from Richardson’s syndrome. Brain 2007;130:1566 -1576. 5. Xie T, Kang UJ, Kuo SH, et al. Comparison of clinical features in pathologically confirmed PSP and MSA patients followed at a tertiary center. npj Parkinson’s Disease 2015;1:15007. 6. Williams DR, de Silva R, Paviour DC, et al. Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson’s syndrome and PSP-parkinsonism. Brain 2005;128:1247-1258. 7. Papapetropoulos S, Singer C, McCorquodale D, et al. Cause, seasonality of death and co-morbidities in progressive supranuclear palsy (PSP). Parkinsonism Relat Disord 2005;11:459-63. 8. Litvan I, Mangone CA, McKee A, et al. Natural history of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) and clinical predictors of survival: a clinicopathological study. J Neurol Neurosurg Psychiatry 1996;61:615-620. 9. Hoglinger GU, Respondek G, Stamelou M, et al. Clinical diagnosis of progressive supranuclear palsy: the movement disorder society criteria. Mov Disord 2017;32:853-864. 10. Boxer AL, Qureshi I, Ahlijanian M, et al. Safety of the tau-directed monoclonal antibody BIIB092 in progressive supranuclear palsy: a randomised, placebo-controlled, multiple ascending dose phase 1b trial. Lancet Neurology 2019;18:549-558. 11. Mueller C, Hussl A, Krismer F, et al. The diagnostic accuracy of the hummingbird and morning glory sign in patients with neurodegenerative parkinsonism. Parkinsonism Relat Disord 2018;54:90-94. 12. Respondek G, Stamelou M, Kurz C, et al. The phenotypic spectrum of progressive supranuclear palsy: a retrospective multicenter study of 100 definite cases. Mov Disord 2014;29:1758-1766.

« Back to MDS Virtual Congress 2021

MDS Abstracts - https://www.mdsabstracts.org/abstract/quantitative-downgaze-palsy-predicts-the-survival-duration-in-patients-with-progressive-supranuclear-palsy/