Objective: To characterise a longitudinal cohort of patients with Progressive Supranuclear Palsy (PSP) and identify factors influencing progression.

Background: Median survival in PSP is approximately 7.3 years from symptom onset but this is highly variable, with longer survival reported in non-PSP-Richardson’s syndrome (PSP-RS) phenotypes. Little is known about what else determines rate of progression or survival. This is important for designing clinical trials.

Method: We identified patients from the PROSPECT-M-UK study with a baseline diagnosis of PSP and a baseline assessment before 1^st February 2024. Most of these patients have follow-up data on survival and progression, while a subset also has detailed in-person neurological and cognitive assessments at multiple timepoints and biomarker collection. All patients had blood or saliva collected for DNA extraction. DNA samples were genotyped using the Global Parkinson’s Genetics Project (GP2) NeuroBooster Array (NBA). We also measured serum biomarkers at baseline using the NUcleic acid Linked Immuno-Sandwich Assay (NULISA) CNS panel. We created a combined endpoint of death or physical dependence using the Schwab and England Activities of Daily Living scale (SEADL), where a score of <70% indicates some dependence and used a Cox proportional hazard model to identify determinants of survival without dependence. We will also assess rate of decline using the following scales: UPDRS, PSPRS, MoCA.

Results: We identified 678 patients with a baseline diagnosis of PSP. 231 of these are still alive. The median disease duration from symptom onset to death was 6.8 years. 229 patients had at least one follow-up assessment. Survival data was available for 645 patients and SEADL data was available for 321. Post-mortem examination data is available for 53 patients, of which 41 (77.4%) had a pathological diagnosis of PSP. Phenotype predicted progression, and disease duration was significantly longer in PSP-Parkinsonism patients as compared to PSP-RS patients (mean 9.2 years vs 7.3 years, p <0.001). Further analysis of biomarkers and genetic data is ongoing.

Conclusion: PSP is a rare neurodegenerative condition with variable survival. Through integration of clinical, genetic and biomarker data we hope to identify factors that predict survival and deterioration in motor and cognitive scales in our large cohort of PSP patients. This will inform future clinical trial design.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/tracking-progression-and-survival-in-a-large-cohort-of-patients-with-progressive-supranuclear-palsy/