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. 2022 Aug 24;12(9):1126.
doi: 10.3390/brainsci12091126.

Epidemiology of Progressive Supranuclear Palsy: Real World Data from the Second Largest Health Plan in Israel

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Epidemiology of Progressive Supranuclear Palsy: Real World Data from the Second Largest Health Plan in Israel

Yael Barer et al. Brain Sci. .

Abstract

Progressive supranuclear palsy (PSP) is a rare and fatal neurodegenerative movement disorder and no disease modifying therapy (DMT) is currently available. This study aims to assess the epidemiology of PSP in Israel and to describe its clinical features. This retrospective analysis identified patients with PSP between 2000 and 2018 over the age of 40 years at first diagnosis (index date). We identified 209 patients with ≥1 diagnosis of PSP. Of those, 88 patients satisfied the inclusion criteria with a mean age at diagnosis of 72 years (SD = 8) and 53% were female. The 2018 prevalence and incidence rates were 5.3 and 1 per 100,000 persons, respectively. Median survival time was 4.9 years (95% CI 3.6-6.1) and median time from initial symptom to diagnosis was 4.2 years. The most common misdiagnoses were Parkinson's disease, cognitive disorder and depression. The present study demonstrates that the clinic-epidemiological features of PSP in Israel are similar to PSP worldwide. In light of PSP's rarity, investigation of PSP cohorts in different countries may create a proper platform for upcoming DMT trials.

Keywords: clinical features; future disease modifying trials; incidence; natural history; prevalence; progressive supranuclear palsy; real world data.

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Conflict of interest statement

The authors Y.B., T.G. and G.C. declare no conflict of interest. X.Y., Z.J., M.G.-J. and R.C. are employees of AbbVie and may own stocks of AbbVie. The study was funded by AbbVie. The design and study conduct were performed in collaboration with AbbVie and Maccabi healthcare services, in addition to their participation in the interpretation of the data, review, and approval of the publication. No honoraria or payments were made for authorship.

Figures

Figure 1
Figure 1
Attrition process of patients with PSP.
Figure 2
Figure 2
PSP age-specific prevalence rates in 2018. [4,21]
Figure 3
Figure 3
Survival curve of patients with PSP, from first diagnosis, calculated using Kaplan–Meier curve.
Figure 4
Figure 4
Time to disability, PwPSP with no disability at baseline (n = 56), calculated using Kaplan–Meier curves.

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