The prevalence and incidence of progressive supranuclear palsy and corticobasal syndrome: a systematic review and meta-analysis

Introduction Progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) are progressive neurodegenerative syndromes characterised by Parkinsonism with additional features including cognitive dysfunction, falls, and oculomotor abnormalities. Understanding the epidemiology of these conditions is critical to planning for future service provision. Methods We conducted a systematic review of studies reporting incidence and prevalence of CBS and PSP. A search of the PubMed and EMBASE data bases was conducted from their date of inception to 13th July 2021. Meta-analysis of studies sharing similar methodologies was carried out to generate estimated pooled prevalence and incidence. Results We found 32 studies meeting our criteria for inclusion. There were 20 studies with data on prevalence and 12 with incidence data of PSP. Prevalence of CBS was reported in eight studies while seven studies reported incidence. Reported estimates of prevalence for PSP ranged from 1.00 (0.9–1.1) to 18 (8–28) per 100,000 while prevalence rates for CBS ranged from 0.83 (0.1–3.0) to 25 (0–59). Incidence rates for PSP and CBS respectively ranged from 0.16 (0.07–0.39) to 2.6 per 100,000 person-years and 0.03 (0–0.18) to 0.8 (0.4–1.3) per 100,000 person-years. A random effects model meta-analysis of studies with similar methodologies yielded a pooled prevalence estimate for PSP of 6.92 (4.33–11.06, I 2 = 89%, τ 2 = 0.3907) and 3.91 (2.03–7.51, I 2 = 72%, τ 2 = 0.2573) per 100,000 for CBS. Conclusion Studies of the epidemiology of PSP and CBS report highly heterogeneous findings. There is a need for further studies using rigorous phenotyping and the most recent diagnostic criteria to understand the true burden of these conditions.