The reported prevalence of progressive supranuclear palsy (PSP) - which usually reflects that of the most common phenotypic subtype, Richardson’s syndrome (PSP-RS) - is around 5-6/100,000 people with no gender preponderance, according to data from the UK.[5]Nath U, Ben-Shlomo Y, Thomson RG, et al. The prevalence of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) in the UK. Brain. 2001 Jul;124(pt 7):1438-49.
https://academic.oup.com/brain/article/124/7/1438/285520
http://www.ncbi.nlm.nih.gov/pubmed/11408338?tool=bestpractice.com
[6]Schrag A, Ben-Shlomo Y, Quinn NP. Prevalence of progressive supranuclear palsy and multiple system atrophy: a cross-sectional study. Lancet. 1999 Nov 20;354(9192):1771-5.
http://www.ncbi.nlm.nih.gov/pubmed/10577638?tool=bestpractice.com
A higher prevalence of about 18/100,000 people has been reported from Japan, probably because of two factors: the inclusion of the parkinsonism (PSP-P) and progressive gait freezing (PSP-PGF) phenotypes in addition to PSP-RS, and better detection and diagnosis due to government support programmes for patients with PSP.[7]Takigawa H, Kitayama M, Wada-Isoe K, et al. Prevalence of progressive supranuclear palsy in Yonago: change throughout a decade. Brain Behav. 2016 Dec;6(12):e00557.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5166993
http://www.ncbi.nlm.nih.gov/pubmed/28031995?tool=bestpractice.com
PSP prevalence increases with age.[8]Swallow DMA, Zheng CS, Counsell CE. Systematic review of prevalence studies of progressive supranuclear palsy and corticobasal syndrome. Mov Disord Clin Pract. 2022 Jul;9(5):604-13.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9274340
http://www.ncbi.nlm.nih.gov/pubmed/35844273?tool=bestpractice.com
[9]Savica R, Grossardt BR, Bower JH, et al. Incidence and pathology of synucleinopathies and tauopathies related to parkinsonism. JAMA Neurol. 2013 Jul;70(7):859-66.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3707980
http://www.ncbi.nlm.nih.gov/pubmed/23689920?tool=bestpractice.com
The typical age of onset is the mid 60s.[10]Bluett B, Pantelyat AY, Litvan I, et al. Best practices in the clinical management of progressive supranuclear palsy and corticobasal syndrome: a consensus statement of the CurePSP Centers of Care. Front Neurol. 2021 Jul 1;12:694872.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8284317
http://www.ncbi.nlm.nih.gov/pubmed/34276544?tool=bestpractice.com
No autopsy-confirmed case has been demonstrated in an individual younger than age 40 years.[3]Höglinger GU, Respondek G, Stamelou M, et al. Clinical diagnosis of progressive supranuclear palsy: the Movement Disorder Society criteria. Mov Disord. 2017 Jun;32(6):853-64.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5516529
http://www.ncbi.nlm.nih.gov/pubmed/28467028?tool=bestpractice.com
The median survival has been reported as 6 to 7.4 years.[11]Bessemer R, Iansavichene A, Jenkins ME, et al. Clinical milestones as triggers for palliative care intervention in progressive supranuclear palsy and multiple system atrophy. J Neurol Sci. 2023 May 15;448:120614.
http://www.ncbi.nlm.nih.gov/pubmed/37001415?tool=bestpractice.com
There are as yet no epidemiological data about any difference in disease incidence, prevalence, or prognosis between different ethnicities.
Of all PSP clinical variants, PSP-RS is the most common phenotype, accounting for 40% to 50% of cases, followed by PSP-P, which accounts for about 30% to 40%.[4]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 Dec;29(14):1758-66.
http://www.ncbi.nlm.nih.gov/pubmed/25370486?tool=bestpractice.com
[12]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 Jun;128(Pt 6):1247-58.
https://academic.oup.com/brain/article/128/6/1247/432017
http://www.ncbi.nlm.nih.gov/pubmed/15788542?tool=bestpractice.com
[13]Morgan JC, Ye X, Mellor JA, et al. Disease course and treatment patterns in progressive supranuclear palsy: a real-world study. J Neurol Sci. 2021 Feb 15;421:117293.
http://www.ncbi.nlm.nih.gov/pubmed/33385754?tool=bestpractice.com
Of the remaining variants, PSP-corticobasal syndrome (PSP-CBS), PSP-frontal presentation (PSP-F), and PSP-speech/language disorder (PSP-SL) are probably more common than PSP-progressive gait freezing (PSP-PGF).[2]Olfati N, Shoeibi A, Litvan I. Clinical spectrum of tauopathies. Front Neurol. 2022 Jul 14;13:944806.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9329580
http://www.ncbi.nlm.nih.gov/pubmed/35911892?tool=bestpractice.com