{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,30]],"date-time":"2026-10-30T17:48:18Z","timestamp":1793382498158,"version":"4.5.0"},"reference-count":49,"publisher":"MDPI AG","issue":"9","license":[{"start":{"date-parts":[[2022,8,24]],"date-time":"2022-08-24T00:00:00Z","timestamp":1661299200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"AbbVie Inc"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Brain Sciences"],"abstract":"<jats:p>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 \u22651 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\u20136.1) and median time from initial symptom to diagnosis was 4.2 years. The most common misdiagnoses were Parkinson\u2019s 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\u2019s rarity, investigation of PSP cohorts in different countries may create a proper platform for upcoming DMT trials.<\/jats:p>","DOI":"10.3390\/brainsci12091126","type":"journal-article","created":{"date-parts":[[2022,8,24]],"date-time":"2022-08-24T21:03:51Z","timestamp":1661375031000},"page":"1126","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":28,"title":["Epidemiology of Progressive Supranuclear Palsy: Real World Data from the Second Largest Health Plan in Israel"],"prefix":"10.3390","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3264-3380","authenticated-orcid":false,"given":"Yael","family":"Barer","sequence":"first","affiliation":[{"name":"Maccabitech, Maccabi Institute for Research and Innovation, Tel Aviv-Yafo 6812509, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gabriel","family":"Chodick","sequence":"additional","affiliation":[{"name":"Maccabitech, Maccabi Institute for Research and Innovation, Tel Aviv-Yafo 6812509, Israel"},{"name":"Sackler School of Medicine, Tel Aviv University, Tel Aviv-Yafo 6997801, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raanan","family":"Cohen","sequence":"additional","affiliation":[{"name":"AbbVie Inc., Hod Hasharon 4511001, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Meital","family":"Grabarnik-John","sequence":"additional","affiliation":[{"name":"AbbVie Inc., Hod Hasharon 4511001, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaolan","family":"Ye","sequence":"additional","affiliation":[{"name":"AbbVie Inc., North Chicago, IL 60064, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jorge","family":"Zamudio","sequence":"additional","affiliation":[{"name":"AbbVie Inc., North Chicago, IL 60064, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tanya","family":"Gurevich","sequence":"additional","affiliation":[{"name":"Sackler School of Medicine, Tel Aviv University, Tel Aviv-Yafo 6997801, Israel"},{"name":"Tel Aviv Sourasky Medical Center, Tel Aviv-Yafo 6423906, Israel"},{"name":"Sagol School of Neuroscience, Tel Aviv University, Tel Aviv-Yafo 6997801, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,8,24]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1125","DOI":"10.3389\/fneur.2019.01125","article-title":"Frontrunner in translation: Progressive supranuclear palsy","volume":"10","author":"Shoeibi","year":"2019","journal-title":"Front. Neurol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"604","DOI":"10.1002\/mdc3.13489","article-title":"Systematic review of prevalence studies of progressive supranuclear palsy and corticobasal syndrome","volume":"9","author":"Swallow","year":"2022","journal-title":"Mov. Disord. Clin. Pract."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"179","DOI":"10.3233\/JPD-191744","article-title":"Incidence and trends of progressive supranuclear palsy and corticobasal syndrome: A population-based study","volume":"10","author":"Stang","year":"2020","journal-title":"J. Parkinson\u2019s Dis."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"571800","DOI":"10.3389\/fneur.2021.571800","article-title":"Clinical Features of Patients with Progressive Supranuclear Palsy in an US Insurance Claims Database","volume":"12","author":"Viscidi","year":"2021","journal-title":"Front. Neurol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"S341","DOI":"10.1016\/S1353-8020(08)70028-2","article-title":"Unraveling progressive supranuclear palsy: From the bedsideback to the bench","volume":"13","author":"Houghton","year":"2007","journal-title":"Parkinsonism Relat. Disord."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"270","DOI":"10.1016\/S1474-4422(09)70042-0","article-title":"Progressive supranuclear palsy: Clinicopathological concepts and diagnostic challenges","volume":"8","author":"Williams","year":"2009","journal-title":"Lancet Neurol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"1247","DOI":"10.1093\/brain\/awh488","article-title":"Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson\u2019s syndrome and PSP-parkinsonism","volume":"128","author":"Williams","year":"2005","journal-title":"Brain"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"552","DOI":"10.1016\/S1474-4422(17)30157-6","article-title":"Advances in progressive supranuclear palsy: New diagnostic criteria, biomarkers, and therapeutic approaches","volume":"16","author":"Boxer","year":"2017","journal-title":"Lancet Neurol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"853","DOI":"10.1002\/mds.26987","article-title":"Clinical diagnosis of progressive supranuclear palsy: The movement disorder society criteria","volume":"32","author":"Respondek","year":"2017","journal-title":"Mov. Disord."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1016\/j.parkreldis.2021.01.015","article-title":"Association of PSP phenotypes with survival: A brain-bank study","volume":"84","author":"Guasp","year":"2021","journal-title":"Parkinsonism Relat. Disord."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"42","DOI":"10.1007\/s11940-016-0422-5","article-title":"Progressive supranuclear palsy and corticobasal degeneration: Pathophysiology and treatment options","volume":"18","author":"Lamb","year":"2016","journal-title":"Curr. Treat. Options Neurol."},{"key":"ref_12","doi-asserted-by":"crossref","unstructured":"H\u00f6llerhage, M., Klietz, M., and H\u00f6glinger, G.U. (2022). Disease modification in Parkinsonism: Obstacles and ways forward. J. Neural Transm.","DOI":"10.1007\/s00702-022-02520-6"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"2503","DOI":"10.1016\/S0140-6736(17)30636-0","article-title":"Health and health care in Israel: An introduction","volume":"389","author":"Clarfield","year":"2017","journal-title":"Lancet"},{"key":"ref_14","unstructured":"Israeli Central Bureau of Statistics (2013). Characterization and Classification of Geographic Units by the Soci-Economic Level of the Population 2008, Israeli Central Bureau of Statistics. Publication No. 1530."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1016\/j.ijcard.2010.08.002","article-title":"The use of an automated patient registry to manage and monitor cardiovascular conditions and related outcomes in a large health organization","volume":"152","author":"Shalev","year":"2011","journal-title":"Int. J. Cardiol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"1143","DOI":"10.1023\/B:EJEP.0000006635.36802.c8","article-title":"The epidemiology of diabetes in a large Israeli HMO","volume":"18","author":"Chodick","year":"2003","journal-title":"Eur. J. Epidemiol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"501","DOI":"10.1161\/HYPERTENSIONAHA.114.03718","article-title":"Prevalence and factors associated with resistant hypertension in a large health maintenance organization in Israel","volume":"64","author":"Weitzman","year":"2014","journal-title":"Hypertension"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"2518","DOI":"10.1001\/jama.2014.6634","article-title":"Decline in estimated glomerular filtration rate and subsequent risk of end-stage renal disease and mortality","volume":"311","author":"Coresh","year":"2014","journal-title":"JAMA"},{"key":"ref_19","unstructured":"Fishler, Y., Chetrit, A., Barchana, M., and Modan, B. (2003). Estimation of Completeness of the Cancer Registry in Israel, Israel Center for Disease Control, Ministry of Health."},{"key":"ref_20","unstructured":"Ahmad, O.B., Boschi-Pinto, C., Lopez, A.D., Murray, C.J., Lozano, R., and Inoue, M. (2001). Age Standardization of Rates: A New WHO Standard, World Health Organization."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"1736","DOI":"10.1212\/WNL.0000000000002638","article-title":"Prevalence, characteristics, and survival of frontotemporal lobar degeneration syndromes","volume":"86","author":"Dick","year":"2016","journal-title":"Neurology"},{"key":"ref_22","unstructured":"Pace, M., Lanzieri, G., Glickman, M., Grande, E., Zupanic, T., Wojtyniak, B., Gissler, M., Cayotte, E., and Agafitei, L. (2013). Revision of the European Standard Population, Publications Office of the European Union. Report of the Eurostat\u2019s Task Force."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"1438","DOI":"10.1093\/brain\/124.7.1438","article-title":"The prevalence of progressive supranuclear palsy (Steele\u2013Richardson\u2013Olszewski syndrome) in the UK","volume":"124","author":"Nath","year":"2001","journal-title":"Brain"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"182","DOI":"10.1111\/j.1600-0404.2010.01442.x","article-title":"Prevalence of Parkinson\u2019s disease and atypical parkinsonian syndromes in a rural Japanese district","volume":"124","author":"Osaki","year":"2011","journal-title":"Acta Neurol. Scand."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"1031","DOI":"10.1212\/WNL.38.7.1031","article-title":"Prevalence and natural history of progressive supranuclear palsy","volume":"38","author":"Golbe","year":"1988","journal-title":"Neurology"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"1771","DOI":"10.1016\/S0140-6736(99)04137-9","article-title":"Prevalence of progressive supranuclear palsy and multiple system atrophy: A cross-sectional study","volume":"354","author":"Schrag","year":"1999","journal-title":"Lancet"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"1239","DOI":"10.1002\/mds.20149","article-title":"Prevalence of progressive supranuclear palsy in Yonago, Japan","volume":"19","author":"Kawashima","year":"2004","journal-title":"Mov. Disord. Off. J. Mov. Disord. Soc."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1016\/j.parkreldis.2018.03.030","article-title":"Descriptive epidemiology of parkinsonism in the Canton of Geneva, Switzerland","volume":"54","author":"Fleury","year":"2018","journal-title":"Parkinsonism Relat. Disord."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"e00557","DOI":"10.1002\/brb3.557","article-title":"Prevalence of progressive supranuclear palsy in Yonago: Change throughout a decade","volume":"6","author":"Takigawa","year":"2016","journal-title":"Brain Behav."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"859","DOI":"10.1001\/jamaneurol.2013.114","article-title":"Incidence and pathology of synucleinopathies and tauopathies related to parkinsonism","volume":"70","author":"Savica","year":"2013","journal-title":"JAMA Neurol."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"1284","DOI":"10.1212\/WNL.49.5.1284","article-title":"Incidence of progressive supranuclear palsy and multiple system atrophy in Olmsted County, Minnesota, 1976 to 1990","volume":"49","author":"Bower","year":"1997","journal-title":"Neurology"},{"key":"ref_32","doi-asserted-by":"crossref","unstructured":"Jecmenica Lukic, M., Respondek, G., Kurz, C., Compta, Y., Gelpi, E., Ferguson, L.W., Rajput, A., Troakes, C., van Swieten, J.C., and Giese, A. (2022). Long-duration progressive supranuclear palsy: Clinical course and pathological underpinnings. Ann. Neurol.","DOI":"10.1002\/ana.26455"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1097\/WCO.0b013e328013f432","article-title":"Epidemiology of vertigo","volume":"20","author":"Neuhauser","year":"2007","journal-title":"Curr. Opin. Neurol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1136\/jnnp.58.2.167","article-title":"Progressive supranuclear palsy: Neuropathologically based diagnostic clinical criteria","volume":"58","author":"Collins","year":"1995","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1212\/WNL.0b013e318223c79d","article-title":"Postural imbalance and falls in PSP correlate with functional pathology of the thalamus","volume":"77","author":"Zwergal","year":"2011","journal-title":"Neurology"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"910","DOI":"10.1212\/01.WNL.0000052991.70149.68","article-title":"Clinical features and natural history of progressive supranuclear palsy: A clinical cohort study","volume":"60","author":"Nath","year":"2003","journal-title":"Neurology"},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1055\/s-0034-1381736","article-title":"Progressive supranuclear palsy","volume":"34","author":"Golbe","year":"2014","journal-title":"Semin. Neurol."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"1801","DOI":"10.1111\/ene.14800","article-title":"First symptom guides diagnosis and prognosis in neurodegenerative diseases\u2014A retrospective study of autopsy proven cases","volume":"28","author":"Kostova","year":"2021","journal-title":"Eur. J. Neurol."},{"key":"ref_39","doi-asserted-by":"crossref","unstructured":"Winikates, J., and Jankovic, J. (1994). Vascular progressive supranuclear palsy. Progressive Supranuclear Palsy: Diagnosis, Pathology, and Therapy, Springer.","DOI":"10.1007\/978-3-7091-6641-3_15"},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"570","DOI":"10.1212\/WNL.37.4.570","article-title":"Progressive supranuclear palsy and a multi-infarct state","volume":"37","author":"Dubinsky","year":"1987","journal-title":"Neurology"},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"166","DOI":"10.1016\/j.parkreldis.2019.07.036","article-title":"Hypertension and progressive supranuclear palsy","volume":"66","author":"Rabadia","year":"2019","journal-title":"Parkinsonism Relat. Disord."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"1104","DOI":"10.1001\/archneur.1997.00550210038010","article-title":"Presymptomatic hypertension is a major feature in the diagnosis of progressive supranuclear palsy","volume":"54","author":"Ghika","year":"1997","journal-title":"Arch. Neurol."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"694","DOI":"10.1002\/mds.10392","article-title":"Lack of association between progressive supranuclear palsy and arterial hypertension: A clinicopathological study","volume":"18","author":"Colosimo","year":"2003","journal-title":"Mov. Disord."},{"key":"ref_44","first-page":"25","article-title":"Supranuclear ophthalmoplegia, pseudobulbar palsy, nuchal dystonia and dementia. A clinical report on eight cases of \u201cheterogenous system degeneration\u201d","volume":"88","author":"Richardson","year":"1963","journal-title":"Trans. Am. Neurol. Assoc."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"114","DOI":"10.1212\/WNL.54.1.114","article-title":"Smoking habits in multiple system atrophy and progressive supranuclear palsy","volume":"54","author":"Vanacore","year":"2000","journal-title":"Neurology"},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"644","DOI":"10.1002\/mds.26512","article-title":"Environmental and occupational risk factors for progressive supranuclear palsy: Case-control study","volume":"31","author":"Litvan","year":"2016","journal-title":"Mov. Disord."},{"key":"ref_47","unstructured":"Ministry of Health, I. (2022, August 17). The Minister of Health\u2019s Report on Smoking in Israel for 2020, Available online: https:\/\/www.gov.il\/BlobFolder\/reports\/smoking-2020\/he\/files_publications_smoking_prevention_smoking_2020.pdf."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"385","DOI":"10.3390\/diagnostics12020385","article-title":"The Strengths and Obstacles in the Differential Diagnosis of Progressive Supranuclear Palsy\u2014Parkinsonism Predominant (PSP-P) and Multiple System Atrophy (MSA) Using Magnetic Resonance Imaging (MRI) and Perfusion Single Photon Emission Computed Tomography (SPECT)","volume":"12","author":"Alster","year":"2022","journal-title":"Diagnostics"},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"459","DOI":"10.1016\/j.pjnns.2018.06.002","article-title":"Difficulties in the diagnosis of four repeats (4R) tauopathic parkinsonian syndromes","volume":"52","author":"Alster","year":"2018","journal-title":"Neurol. Neurochir. Pol."}],"container-title":["Brain Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2076-3425\/12\/9\/1126\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T00:14:53Z","timestamp":1760141693000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2076-3425\/12\/9\/1126"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,8,24]]},"references-count":49,"journal-issue":{"issue":"9","published-online":{"date-parts":[[2022,9]]}},"alternative-id":["brainsci12091126"],"URL":"https:\/\/doi.org\/10.3390\/brainsci12091126","relation":{},"ISSN":["2076-3425"],"issn-type":[{"value":"2076-3425","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,8,24]]}}}