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The prevalence of clinicopathologically defined PSP subjects in the entire autopsy dataset was 9.1%, while the incidence rate was estimated at 780 per 100\u200a000 persons per year, roughly 50-fold greater than most previous clinically determined PSP incidence estimates. A clinical diagnosis of PSP was 99.6% specific but only 9.2% sensitive based on first examination, and 99.3% specific and 20.7% sensitive based on the final clinical exam. Of the clinicopathologically defined PSP cases, 35\/87 (\u223c40%) had no form of parkinsonism at first assessment, while this decreased to 18\/83 (21.7%) at final assessment. Our study confirms a high specificity but low sensitivity for the clinical diagnosis of PSP. The low clinical sensitivity for PSP is likely primarily responsible for previous underestimates of the PSP population incidence rate.<\/jats:p>","DOI":"10.1093\/jnen\/nlad025","type":"journal-article","created":{"date-parts":[[2023,4,11]],"date-time":"2023-04-11T18:27:57Z","timestamp":1681237677000},"page":"438-451","source":"Crossref","is-referenced-by-count":28,"title":["Low clinical sensitivity and unexpectedly high incidence for neuropathologically diagnosed progressive supranuclear palsy"],"prefix":"10.1093","volume":"82","author":[{"given":"Erika D","family":"Driver-Dunckley","sequence":"first","affiliation":[{"name":"Department of Neurology, Parkinson\u2019s Disease and Movement Disorders Center, Mayo Clinic , Scottsdale, Arizona, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nan","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Quantitative Health Sciences, Section of Biostatistics, Mayo Clinic , Scottsdale, Arizona, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Geidy E","family":"Serrano","sequence":"additional","affiliation":[{"name":"Banner Sun Health Research Institute, Banner Health , Sun City, Arizona, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nathaniel A","family":"Dunckley","sequence":"additional","affiliation":[{"name":"Banner Sun Health Research Institute, Banner Health , Sun City, Arizona, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lucia I","family":"Sue","sequence":"additional","affiliation":[{"name":"Banner Sun Health Research Institute, Banner Health , Sun City, Arizona, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Holly A","family":"Shill","sequence":"additional","affiliation":[{"name":"Department of Neurology, Barrow Neurological Institute , Phoenix, Arizona, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shyamal H","family":"Mehta","sequence":"additional","affiliation":[{"name":"Department of Neurology, Parkinson\u2019s Disease and Movement Disorders Center, Mayo Clinic , Scottsdale, Arizona, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christine","family":"Belden","sequence":"additional","affiliation":[{"name":"Banner Sun Health Research Institute, Banner Health , Sun City, Arizona, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cecilia","family":"Tremblay","sequence":"additional","affiliation":[{"name":"Banner Sun Health Research Institute, Banner Health , Sun City, Arizona, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alireza","family":"Atri","sequence":"additional","affiliation":[{"name":"Banner Sun Health Research Institute, Banner Health , Sun City, Arizona, USA"},{"name":"Department of Neurology, Center for Mind\/Brain Medicine, Brigham & Women\u2019s Hospital & Harvard Medical School , Boston, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charles H","family":"Adler","sequence":"additional","affiliation":[{"name":"Department of Neurology, Parkinson\u2019s Disease and Movement Disorders Center, Mayo Clinic , Scottsdale, Arizona, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas G","family":"Beach","sequence":"additional","affiliation":[{"name":"Banner Sun Health Research Institute, Banner Health , Sun City, Arizona, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2023,4,11]]},"reference":[{"key":"2024032523513962700_nlad025-B1","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1001\/archneur.1964.00460160003001","article-title":"A heterogeneous degeneration involving the brain stem, basal ganglia and cerebellum with vertical gaze and pseudobulbar palsy, nuchal dystonia and dementia","volume":"10","author":"Steele","year":"1964","journal-title":"Arch 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