@article{ff1b053482a24432a6ae91335a4cbe36,
title = "Rainwater Charitable Foundation criteria for the neuropathologic diagnosis progressive supranuclear palsy",
abstract = "Neuropathologic criteria for progressive supranuclear palsy (PSP) proposed by a National Institute of Neurological Disorders and Stroke (NINDS) working group were published in 1994 and based on the presence of neurofibrillary tangles in basal ganglia and brainstem. These criteria did not stipulate detection methods or incorporate glial tau pathology. In this study, a group of 14 expert neuropathologists scored digital slides from 10 brain regions stained with hematoxylin and eosin (H&E) and phosphorylated tau (AT8) immunohistochemistry. The cases included 15 typical and atypical PSP cases and 10 other tauopathies. Blinded to clinical and neuropathological information, raters provided a categorical diagnosis (PSP or not-PSP) based upon provisional criteria that required neurofibrillary tangles or pretangles in two of three regions (substantia nigra, subthalamic nucleus, globus pallidus) and tufted astrocytes in one of two regions (peri-Rolandic cortices, putamen). The criteria showed high sensitivity (0.97) and specificity (0.91), as well as almost perfect inter-rater reliability for diagnosing PSP and differentiating it from other tauopathies (Fleiss kappa 0.826). Most cases (17/25) had 100% agreement across all 14 raters. The Rainwater Charitable Foundation criteria for the neuropathologic diagnosis of PSP feature a simplified diagnostic algorithm based on phosphorylated tau immunohistochemistry and incorporate tufted astrocytes as an essential diagnostic feature.",
keywords = "Autopsy cohort, Criteria, Human, Neurofibrillary tangles, Neuropathology, Oligodendroglia, Phosphorylated tau, Progressive supranuclear palsy (PSP), Threads, Tufted astrocytes",
author = "Roemer, {Shanu F.} and Grinberg, {Lea T.} and Crary, {John F.} and Seeley, {William W.} and McKee, {Ann C.} and Kovacs, {Gabor G.} and Beach, {Thomas G.} and Charles Duyckaerts and Ferrer, {Isidro A.} and Ellen Gelpi and Lee, {Edward B.} and Tamas Revesz and White, {Charles L.} and Mari Yoshida and Pereira, {Felipe L.} and Kristen Whitney and Ghayal, {Nikhil B.} and Dickson, {Dennis W.}",
note = "Funding Information: We are deeply grateful to the PSP patients and their families who made brain donations supported by CurePSP for diagnosis and research that enabled this effort to update neuropathologic criteria, but also provide resources for current and future research on PSP. We acknowledge the generous support from the Rainwater Charitable Foundation that made this study possible. We acknowledge excellent histological services provided by Ms. Monica Castanedes-Casey and Ms. Virginia Philips at Mayo Clinic and excellent administrative help provided by Ms. Laura Wise at UCSF. After the completion of this study, Dr. Duyckaerts sadly passed away after a long term illness. We dedicate these revised neuropathologic criteria to our late highly esteemed colleagues, Dr. Charles Duyckaerts, Dr. Peter Davies, Dr. John Trojanowski and Dr. John Steele whose endeavors have shaped the field of tauopathies and PSP in particular. This study was supported in part by the following National Institute of Health grants: Thomas G. Beach—P30AG019610, U24NS072026 and R01NS118669; John F. Crary—R01-AG062348, R01-NS095252, U54NS115266, R01AG054008, RF1 AG060961, R01NS086736 and P30AG066514; Dennis W. Dickson—R01AG062348, U54NS110435, U19AG069701, UG3NS104095 and P30AG062677; Lea T. Grinberg—R01AG060477, K24AG053435, U54NS100717, P30AG062422, P50AG023501, R01AG064314, R01AG070826 and R01AG075802; Edward B. Lee—P30AG072979, U54NS115322, P01AG066597 and U19AG062418; Ann C. McKee—R01AG062348, U54NS115266, U19AG068753 and P30AG072978; William W. Seeley—P30AG062422, U19AG063911, P01AG019724, R01NS104437 and P50AG023501 Funding Information: We are deeply grateful to the PSP patients and their families who made brain donations supported by CurePSP for diagnosis and research that enabled this effort to update neuropathologic criteria, but also provide resources for current and future research on PSP. We acknowledge the generous support from the Rainwater Charitable Foundation that made this study possible. We acknowledge excellent histological services provided by Ms. Monica Castanedes-Casey and Ms. Virginia Philips at Mayo Clinic and excellent administrative help provided by Ms. Laura Wise at UCSF. After the completion of this study, Dr. Duyckaerts sadly passed away after a long term illness. We dedicate these revised neuropathologic criteria to our late highly esteemed colleagues, Dr. Charles Duyckaerts, Dr. Peter Davies, Dr. John Trojanowski and Dr. John Steele whose endeavors have shaped the field of tauopathies and PSP in particular. This study was supported in part by the following National Institute of Health grants: Thomas G. Beach—P30AG019610, U24NS072026 and R01NS118669; John F. Crary—R01-AG062348, R01-NS095252, U54NS115266, R01AG054008, RF1 AG060961, R01NS086736 and P30AG066514; Dennis W. Dickson—R01AG062348, U54NS110435, U19AG069701, UG3NS104095 and P30AG062677; Lea T. Grinberg—R01AG060477, K24AG053435, U54NS100717, P30AG062422, P50AG023501, R01AG064314, R01AG070826 and R01AG075802; Edward B. Lee—P30AG072979, U54NS115322, P01AG066597 and U19AG062418; Ann C. McKee—R01AG062348, U54NS115266, U19AG068753 and P30AG072978; William W. Seeley—P30AG062422, U19AG063911, P01AG019724, R01NS104437 and P50AG023501 Publisher Copyright: {\textcopyright} 2022, The Author(s).",
year = "2022",
month = oct,
doi = "10.1007/s00401-022-02479-4",
language = "English (US)",
journal = "Acta neuropathologica",
issn = "0001-6322",
publisher = "Springer Verlag",
}