Rainwater Charitable Foundation criteria for the neuropathologic diagnosis progressive supranuclear palsy

Shanu F. Roemer; Lea T. Grinberg; John F. Crary; William W. Seeley; Ann C. McKee; Gabor G. Kovacs; Thomas G. Beach; Charles Duyckaerts; Isidro A. Ferrer; Ellen Gelpi; Edward B. Lee; Tamas Revesz; Charles L. White; Mari Yoshida; Felipe L. Pereira; Kristen Whitney; Nikhil B. Ghayal; Dennis W. Dickson

doi:10.1007/s00401-022-02479-4

Rainwater Charitable Foundation criteria for the neuropathologic diagnosis progressive supranuclear palsy

Shanu F. Roemer, Lea T. Grinberg, John F. Crary, William W. Seeley, Ann C. McKee, Gabor G. Kovacs, Thomas G. Beach, Charles Duyckaerts, Isidro A. Ferrer, Ellen Gelpi, Edward B. Lee, Tamas Revesz, Charles L. White, Mari Yoshida, Felipe L. Pereira, Kristen Whitney, Nikhil B. Ghayal, Dennis W. Dickson

Neuroscience

Research output: Contribution to journal › Article › peer-review

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.

Original language	English (US)
Journal	Acta neuropathologica
DOIs	https://doi.org/10.1007/s00401-022-02479-4
State	Published - Oct 2022

Keywords

Autopsy cohort
Criteria
Human
Neurofibrillary tangles
Neuropathology
Oligodendroglia
Phosphorylated tau
Progressive supranuclear palsy (PSP)
Threads
Tufted astrocytes

ASJC Scopus subject areas

Pathology and Forensic Medicine
Clinical Neurology
Cellular and Molecular Neuroscience

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10.1007/s00401-022-02479-4

Cite this

Roemer, S. F., Grinberg, L. T., Crary, J. F., Seeley, W. W., McKee, A. C., Kovacs, G. G., Beach, T. G., Duyckaerts, C., Ferrer, I. A., Gelpi, E., Lee, E. B., Revesz, T., White, C. L., Yoshida, M., Pereira, F. L., Whitney, K., Ghayal, N. B., & Dickson, D. W. (2022). Rainwater Charitable Foundation criteria for the neuropathologic diagnosis progressive supranuclear palsy. Acta neuropathologica. https://doi.org/10.1007/s00401-022-02479-4

Roemer, SF, Grinberg, LT, Crary, JF, Seeley, WW, McKee, AC, Kovacs, GG, Beach, TG, Duyckaerts, C, Ferrer, IA, Gelpi, E, Lee, EB, Revesz, T, White, CL, Yoshida, M, Pereira, FL, Whitney, K, Ghayal, NB & Dickson, DW 2022, 'Rainwater Charitable Foundation criteria for the neuropathologic diagnosis progressive supranuclear palsy', Acta neuropathologica. https://doi.org/10.1007/s00401-022-02479-4

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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 = "Publisher Copyright: {\textcopyright} 2022, The Author(s).",

year = "2022",

month = oct,

doi = "10.1007/s00401-022-02479-4",

language = "English (US)",

journal = "Acta neuropathologica",

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T1 - Rainwater Charitable Foundation criteria for the neuropathologic diagnosis progressive supranuclear palsy

AU - Roemer, Shanu F.

AU - Grinberg, Lea T.

AU - Crary, John F.

AU - Seeley, William W.

AU - McKee, Ann C.

AU - Kovacs, Gabor G.

AU - Beach, Thomas G.

AU - Duyckaerts, Charles

AU - Ferrer, Isidro A.

AU - Gelpi, Ellen

AU - Lee, Edward B.

AU - Revesz, Tamas

AU - White, Charles L.

AU - Yoshida, Mari

AU - Pereira, Felipe L.

AU - Whitney, Kristen

AU - Ghayal, Nikhil B.

AU - Dickson, Dennis W.

PY - 2022/10

Y1 - 2022/10

N2 - 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.

AB - 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.

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KW - Criteria

KW - Human

KW - Neurofibrillary tangles

KW - Neuropathology

KW - Oligodendroglia

KW - Phosphorylated tau

KW - Progressive supranuclear palsy (PSP)

KW - Threads

KW - Tufted astrocytes

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DO - 10.1007/s00401-022-02479-4

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SN - 0001-6322

JO - Acta neuropathologica

JF - Acta neuropathologica

ER -

Rainwater Charitable Foundation criteria for the neuropathologic diagnosis progressive supranuclear palsy

Abstract

Keywords

ASJC Scopus subject areas

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