ArticleActa neuropathologica2022
Rainwater Charitable Foundation criteria for the neuropathologic diagnosis of progressive supranuclear palsy.
Article in Acta neuropathologica, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 84 papers.
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84 citing papers in PubMed, 130 citations in OpenAlex.
- Incident versus prevalent progressive supranuclear palsy: analyses of U.S. Alzheimer's Disease Research Centers data indicate clinical and neuropathological features of a relatively common and underrecognized tauopathy in the aging brain.Acta neuropathologica · 2026Article
- Data-driven modelling of tau pathology reveals distinct progressive supranuclear palsy subtypes.Brain : a journal of neurology · 2026Article
- Tau-associated neuronal loss in the intermediate nucleus of the human hypothalamus, a putative VLPO analog, in progressive supranuclear palsy and Alzheimer's disease.Alzheimer's & dementia : the journal of the Alzheimer's Association · 2026Article
- Regional mapping of CSF1R-positive microglia in neurodegenerative diseases and progressive MS, with exploratory presynaptic marker analyses.Journal of neuroinflammation · 2026Article
- Neuropathology in a diverse cohort of oldest-old: The LifeAfter90 study.Alzheimer's & dementia : the journal of the Alzheimer's Association · 2026Article
- Article
- CTE has multiple pathologic variants that might relate to different clinical symptom presentation.Acta neuropathologica · 2026Article
- Synucleinopathy-like clinical features and biologically defined synuclein seeding in PSP-Parkinsonism: an imperfect match.NPJ Parkinson's disease · 2026Article
- Alzheimer's disease and mixed pathologies as a hidden contributor to fatal hypothermia: A large-scale forensic autopsy-based study.Brain pathology (Zurich, Switzerland) · 2026Article
- Dementia in Progressive Supranuclear Palsy: A Narrative Review.Neurology and therapy · 2026Review
- Incidence of altered proteins in the aging brain: Implications for biological diagnostic markers.Journal of Alzheimer's disease : JAD · 2026Article
- Quantitative Susceptibility Mapping and Iron Deposition in Autopsy-Confirmed Progressive Supranuclear Palsy.Movement disorders clinical practice · 2026Article
- The effect of astrocyte depletion and repopulation approaches in pathological condition of CNS.Acta neurologica Belgica · 2026Review
- Neuropathologic basis of quantitative susceptibility mapping in the substantia nigra: contributions of tau, pigmented neurons, and iron.Acta neuropathologica · 2026Article
- Dementia with Lewy bodies and additional progressive supranuclear palsy presenting with early postural instability and frequent falls: an autopsy case.BMC neurology · 2026Article
- From clinical phenotypes to molecular stratification: early differential diagnosis of four-repeat tauopathies.Frontiers in aging neuroscience · 2026Review
- Distinct neuroinflammatory profiles in progressive supranuclear palsy associated withBrain communications · 2026Article
- From clinical phenotypes to molecular precision: multimodal biomarkers for progressive supranuclear palsy.Frontiers in neuroscience · 2026Review
- Targeting protein aggregate co-pathologies in neurodegeneration: a viable therapeutic strategy?Molecular neurodegeneration advances · 2026Review
- Proteomic analysis of human chronic traumatic encephalopathy brain implicates proteasome and ribosome dysfunction in disease severity.Molecular neurodegeneration advances · 2026Article
24 more citing papers are in PubMed but not listed here.
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18 authors at 15 institutions in 8 countries.
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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.
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