{
  "abstract": "Progressive supranuclear palsy (PSP) is an adult-onset neurodegenerative disease with a heterogeneous spectrum of clinical and pathological features. The epidemiology of PSP-as defined by the gold standard of autopsy diagnosis-remains unresolved, but prevalence estimates are typically ~1:10,000. Here, we analyzed the National Alzheimer's Coordinating Center (NACC) Neuropathology Data Set, aggregated from 38 U.S. Alzheimer's Disease Research Centers (ADRCs). We compared clinical and neuropathologic features of autopsied participants, stratifying on cognitive status at recruitment into the cohort. The sample comprised 6845 individuals who were followed longitudinally for 4.0 (± 2.9 stdev) approximately yearly visits, n = 297 with eventual autopsy-verified PSP. Participants were not necessarily diagnosed or followed in movement disorder clinics. Among those with dementia or PSP at recruitment (n = 4310), 3.3% had corticobasal degeneration (CBD), 3.0% Pick's disease, and 4.5% PSP pathology. Among those recruited while cognitively normal (n = 1451), 0.7% had CBD, and 0.1% Pick's disease, while 3.2% had PSP pathology. This frequency of incident PSP pathology was higher than expected based on prior epidemiology studies. In comparing between incident (recruited normal) and prevalent (recruited with dementia or clinical PSP) cases with PSP pathology, those with incident PSP died older: 89.5 years vs 76.3 years on average. Furthermore, incident PSP pathology cases were less likely to manifest stereotypical PSP clinical features, but more likely to have parkinsonism. In a convenience sample of individuals with PSP pathology from the University of Kentucky ADRC (n = 23), digital pathology analyses using HALO software and AI-based analytic modules revealed that PSP tau pathology was more severe overall in prevalent PSP. Within the putamen, incident cases had a higher proportion of tufted astrocytes and lower proportion of NFTs. In conclusion, in this large multicenter sample, PSP pathology was more common than other FTLD-Tau subtypes, and incident PSP often differed clinically from \"classic\" PSP.",
  "abstract_shown": true,
  "affiliations": [
    "Department of Pathology and Laboratory Medicine, University of Kentucky, Lexington, USA.",
    "Sanders-Brown Center on Aging, University of Kentucky, 800 S. Limestone Ave, Lexington, KY, 40536-0230, USA.",
    "Department of Environmental Health and Epidemiology, University of Kentucky, Lexington, USA.",
    "Department of Neurology, University of Kentucky, Lexington, USA.",
    "Department of Laboratory Medicine and Pathobiology at the University of Toronto, Toronto, Canada.",
    "Tanz Centre for Research in Neurodegenerative Disease, University of Toronto, Toronto, ON, Canada.",
    "Department of Pathology and Laboratory Medicine, University of Kentucky, Lexington, USA. peter.nelson@uky.edu.",
    "Sanders-Brown Center on Aging, University of Kentucky, 800 S. Limestone Ave, Lexington, KY, 40536-0230, USA. peter.nelson@uky.edu."
  ],
  "article_type": "research article",
  "arxiv": null,
  "author_total": 11,
  "authors": [
    {
      "affs": [
        0
      ],
      "name": "Benjamin J Cushing"
    },
    {
      "affs": [
        1
      ],
      "name": "Ryan K Shahidehpour"
    },
    {
      "affs": [
        1,
        2
      ],
      "name": "Erin L Abner"
    },
    {
      "affs": [
        1,
        3
      ],
      "name": "Gregory A Jicha"
    },
    {
      "affs": [
        0,
        1
      ],
      "name": "Janna M Neltner"
    },
    {
      "affs": [
        1
      ],
      "name": "Madeline K Breig"
    },
    {
      "affs": [
        1
      ],
      "name": "Kailen L Teodorescu"
    },
    {
      "affs": [
        1
      ],
      "name": "Allison M Neltner"
    },
    {
      "affs": [
        1,
        3
      ],
      "name": "E Pinar Coskun"
    },
    {
      "affs": [
        4,
        5
      ],
      "name": "Gabor G Kovacs"
    },
    {
      "affs": [
        6,
        7
      ],
      "name": "Peter T Nelson"
    }
  ],
  "bibtex": "https://research.draconitic.com/paper/11yd7610fp4jv.bib",
  "cited_by": 0,
  "doi": "10.1007/s00401-026-03100-8",
  "fulltext_url": "https://europepmc.org/articles/PMC13661695",
  "id": "11yd7610fp4jv",
  "issns": [
    "0001-6322",
    "1432-0533"
  ],
  "landing_url": "https://europepmc.org/article/MED/42867534",
  "language": "eng",
  "licence": "CC-BY-NC-ND",
  "licence_label": "CC BY-NC-ND",
  "licence_source": "article",
  "licence_url": "https://creativecommons.org/licenses/by-nc-nd/4.0/",
  "metadata_licence": null,
  "native_subjects": [
    [
      "openalex",
      "2738|Dementia and Cognitive Impairment Research"
    ]
  ],
  "openalex": "W7166690987",
  "pdf_url": "https://europepmc.org/articles/PMC13661695?pdf=render",
  "pmcid": "PMC13661695",
  "pmid": "42867534",
  "published": "2026-10-13",
  "publisher": null,
  "ris": "https://research.draconitic.com/paper/11yd7610fp4jv.ris",
  "sources": [
    {
      "first_seen": "2026-10-30T12:19:00Z",
      "native_id": "MED:42867534",
      "source": "europepmc",
      "url": "https://europepmc.org/article/MED/42867534"
    }
  ],
  "subjects": [
    {
      "field": "medicine",
      "origin": "openalex:2738|Dementia and Cognitive Impairment Research",
      "subfield": "psychiatry"
    }
  ],
  "title": "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.",
  "title_text": "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.",
  "type": "peer-reviewed",
  "updated_at": "2026-10-30T12:19:00Z",
  "url": "https://research.draconitic.com/paper/11yd7610fp4jv",
  "venue": "Acta neuropathologica",
  "versions": []
}