ArticleEuropean journal of neurology2022
Protracted course progressive supranuclear palsy.
Article in European journal of neurology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers.
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Who cites it
10 citing papers in PubMed, 20 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
- Deciphering Freezing of Gait: What Neuropathology Reveals About an Episodic Phenomenon.The European journal of neuroscience · 2026Review
- Comorbid pathologies and their impact on progressive supranuclear palsy: current view.Journal of neural transmission (Vienna, Austria : 1996) · 2026Review
- Amyloid and Tau Co-pathology in Parkinson Disease and Atypical Parkinsonism.Current neurology and neuroscience reports · 2026Review
- Refining a mouse model of progressive supranuclear palsy through inoculation of human post-mortem brain-derived tau.BMC research notes · 2026Article
- 4R-tau seeding activity reveals molecular subtypes in progressive supranuclear palsy.Nature communications · 2025Article
- Genetic forms of tauopathies: inherited causes and implications of Alzheimer's disease-like TAU pathology in primary and secondary tauopathies.Journal of neurology · 2024Review
- Pathomechanisms of cognitive impairment in progressive supranuclear palsy.Journal of neural transmission (Vienna, Austria : 1996) · 2023Review
- Uncovering spatiotemporal patterns of atrophy in progressive supranuclear palsy using unsupervised machine learning.Brain communications · 2023Article
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Authors and funding
13 authors at 4 institutions in 2 countries.
Funding
Abstract
background and purposeProgressive supranuclear palsy (PSP) encompasses a broader range of disease courses than previously appreciated. The most frequent clinical presentations of PSP are Richardson syndrome (RS) and PSP with a predominant Parkinsonism phenotype (PSP-P). Time to reach gait dependence and cognitive impairment have been proposed as prognostic disease milestones. Genetic polymorphisms in TRIM11 and SLC2A13 genes have been associated with longer disease duration (DD).
methodsMethods used include retrospective chart review, genetic single nucleotide polymorphism analyses (in three cases), and neuropathology.
resultsWe identified four cases with long (>10-15 years) or very long (>15 years) DD. Stage 1 PSP tau pathology was present in two cases (one PSP-P and one undifferentiated phenotype), whereas pallidonigroluysian atrophy (PSP-RS) and Stage 4/6 (PSP-P) PSP pathology were found in the other two cases. Three cases were homozygous for the rs564309-C allele of the TRIM11 gene and the H1 MAPT haplotype. Two were heterozygous for rs2242367 (G/A) in SLC2A13, whereas the third was homozygous for the G-allele.
conclusionsWe propose a protracted course subtype of PSP (PC-PSP) based on clinical or neuropathological criteria in two cases with anatomically restricted PSP pathology, and very long DD and slower clinical progression in the other two cases. The presence of the rs564309-C allele may influence the protracted disease course. Crystallizing the concept of PC-PSP is important to further understand the pathobiology of tauopathies in line with current hypotheses of protein misfolding, seeding activity, and propagation.
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