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Progressive supranuclear palsy: clinicopathological concepts and diagnostic challenges
Williams DR, Lees AJ.. The Lancet. Neurology, 2009-03-01
Topic: Risk, prevention and care in population studies
Progressive supranuclear palsy (PSP) is a clinical syndrome comprising supranuclear palsy, postural instability, and mild dementia. Neuropathologically, PSP is defined by the accumulation of neurofibrillary tangles. Since the first description of PSP in 1963, several distinct clinical syndromes have been described that are associated with PSP; this discovery challenges the traditional clinicopathological definition and complicates diagnosis in the absence of a reliable, disease-specific biomarker. We review the emerging nosology in this field and contrast the clinical and pathological characteristics of the different disease subgroups. These new insights emphasise that the pathological events and processes that lead to the accumulation of phosphorylated tau protein in the brain are best considered as dynamic processes that can develop at different rates, leading to different clinical phenomena. Moreover, for patients for whom the diagnosis is unclear, clinicians must continue to describe accurately the clinical picture of each individual, rather than label them with inaccurate diagnostic categories, such as atypical parkinsonism or PSP mimics. In this way, the development of the clinical features can be informative in assigning less common nosological categories that give clues to the underlying pathology and an understanding of the expected clinical course.
Key findings
- Since the first description of PSP in 1963, several distinct clinical syndromes have been described that are associated with PSP; this discovery challenges the traditional clinicopathological definition and complicates diagnosis in the absence of a reliable, disease-specific biomarker.
Why it matters
This review describes progressive supranuclear palsy, a condition defined neuropathologically by accumulation of neurofibrillary tangles, and notes that several distinct clinical syndromes are associated with it, complicating diagnosis in the absence of a reliable disease-specific biomarker. It concerns diagnosis and disease classification of progressive supranuclear palsy, not Alzheimer's disease treatment, cure or prevention.
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Study details
- Evidence strengthLow
How this study scored 54/100
Published in a leading specialty journal. Scored zero for study design, sample size, effect reported, studied in people and randomised / blinded because the record does not state it.
| Component | Points | Why |
|---|---|---|
| Journal tier | 9 / 10 | Tier 2 — leading specialty journal (The Lancet. Neurology) |
| Journal's citation record | 3 / 4 | The Lancet Neurology: journal h-index 457 (OpenAlex, 2026-10-29) |
| Study design | 0 / 12 | No study design recorded — scores zero, not guessed (not stated in the record — scored 0) |
| Strength of evidence | 1 / 5 | Rated “low” in our summary |
| Peer-review validation | 10 / 10 | Peer-reviewed. Published as an article in a named peer-reviewed journal. |
| How recent | 1 / 5 | 2009 — within 20 year(s) of the newest study here |
| Sample size | 0 / 4 | Participant count not stated — scores zero (not stated in the record — scored 0) |
| Effect reported | 0 / 3 | No effect size reported — scores zero (not stated in the record — scored 0) |
| Studied in people | 0 / 3 | Not stated whether this was studied in people — scores zero (not stated in the record — scored 0) |
| Randomised / blinded | 0 / 2 | No randomisation or blinding stated — scores zero (not stated in the record — scored 0) |
| Times cited | 6 / 7 | Cited 653 times (OpenAlex, 2026-10-10) |
| Citations per year | 4 / 5 | 34.4 citations a year over 19 year(s) since 2009 |
| Impact for its field and year | 5 / 6 | Cited 23.1× the average for papers of its field and year (OpenAlex FWCI) |
| Cited by studies here | 3 / 6 | Cited by 14 other studies in this library |
| Still being cited | 3 / 4 | Cited 90 times in 2024–2026 (2026 still in progress; OpenAlex) |
| Senior author's track record | 4 / 4 | Last-listed author Andrew John Lees: h-index 166 (OpenAlex, whole career) |
| Institution's track record | 2 / 3 | University College London: institution h-index 2,029 (OpenAlex) |
| Multi-centre collaboration | 1 / 3 | OpenAlex lists 3 institution(s) in 2 countries |
| Indexed in PubMed | 2 / 2 | Indexed in PubMed (PMID 19233037) |
| Free to read | 0 / 2 | Behind a paywall — scores zero |
| Total | 54 / 100 | — |
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Built from the record alzhub holds for this paper, not looked up. Volume, issue and page numbers are not held in the record. Check the citation against the original before you use it.
APA 7th
Williams, D. R., & Lees, A. J. (2009). Progressive supranuclear palsy: clinicopathological concepts and diagnostic challenges. The Lancet. Neurology. https://doi.org/10.1016/s1474-4422(09)70042-0
Vancouver
Williams DR, Lees AJ. Progressive supranuclear palsy: clinicopathological concepts and diagnostic challenges. The Lancet. Neurology. 2009. doi:10.1016/s1474-4422(09)70042-0. PMID: 19233037.
BibTeX
@article{williams2009-19233037,
author = {Williams, D. R. and Lees, A. J.},
title = {{Progressive supranuclear palsy: clinicopathological concepts and diagnostic challenges}},
journal = {The Lancet. Neurology},
year = {2009},
doi = {10.1016/s1474-4422(09)70042-0},
pmid = {19233037},
url = {https://doi.org/10.1016/s1474-4422(09)70042-0}
}
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