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Autopsy-proven progressive supranuclear palsy presenting as behavioral variant frontotemporal dementia
Hassan A, Parisi JE, Josephs KA.. Neurocase, 2011-12-20
Topic: Brain proteins, microglia and lab models
Background Progressive supranuclear palsy (PSP) is a neurodegenerative disorder pathologically characterized by neuronal loss, gliosis and tau-positive neurofibrillary tangles in basal ganglia, brainstem and cerebellar nuclei. Five presenting clinical syndromes of PSP are well-described: (i) the classic Richardson's syndrome; (ii) asymmetric parkinsonism with tremor; (iii) freezing of gait; (iv) asymmetric limb apraxia, and (v) apraxia of speech. Aim To determine whether autopsy-proven PSP cases may present with another clinical phenotype. Methods Medical records of 66 autopsy-proven PSP cases between 1973 and 2010 were reviewed to determine whether all could be classified into one of five well-defined syndromes listed above. Three cases presented with prominent behavioral and personality changes, meeting diagnosis of behavioral variant frontotemporal dementia. MRI midbrain and pons volumes and pons/midbrain ratios were compared to healthy controls and typical PSP cases. Results All three bvFTD cases developed at least one PSP symptom or sign that emerged up to 5 years after initial presentation. One case was re-diagnosed as PSP 6 years after presentation as bvFTD. Compared to controls, midbrain volume was significantly smaller in both bvFTD (p = .03) and PSP cases (p = .008), without significant difference between PSP and bvFTD cases (.44). However pontine volumes were similar across all three groups. Conclusions While most autopsy-confirmed PSP cases present with one of the five well-described syndromes, there are cases that may present as bvFTD. In these, at least one cardinal symptom or sign of PSP later emerges, associated with smaller midbrain volume and increased pons/midbrain ratio. Thus underlying PSP pathology should be considered in these cases.
Key findings
- MRI midbrain and pons volumes and pons/midbrain ratios were compared to healthy controls and typical PSP cases.
- Results All three bvFTD cases developed at least one PSP symptom or sign that emerged up to 5 years after initial presentation.
- Compared to controls, midbrain volume was significantly smaller in both bvFTD (p = .03) and PSP cases (p = .008), without significant difference between PSP and bvFTD cases (.44).
- In these, at least one cardinal symptom or sign of PSP later emerges, associated with smaller midbrain volume and increased pons/midbrain ratio.
Why it matters
Three autopsy-proven cases of progressive supranuclear palsy initially presented as behavioral variant frontotemporal dementia, with PSP symptoms emerging up to 5 years later. This highlights diagnostic challenges and does not address treating, curing, or preventing Alzheimer's disease.
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Study details
- Evidence strengthLow
How this study scored 40/100
Published in a peer-reviewed journal. Scored zero for study design, sample size, effect reported and randomised / blinded because the record does not state it.
| Component | Points | Why |
|---|---|---|
| Journal tier | 7 / 10 | Tier 3 — peer-reviewed journal (Neurocase) |
| Journal's citation record | 1 / 4 | Neurocase: journal h-index 87 (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 | 2012 — 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 | 3 / 3 | Studied in people (pubmed indexes it as a clinical or observational study) |
| Randomised / blinded | 0 / 2 | No randomisation or blinding stated — scores zero (not stated in the record — scored 0) |
| Times cited | 4 / 7 | Cited 74 times (OpenAlex, 2026-10-10) |
| Citations per year | 2 / 5 | 4.6 citations a year over 16 year(s) since 2012 |
| Impact for its field and year | 1 / 6 | Cited 0.6× the average for papers of its field and year (OpenAlex FWCI) |
| Cited by studies here | 1 / 6 | Cited by 1 other studies in this library |
| Still being cited | 1 / 4 | Cited 10 times in 2024–2026 (2026 still in progress; OpenAlex) |
| Senior author's track record | 4 / 4 | Last-listed author Keith A. Josephs: h-index 119 (OpenAlex, whole career) |
| Institution's track record | 2 / 3 | Mayo Clinic: institution h-index 1,606 (OpenAlex) |
| Multi-centre collaboration | 0 / 3 | OpenAlex lists 1 institution(s) in 1 country — scores zero (affiliation lists can be incomplete) |
| Indexed in PubMed | 2 / 2 | Indexed in PubMed (PMID 22181323) |
| Free to read | 0 / 2 | Behind a paywall — scores zero |
| Total | 40 / 100 | — |
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Cite this study APA, Vancouver, BibTeX · download .bib or .ris
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
Hassan, A., Parisi, J. E., & Josephs, K. A. (2012). Autopsy-proven progressive supranuclear palsy presenting as behavioral variant frontotemporal dementia. Neurocase. https://doi.org/10.1080/13554794.2011.627345
Vancouver
Hassan A, Parisi JE, Josephs KA. Autopsy-proven progressive supranuclear palsy presenting as behavioral variant frontotemporal dementia. Neurocase. 2012. doi:10.1080/13554794.2011.627345. PMID: 22181323.
BibTeX
@article{hassan2012-22181323,
author = {Hassan, A. and Parisi, J. E. and Josephs, K. A.},
title = {{Autopsy-proven progressive supranuclear palsy presenting as behavioral variant frontotemporal dementia}},
journal = {Neurocase},
year = {2012},
doi = {10.1080/13554794.2011.627345},
pmid = {22181323},
url = {https://doi.org/10.1080/13554794.2011.627345}
}
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