Home › Research › Brain tissue and pathology
Frontotemporal Lobar degeneration with TDP-43 presenting as progressive supranuclear palsy syndrome
Aya Murakami, Shunsuke Koga, Hiroaki Sekiya, Masataka Nakamura, Yusuke Yakushiji, Dennis W. Dickson. Acta Neuropathologica Communications, 2025-07-09
Topic: Brain proteins, microglia and lab models
The most common clinical symptoms in FTLD-TDP-PSP were memory loss (100%) followed by parkinsonism (80%), early falls (60%), and behavioral variant FTD (60%). All patients with PSP-RS met the Movement Disorder Society's criteria for probable PSP, while only one FTLD-TDP-PSP met the probable PSP. Two of the five patients with FTLD-TDP-PSP had moderate or severe neuronal loss in the substantia nigra and one had moderate or severe neuronal loss in the putamen and globus pallidus. A small subset of patients with FTLD-TDP can, in rare instances, present with symptoms associated with PSP. Therefore, FTLD-TDP may be considered in differential diagnosis, especially in patients who do not meet the diagnostic criteria. Our findings emphasize the need for further clinical and biomarker studies of FTLD-TDP.
Key findings
- The most common clinical symptoms in FTLD-TDP-PSP were memory loss (100%) followed by parkinsonism (80%), early falls (60%), and behavioral variant FTD (60%).
- All patients with PSP-RS met the Movement Disorder Society's criteria for probable PSP, while only one FTLD-TDP-PSP met the probable PSP.
- Two of the five patients with FTLD-TDP-PSP had moderate or severe neuronal loss in the substantia nigra and one had moderate or severe neuronal loss in the putamen and globus pallidus.
- A small subset of patients with FTLD-TDP can, in rare instances, present with symptoms associated with PSP.
- Therefore, FTLD-TDP may be considered in differential diagnosis, especially in patients who do not meet the diagnostic criteria.
Why it matters
This study describes a small subset of patients with frontotemporal lobar degeneration with TDP-43 pathology who presented with symptoms resembling progressive supranuclear palsy, with memory loss in all five patients and parkinsonism in most. It is a small case series about diagnosis and does not test any treatment, cure or prevention.
Study details
- Participants270
- Evidence strengthLow
How this study scored 44/100
Published in a peer-reviewed journal. Scored zero for study design, effect reported and randomised / blinded because the record does not state it.
| Component | Points | Why |
|---|---|---|
| Journal tier | 7 / 10 | Tier 3 — peer-reviewed journal (Acta Neuropathologica Communications) |
| Journal's citation record | 1 / 4 | Acta Neuropathologica Communications: journal h-index 129 (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 | 4 / 5 | 2025 — within 3 year(s) of the newest study here |
| Sample size | 2 / 4 | 270 participants |
| 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 (participant count stated) |
| Randomised / blinded | 0 / 2 | No randomisation or blinding stated — scores zero (not stated in the record — scored 0) |
| Times cited | 1 / 7 | Cited 2 times (OpenAlex, 2026-10-10) |
| Citations per year | 0 / 5 | 0.7 citations a year over 3 year(s) since 2025 |
| Impact for its field and year | 2 / 6 | Cited 1.1× 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 | 0 / 4 | Cited 2 times in 2024–2026 (2026 still in progress; OpenAlex) |
| Senior author's track record | 4 / 4 | Last-listed author Dennis W. Dickson: h-index 218 (OpenAlex, whole career) |
| Institution's track record | 2 / 3 | University of Pennsylvania: institution h-index 1,859 (OpenAlex) |
| Multi-centre collaboration | 2 / 3 | OpenAlex lists 4 institution(s) in 2 countries |
| Indexed in PubMed | 2 / 2 | Indexed in PubMed (PMID 40635087) |
| Free to read | 2 / 2 | Free to read |
| Total | 44 / 100 | — |
What each of these 20 components measures, and what this score is not →
Read the original
- DOI: 10.1186/s40478-025-02058-0
- PubMed: 40635087
- Original source →
- Free full text: Acta Neuropathologica Communications →
Full text: free to read — OpenAlex lists this copy as free to read: the link above (checked 6 Feb 1020).
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
Murakami, A., Koga, S., Sekiya, H., Nakamura, M., Yakushiji, Y., & Dickson, D. W. (2025). Frontotemporal Lobar degeneration with TDP-43 presenting as progressive supranuclear palsy syndrome. Acta Neuropathologica Communications. https://doi.org/10.1186/s40478-025-02058-0
Vancouver
Murakami A, Koga S, Sekiya H, Nakamura M, Yakushiji Y, Dickson DW. Frontotemporal Lobar degeneration with TDP-43 presenting as progressive supranuclear palsy syndrome. Acta Neuropathologica Communications. 2025. doi:10.1186/s40478-025-02058-0. PMID: 40635087.
BibTeX
@article{murakami2025-40635087,
author = {Murakami, Aya and Koga, Shunsuke and Sekiya, Hiroaki and Nakamura, Masataka and Yakushiji, Yusuke and Dickson, Dennis W.},
title = {{Frontotemporal Lobar degeneration with TDP-43 presenting as progressive supranuclear palsy syndrome}},
journal = {Acta Neuropathologica Communications},
year = {2025},
doi = {10.1186/s40478-025-02058-0},
pmid = {40635087},
url = {https://doi.org/10.1186/s40478-025-02058-0}
}
Studies this one cites
Referenced by this study and also in the alzhub library, so you can read them here.
Later studies that cite this one
1 study in the alzhub library lists this one in its references.
Related studies in Brain tissue and pathology
Also tagged “bodies”
1,017 studies in the library carry the topic tag “bodies”; these are this study's neighbours in a fixed, alphabetical-by-hash tour of them, not the closest in content. All 1,017 tagged studies.