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Azygos Vein Stenosis in Frontotemporal Dementia Sagging Brain Syndrome
Wouter I. Schievink, M. Marcel Maya, Rola Saouaf, HERBERT LIPSHUTZ, Rachelle B. Taché, Daniel J. Scoffings, Jeremy Dan Schmahmann. American Journal of Neuroradiology, 2025-01-23
Topic: Brain proteins, microglia and lab models
The mean age of the 3 women and 18 men was 50 years (range, 26-68 years). Seven patients were found to have venous stenosis. Endovascular stent placement of moderate to high-grade azygos vein stenosis in 3 patients resulted in prompt and remarkable improvement of symptoms in 2 patients (SIHDAS: very severe disability to no or mild disability) and mild improvement in 1 patient (SIHDAS: very severe disability to severe disability). Treatment of internal jugular vein and inferior vena cava stenosis in 2 patients each did not result in any improvement of symptoms. Endovascular (5 patients) or surgical (2 patients) interruption of multiple epidural spinal venous pathways did not result in any clinical improvement. The azygos vein is the main conduit between the spinal CSF space and the systemic venous circulation and this study demonstrates that isolated azygos vein stenosis may be a cause of spinal CSF loss and sagging brain syndrome. In this study, the yield of finding a clinically important treatable venous lesion among patients with recalcitrant bvFTD sagging brain syndrome was relatively low (15%). However, high quality and safe noninvasive imaging of the systemic venous system is available, eg, MR-venography, and should be considered for those patients who have exhausted treatments for this devastating condition, focusing on the azygos system.
Cite this study APA, Vancouver, BibTeX · download .bib or .ris
APA 7th
Schievink, W. I., Maya, M. M., Saouaf, R., LIPSHUTZ, H., Taché, R. B., Scoffings, D. J., et al. (2025). Azygos Vein Stenosis in Frontotemporal Dementia Sagging Brain Syndrome. American Journal of Neuroradiology. https://doi.org/10.3174/ajnr.a8532
Vancouver
Schievink WI, Maya MM, Saouaf R, LIPSHUTZ H, Taché RB, Scoffings DJ, et al. Azygos Vein Stenosis in Frontotemporal Dementia Sagging Brain Syndrome. American Journal of Neuroradiology. 2025. doi:10.3174/ajnr.a8532. PMID: 39848780.
BibTeX
@article{schievink2025-39848780,
author = {Schievink, Wouter I. and Maya, M. Marcel and Saouaf, Rola and LIPSHUTZ, HERBERT and Taché, Rachelle B. and Scoffings, Daniel J. and Schmahmann, Jeremy Dan},
title = {{Azygos Vein Stenosis in Frontotemporal Dementia Sagging Brain Syndrome}},
journal = {American Journal of Neuroradiology},
year = {2025},
doi = {10.3174/ajnr.a8532},
pmid = {39848780},
url = {https://doi.org/10.3174/ajnr.a8532}
}
Built from the record alzhub holds for this paper, not looked up. Volume, issue and page numbers are not held in the record. The record names 7 authors; APA and Vancouver show the first six, BibTeX and the downloads carry all of them. Check the citation against the original before you use it.
Key findings
- The mean age of the 3 women and 18 men was 50 years (range, 26-68 years).
- Seven patients were found to have venous stenosis.
- Endovascular stent placement of moderate to high-grade azygos vein stenosis in 3 patients resulted in prompt and remarkable improvement of symptoms in 2 patients (SIHDAS: very severe disability to no or mild disability) and mild improvement in 1 patient (SIHDAS: very severe disability to severe disability).
- Treatment of internal jugular vein and inferior vena cava stenosis in 2 patients each did not result in any improvement of symptoms.
- Endovascular (5 patients) or surgical (2 patients) interruption of multiple epidural spinal venous pathways did not result in any clinical improvement.
Why it matters
In this small study of 21 patients, endovascular stent placement of moderate to high-grade azygos vein stenosis resulted in improvement of symptoms in some patients, but the yield of finding a clinically important treatable venous lesion was relatively low at 15%. This is a small, preliminary study and does not establish a treatment, cure or prevention for frontotemporal dementia.
Study details
- Evidence strengthLow
How this study scored 41/100
Published in a peer-reviewed 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 | 7 / 10 | Tier 3 — peer-reviewed journal (American Journal of Neuroradiology) |
| Journal's citation record | 2 / 4 | American Journal of Neuroradiology: journal h-index 182 (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 | 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 | 1 / 7 | Cited 3 times (OpenAlex, 2026-10-10) |
| Citations per year | 1 / 5 | 1.0 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 | 0 / 6 | No other study in this library cites it |
| Still being cited | 1 / 4 | Cited 3 times in 2024–2026 (2026 still in progress; OpenAlex) |
| Senior author's track record | 3 / 4 | Last-listed author Jeremy Dan Schmahmann: h-index 89 (OpenAlex, whole career) |
| Institution's track record | 3 / 3 | Harvard University: institution h-index 3,235 (OpenAlex) |
| Multi-centre collaboration | 2 / 3 | OpenAlex lists 4 institution(s) in 2 countries |
| Indexed in PubMed | 2 / 2 | Indexed in PubMed (PMID 39848780) |
| Free to read | 2 / 2 | Free to read |
| Total | 41 / 100 | — |
What each of these 20 components measures, and what this score is not →
Read the original
- DOI: 10.3174/ajnr.a8532
- PubMed: 39848780
- Original source →
- Free full text: American Journal of Neuroradiology →
Full text: free to read — OpenAlex lists this copy as free to read: the link above (checked 6 Feb 1020).
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