Home › Research › Risk factors
Susac's Syndrome--update
Rennebohm R, Susac JO, Egan RA, Daroff RB.. Journal of the neurological sciences, 2010-09-19
Topic: Inflammation, microglia and astrocytes
Susac's Syndrome (SS) consists of the clinical triad of encephalopathy, branch retinal artery occlusion (BRAO), and hearing loss (HL). It is an autoimmune endotheliopathy affecting the precapillary arterioles of the brain, retina, and inner ear (cochlea and semicircular canals). The age range extends from 7 to 72 years, but young women (20-40) are most vulnerable. Headache routinely accompanies the encephalopathy and may be constant (best explained by leptomeningeal involvement), migrainous, or both. Multifocal neurological manifestations--particularly bilateral long-tract signs--commonly accompany the encephalopathy, which is laden with psychiatric features, confusion, memory loss and other cognitive changes. Left untreated, dementia can ensue. SS has an unexplained proclivity for attacking the central corpus callosum. In its encephalopathic form, pathognomonic callosal lesions permit an immediate diagnosis. We believe that the diagnosis of SS can be made when only the encephalopathy and pathognomonic MRI lesions are present; the BRAO and HL need not be present. We have also found the "string of pearls" MRI finding--the studding of the internal capsules with microinfarcts--to be most helpful--if not pathognomonic. This sign is always associated with the clusters of corpus callosum lesions, is especially striking on diffusion weighted imaging, and is associated with long-tract findings. We discuss the newly appreciated BRAO subset of SS and offer preliminary treatment suggestions for this subset. We also call attention to our development of an International Collaborative Study of SS and an educational website (http://www.ucalgary.ca/susac).
Key findings
- This sign is always associated with the clusters of corpus callosum lesions, is especially striking on diffusion weighted imaging, and is associated with long-tract findings.
New to this subject? Start with our plain-English guide: Risk factors, ranked →
Study details
- Evidence strengthLow
How this study scored 36/100
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. We have no data on journal's citation record, times cited, citations per year, impact for its field and year, cited by studies here, still being cited, senior author's track record, institution's track record and multi-centre collaboration, so those were left out: it earned 21 of the 58 points we could judge it on, which is 36 out of 100.
| Component | Points | Why |
|---|---|---|
| Journal tier | 7 / 10 | Tier 3 — peer-reviewed journal (Journal of the neurological sciences) |
| Journal's citation record | — / 4 | No journal record from OpenAlex — left out of this score |
| 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 | 2010 — 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 | — / 7 | No citation count (not found in OpenAlex) — left out of this score |
| Citations per year | — / 5 | No citation count or no year — left out of this score |
| Impact for its field and year | — / 6 | No field-weighted citation impact (OpenAlex has none yet) — left out of this score |
| Cited by studies here | — / 6 | Not yet checked against this library's citation graph — left out of this score |
| Still being cited | — / 4 | No yearly citation counts — left out of this score |
| Senior author's track record | — / 4 | Senior author's record not available — left out of this score |
| Institution's track record | — / 3 | No institution listed in OpenAlex — left out of this score |
| Multi-centre collaboration | — / 3 | Institutions not listed — left out of this score |
| Indexed in PubMed | 2 / 2 | Indexed in PubMed (PMID 20855088) |
| Free to read | 0 / 2 | Behind a paywall — scores zero |
| Points | 21 / 58 | Scored on 11 of 20 parts. We have no data for the rest, so they are left out: 21 of the 58 points we could judge it on is 36 out of 100. |
| Total | 36 / 100 | — |
What each of these 20 components measures, and what this score is not →
Read the original
Free copy unknown: no free-copy check has an answer for this record yet. That does not mean it is paywalled.
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
Rennebohm, R., Susac, J. O., Egan, R. A., & Daroff, R. B. (2010). Susac's Syndrome--update. Journal of the neurological sciences. https://doi.org/10.1016/j.jns.2010.08.032
Vancouver
Rennebohm R, Susac JO, Egan RA, Daroff RB. Susac's Syndrome--update. Journal of the neurological sciences. 2010. doi:10.1016/j.jns.2010.08.032. PMID: 20855088.
BibTeX
@article{rennebohm2010-20855088,
author = {Rennebohm, R. and Susac, J. O. and Egan, R. A. and Daroff, R. B.},
title = {{Susac's Syndrome--update}},
journal = {Journal of the neurological sciences},
year = {2010},
doi = {10.1016/j.jns.2010.08.032},
pmid = {20855088},
url = {https://doi.org/10.1016/j.jns.2010.08.032}
}
Related studies in Risk factors
Also tagged “hydrocephalus”
73 studies in the library carry the topic tag “hydrocephalus”; these are this study's neighbours in a fixed, alphabetical-by-hash tour of them, not the closest in content. All 73 tagged studies.