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Cited by 65Ocular Diseases and Behçet’s SyndromeRetinal and Optic ConditionsMultiple Sclerosis Research Studies

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Retinal pathology in Susac syndrome detected by spectral-domain optical coherence tomography

Ringelstein, Marius, Albrecht, Philipp, Kleffner, Ilka, Bühn, Björn, Harmel, Jens, Müller, Ann-Kristin, Finis, David, Guthoff, Rainer, Bergholz, Richard, Duning, Thomas, Krämer, Markus, Paul, Friedemann, Brandt, Alexander, Oberwahrenbrock, Timm, Mikolajczak, Janine, Wildemann, Brigitte, Jarius, Sven, Hartung, Hans-Peter, Aktas, Orhan, Dörr, Jan·2015·Neurology
DOI 10.1212/wnl.0000000000001852
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Abstract

Objective The aim of this non-interventional study was to characterize retinal layer pathology in Susac syndrome (SuS), a disease with presumably autoimmune-mediated microvessel occlusions in the retina, brain, and inner ear, in comparison to the most important differential diagnosis multiple sclerosis (MS). Methods Seventeen patients with SuS and 17 age- and sex-matched patients with relapsing-remitting MS (RRMS) and healthy controls (HC) were prospectively investigated by spectral-domain optical coherence tomography (OCT) including intraretinal layer segmentation in a multicenter study. Patients with SuS additionally received retinal fluorescein angiography (FA) and automated perimetry. Results Patchy thinning of the retinal nerve fiber layer, ganglion cell layer, inner plexiform layer, inner nuclear layer, and outer plexiform layer compared to corresponding sectors in RRMS and HC eyes (p Conclusion Distinct OCT patterns of scattered, scar-like intraretinal pathology in SuS eyes, sparing the ONL and PRL, suggest a retinal, but not choroidal, vascular pathomechanism and clearly differentiate SuS from RRMS. Depending on the disease stage, OCT and FA provide specific complementary diagnostic information in SuS.

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Metadata

Authors
Ringelstein, M., Albrecht, P., Kleffner, I., Bühn, B., Harmel, J., Müller, A.-K., Finis, D., Guthoff, R., Bergholz, R., Duning, T., Krämer, M., Paul, F., Brandt, A., Oberwahrenbrock, T., Mikolajczak, J., Wildemann, B., Jarius, S., Hartung, H.-P., Aktas, O., & Dörr, J.
Affiliations
  • University of Münster (DE)
  • From the Departments of Neurology (M.R., P.A., B.B., J.H., A.-K.M., H.-P.H., O.A.) and Ophthalmology (D.F., R.G.), Medical Faculty, Heinrich-Heine University Düsseldorf; the Department of Neurology (I.K., T.D.), University of Münster; the Department of Ophthalmology (R.B.), NeuroCure Clinical Research Center (F.P., A.B., T.O., J.M., J.D.), and Clinical and Experimental Multiple Sclerosis Research Center, Department of Neurology (F.P., J.D.), Charité-Universitätsmedizin Berlin; the Department of...
ORCID
Venue
Neurology
Publisher
Ovid Technologies (Wolters Kluwer Health)
Published
2015-08-18
Volume
85
Issue
7
Pages
610-618
ISSN
  • 0028-3878 (ISSN-L)
  • 1526-632X
DOI
10.1212/wnl.0000000000001852
OpenAlex
W1698564094
Cited by
65
References
1
Citations by year
2026: 4 · 2025: 1 · 2024: 5 · 2023: 2 · 2022: 6 · 2021: 7 · 2020: 9 · 2019: 7 · 2018: 7 · 2017: 7 · 2016: 8 · 2015: 2
FWCI
7.68
Type
journal article
Language
en
Open access
closed
Locations
  • Neurology (publishedVersion)
  • PubMed (publishedVersion)
  • MDC Repository (Max-Delbrueck-Center for Molecular Medicine) (submittedVersion)
Topics
  • Health Sciences › Medicine › Ophthalmology › Ocular Diseases and Behçet’s Syndrome
  • Health Sciences › Medicine › Ophthalmology › Retinal and Optic Conditions
  • Health Sciences › Medicine › Clinical Neurology › Multiple Sclerosis Research Studies
Keywords
Susac syndrome, retinal disease, retinal nerve fiber layer thinning, retinal layer segmentation, spectral-domain optical coherence tomography, relapsing-remitting multiple sclerosis, differential diagnosis, retinal fluorescein angiography, visual field defects, inner plexiform layer, inner nuclear layer
MeSH terms
Adult, Female, Fluorescein Angiography, Humans, Male, Middle Aged, Retinal Diseases, Retinal Vessels, Multiple Sclerosis, Relapsing-Remitting, Tomography, Optical Coherence, Retinal Neurons, Susac Syndrome

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CoChat. (2026). Citation check: Retinal pathology in Susac syndrome detected by spectral-domain optical coherence tomography. https://app.cochat.ai/papers/10.1212/wnl.0000000000001852
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Ringelstein, M., Albrecht, P., Kleffner, I., Bühn, B., Harmel, J., Müller, A.-K., Finis, D., Guthoff, R., Bergholz, R., Duning, T., Krämer, M., Paul, F., Brandt, A., Oberwahrenbrock, T., Mikolajczak, J., Wildemann, B., Jarius, S., Hartung, H.-P., Aktas, O., & Dörr, J. (2015). Retinal pathology in Susac syndrome detected by spectral-domain optical coherence tomography. Neurology. https://doi.org/10.1212/wnl.0000000000001852