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

Item Type:Article
Title:Retinal pathology in Susac syndrome detected by spectral-domain optical coherence tomography
Creators Name:Ringelstein, M. and Albrecht, P. and Kleffner, I. and Bühn, B. and Harmel, J. and Müller, A.K. and Finis, D. and Guthoff, R. and Bergholz, R. and Duning, T. and Krämer, M. and Paul, F. and Brandt, A. and Oberwahrenbrock, T. and Mikolajczak, J. and Wildemann, B. and Jarius, S. and Hartung, H.P. and Aktas, O. and Dörr, J.
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 < 0.003 for SuS vs RRMS and HC) was observed in 23/34 (68%) SuS eyes, particularly in temporal quadrants. The outer nuclear layer (ONL) and photoreceptor layers (PRL) were not affected. FA performed in 15/17 patients with SuS was negative for disease-specific branch retinal artery occlusions in all but 1 eye at the time of OCT examination and revealed no additional vascular abnormalities, even in severely damaged OCT areas. In a subset of patients with SuS, associations of visual field data with distinct retinal layers were observed. 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.
Keywords:Fluorescein Angiography, Optical Coherence Tomography, Relapsing-Remitting Multiple Sclerosis, Retinal Diseases, Retinal Neurons, Retinal Vessels, Susac Syndrome
Publisher:Lippincott Williams & Wilkins
Page Range:610-618
Date:18 August 2015
Official Publication:https://doi.org/10.1212/WNL.0000000000001852
PubMed:View item in PubMed

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