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In vivo Structural Assessments of Ocular Disease in Rodent Models using Optical Coherence Tomography
Published on: July 24, 2020
Retinal pathology in Susac syndrome detected by spectral-domain optical coherence tomography
Marius Ringelstein1, Philipp Albrecht1, Ilka Kleffner1
1From 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 Neurology (M.K.), Alfried Krupp Hospital, Essen; and Molecular Neuroimmunology (B.W., S.J.), Department of Neurology, University of Heidelberg, Germany.
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.
