Marius Ringelstein1, Philipp Albrecht1, Ilka Kleffner1, Björn Bühn1, Jens Harmel1, Ann-Kristin Müller1, David Finis1, Rainer Guthoff1, Richard Bergholz1, Thomas Duning1, Markus Krämer1, Friedemann Paul1, Alexander Brandt1, Timm Oberwahrenbrock1, Janine Mikolajczak1, Brigitte Wildemann1, Sven Jarius1, Hans-Peter Hartung1, Orhan Aktas2, Jan Dörr1. 1. 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 Neurology (M.K.), Alfried Krupp Hospital, Essen; and Molecular Neuroimmunology (B.W., S.J.), Department of Neurology, University of Heidelberg, Germany. 2. 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 Neurology (M.K.), Alfried Krupp Hospital, Essen; and Molecular Neuroimmunology (B.W., S.J.), Department of Neurology, University of Heidelberg, Germany. orhan.aktas@uni-duesseldorf.de.
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.
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.
Authors: Kay Gawlik; Frank Hausser; Friedemann Paul; Alexander U Brandt; Ella Maria Kadas Journal: Biomed Opt Express Date: 2018-11-28 Impact factor: 3.732
Authors: Philipp Albrecht; Christine Blasberg; Marius Ringelstein; Ann-Kristin Müller; David Finis; Rainer Guthoff; Ella-Maria Kadas; Wolf Lagreze; Orhan Aktas; Hans-Peter Hartung; Friedemann Paul; Alexander U Brandt; Axel Methner Journal: J Neurol Date: 2017-06-05 Impact factor: 4.849
Authors: Andrés Cruz-Herranz; Lisanne J Balk; Timm Oberwahrenbrock; Shiv Saidha; Elena H Martinez-Lapiscina; Wolf A Lagreze; Joel S Schuman; Pablo Villoslada; Peter Calabresi; Laura Balcer; Axel Petzold; Ari J Green; Friedemann Paul; Alexander U Brandt; Philipp Albrecht Journal: Neurology Date: 2016-05-25 Impact factor: 9.910