title: Retinal pathology in Susac syndrome detected by spectral-domain optical coherence tomography.
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.
  
  
  CONCLUSIONS
  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.affiliation: 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.
authors.forename: Marius
authors.initials: M
authors.lastname: Ringelstein
authors.affiliation: 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.
authors.forename: Philipp
authors.initials: P
authors.lastname: Albrecht
authors.affiliation: 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.
authors.forename: Ilka
authors.initials: I
authors.lastname: Kleffner
authors.affiliation: 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.
authors.forename: Björn
authors.initials: B
authors.lastname: Bühn
authors.affiliation: 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.
authors.forename: Jens
authors.initials: J
authors.lastname: Harmel
authors.affiliation: 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.
authors.forename: Ann-Kristin
authors.initials: AK
authors.lastname: Müller
authors.affiliation: 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.
authors.forename: David
authors.initials: D
authors.lastname: Finis
authors.affiliation: 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.
authors.forename: Rainer
authors.initials: R
authors.lastname: Guthoff
authors.affiliation: 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.
authors.forename: Richard
authors.initials: R
authors.lastname: Bergholz
authors.affiliation: 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.
authors.forename: Thomas
authors.initials: T
authors.lastname: Duning
authors.affiliation: 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.
authors.forename: Markus
authors.initials: M
authors.lastname: Krämer
authors.affiliation: 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.
authors.forename: Friedemann
authors.initials: F
authors.lastname: Paul
authors.affiliation: 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.
authors.forename: Alexander
authors.initials: A
authors.lastname: Brandt
authors.affiliation: 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.
authors.forename: Timm
authors.initials: T
authors.lastname: Oberwahrenbrock
authors.affiliation: 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.
authors.forename: Janine
authors.initials: J
authors.lastname: Mikolajczak
authors.affiliation: 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.
authors.forename: Brigitte
authors.initials: B
authors.lastname: Wildemann
authors.affiliation: 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.
authors.forename: Sven
authors.initials: S
authors.lastname: Jarius
authors.affiliation: 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.
authors.forename: Hans-Peter
authors.initials: HP
authors.lastname: Hartung
authors.affiliation: 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.
authors.forename: Orhan
authors.initials: O
authors.lastname: Aktas
authors.affiliation: 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.
authors.forename: Jan
authors.initials: J
authors.lastname: Dörr
mesh_terms: D000328:Adult; D005260:Female; D005451:Fluorescein Angiography; D006801:Humans; D008297:Male; D008875:Middle Aged; D020529:Multiple Sclerosis, Relapsing-Remitting / Q000175:diagnosis; D012164:Retinal Diseases / Q000175:diagnosis* / Q000209:etiology; D055351:Retinal Neurons / Q000473:pathology*; D012171:Retinal Vessels / Q000473:pathology*; D055955:Susac Syndrome / Q000150:complications / Q000175:diagnosis*; D041623:Tomography, Optical Coherence