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.

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: Marius Ringelstein, Philipp Albrecht, Ilka Kleffner, Björn Bühn, Jens Harmel, Ann-Kristin Müller, David Finis, Rainer Guthoff, Richard Bergholz, Thomas Duning, Markus Krämer, Friedemann Paul, Alexander Brandt, Timm Oberwahrenbrock, Janine Mikolajczak, Brigitte Wildemann, Sven Jarius, Hans-Peter Hartung, Orhan Aktas, Jan Dörr
• Journal: Neurology (Neurology) Vol. 85 Issue 7 Pg. 610-8 (Aug 18 2015) ISSN: 1526-632X [Electronic] United States
• PMID: 26203089 (Publication Type: Journal Article, Multicenter Study, Research Support, Non-U.S. Gov't)
• Copyright: © 2015 American Academy of Neurology.

Topics

• Adult
• Female
• Fluorescein Angiography
• Humans
• Male
• Middle Aged
• Multiple Sclerosis, Relapsing-Remitting (diagnosis)
• Retinal Diseases (diagnosis, etiology)
• Retinal Neurons (pathology)
• Retinal Vessels (pathology)
• Susac Syndrome (complications, diagnosis)
• Tomography, Optical Coherence