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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.
GLOSSARY
- AWH=
- arterial wall hyperfluorescence;
- BRAO=
- branch retinal artery occlusion;
- EDSS=
- Expanded Disability Status Scale;
- ETDRS=
- Early Treatment Diabetic Retinopathy Study;
- FA=
- fluorescein angiography;
- GCL=
- ganglion cell layer;
- GEE=
- generalized estimation equation;
- HC=
- healthy controls;
- HON=
- history of optic neuritis;
- ICC=
- intraclass correlation coefficient;
- INL=
- inner nuclear layer;
- IPL=
- inner plexiform layer;
- mRNFL=
- macular retinal nerve fiber layer;
- MS=
- multiple sclerosis;
- noHON=
- no history of optic neuritis;
- OCT=
- optical coherence tomography;
- ONL=
- outer nuclear layer;
- OPL=
- outer plexiform layer;
- PRL=
- photoreceptor layers;
- pRNFL=
- peripapillary retinal nerve fiber layer;
- RRMS=
- relapsing-remitting multiple sclerosis;
- SD-OCT=
- spectral-domain optical coherence tomography;
- SuS=
- Susac syndrome;
- TMV=
- total macular volume
Footnotes
↵* These authors contributed equally to this work.
Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
Supplemental data at Neurology.org
- Received November 14, 2014.
- Accepted in final form April 24, 2015.
- © 2015 American Academy of Neurology
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