Retinal pathology in Susac syndrome detected by spectral-domain optical coherence tomography

Abstract

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.

Diagnostic criteria for Susac syndrome

“…To meet the ‘retinal involvement criterion’, at least one of the following findings should be documented in the ophthalmological examination: (1) at least one acute BRAO in FLA or funduscopy, (2) focal segmental staining of the arterial wall (arterial wall hyperfluorescence, AWH) near the site of obstruction (figure 2) in FLA,10 or (3) sectorial damage of the inner retinal layers from the retinal nerve fibre layer through to the outer plexiform layer in optical coherence tomography (OCT) 11. AWH in patients with SuS indicates the impaired integrity of the retinal vessel wall, has to be differentiated from fluorescein leakage, can be considered as a marker for acute disease activity and has not been demonstrated in other retinal vasculitides 10.…”

“…In particular, peripheral retinal arterial wall plaques are typical for SuS, but they are often transient 2. Spectral domain OCT (SD-OCT) recently turned out to reliably detect highly disease-specific retinal pathologies, rendering it a seminal tool for retinal involvement analyses in later disease stages, when there may be no more BRAO and AWH detectable by FLA 11 12…”

Optical coherence tomography in multiple sclerosis

“…Furthermore, the distribution of RNFL loss tends to involve the temporal quadrant in MSON, whereas it is more diffusely distributed in neuromyelitis optica spectrum disorder [6]. In Susac syndrome, the pattern of GCIPL and RNFL loss may help differentiate it from MS [7,8]. These observations can be found in patients long after their branch retinal arterial occlusion, which makes OCT more useful than fluorescein angiography, which will only detect a branch retinal arterial occlusion in the acute phase [8].…”

“…In Susac syndrome, the pattern of GCIPL and RNFL loss may help differentiate it from MS [7,8]. These observations can be found in patients long after their branch retinal arterial occlusion, which makes OCT more useful than fluorescein angiography, which will only detect a branch retinal arterial occlusion in the acute phase [8]. In idiopathic intracranial hypertension, there is also swelling of the optic nerve head.…”

Whole-body positional manipulators for ocular imaging of anaesthetised mice and rats: a do-it-yourself guide

“…In recent years, in vivo retinal imaging has gained increasing relevance 1-3 not only in ophthalmological [4][5][6][7][8] but also in neurological [9][10][11][12][13][14][15][16][17][18][19] cases. Optical coherence tomography (OCT) and confocal scanning laser ophthalmoscopy (cSLO) have been identified as useful diagnostic tools to evaluate a large variety of retinopathies and retinal manifestations of neurological diseases.…”