Abstract Details

Title Optical Coherence Tomography in Susac's Syndrome

Topic MS and Related Diseases

Presentation(s) P02 - (-)

Poster/Presentation
Number 105

Objective

Background BACKGROUND: Susac's syndrome is an autoimmune endotheliopathy with predilection for brain, retina and cochlea. It should be differentiated from central nervous system (CNS) demyelinating disorders such as multiple sclerosis (MS). As Susac's syndrome can have devastating neurological consequences, early diagnosis and treatment is essential to avoid a poor outcome. OCT is a noninvasive test which has recently been elaborated in the diagnosis of CNS demyelinating disease, but it has not been studied in Susac's syndrome so far.

Design/Methods DESIGN/METHODS: Three patients with confirmed diagnosis of Susac's syndrome underwent Spectral Domain OCT using Heidelberg Spectralis[copy] OCT, to determine retinal nerve fiber layer (RNFL) and macular thickness. The patients were in different stages of Susac's syndrome: acute, chronic- optimally treated, and chronic- untreated. The OCT results were analyzed using normative database which was provided by Heidelberg Spectralis[copy].

Results RESULTS: The OCT demonstrated decreased RNFL thickness and loss of foveal contour in all of our patients, the degree of which correlated with the stage and severity of the disease. RNFL abnormalities were patchy and more severe in the nasal quadrant, contrasting with typically temporal quadrant abnormalities described in MS .

Conclusions CONCLUSIONS: We demonstrate for the first time in this pilot study the OCT changes in different stages of Susac's syndrome. OCT may prove to be a useful diagnostic tool in Susac's syndrome, and help differentiate it from MS. Furthermore, OCT may be a non-invasive alternative to fluorescein angiography in longitudinal follow up of these patients.

Authors/Disclosures
Jacqueline Bernard, MD, FAAN (Oregon Health and Sciences University) PRESENTER	Dr. Bernard has received personal compensation in the range of $500-$4,999 for serving as a Consultant for TG Therapeutics. Dr. Bernard has received personal compensation in the range of $500-$4,999 for serving as a Consultant for 2ND MD. Dr. Bernard has received publishing royalties from a publication relating to health care.
Rebecca Romero, MD, FAAN (UTHSCSA)	Dr. Romero has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alexion. Dr. Romero has received personal compensation in the range of $500-$4,999 for serving as a Consultant for TG Therapeutics . Dr. Romero has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Genentech. Dr. Romero has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Horizon.
	No disclosure on file
Alasdair Coles, MD, PhD (University of Cambridge)	Dr. Coles has nothing to disclose.
	No disclosure on file
Kourosh Rezania, MD, FAAN (University of Chicago)	Dr. Rezania has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Akcea. Dr. Rezania has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Alnylam.

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