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Abstract Details

Susac’s Syndrome Presenting as Recurrent Ischemic Strokes
Cerebrovascular Disease and Interventional Neurology
P1 - Poster Session 1 (11:45 AM-12:45 PM)
14-013
Highlight an uncommon presentation of Susac’s syndrome.
Susac’s syndrome (SS), characterized by the clinical triad of encephalopathy, hearing loss, and branch retinal artery occlusion (BRAO), is a rare autoimmune endotheliopathy affecting the microvasculature of the central nervous system. This case highlights that, as a small vessel vasculopathy, SS can present as ischemic strokes.

A woman in her 20s presented for evaluation of headaches with nausea/photophobia and episodic confusion/disorientation. Initially diagnosed with rhinosinusitis, her symptoms continued to worsen despite treatment.

MRI brain with/without contrast revealed acute and subacute lacunar infarcts in the right frontal lobe, corpus callosum, and left parietal lobe. CT angiogram and echocardiogram with bubble were normal. Hypercoagulable workup and body CT screening for malignancy were negative. Long-term EEG showed no epileptiform activity. Cerebrospinal fluid (CSF) analysis demonstrated elevated protein (182), normal glucose/cellularity, and negative cultures. Headaches and episodic encephalopathy continued to worsen. Repeat MRI brain demonstrated new callosal and infratentorial lacunar infarcts. CSF protein increased on repeat analysis, but autoantibody panel resulted negative.

Lesion location (predominantly small vessel with callosal involvement), elevated CSF protein, and lack of typical vascular risk factors prompted concern for SS. Initial in-hospital audiology and ophthalmology evaluations were unrevealing; however, retinal fluorescein angiography (RFA) revealed binocular microangiopathy and BRAO. She was given methylprednisolone with symptomatic improvement and continues on maintenance IVIG therapy.
Though a clinical triad typifies SS, only 13% of cases present with all three symptoms at diagnosis.  Providers should consider this condition in patients with lacunar infarcts without typical cerebrovascular risk factors. When suspected, audiologic and ophthalmologic assessments are crucial, as prompt treatment reduces disease-related disability. Audiology can reveal sensorineural hearing loss while RFA can reveal retinal infarction, BRAO, and segmental arteriolar wall hyperfluorescence. 
Authors/Disclosures
John Kevin Pendas, MD
PRESENTER 		Dr. Pendas has nothing to disclose.
John Cassel, MD (University of South Florida) Dr. Cassel has nothing to disclose.
John Ciotti, MD (University of South Florida) Dr. Ciotti has received personal compensation in the range of $500-$4,999 for serving as a Consultant for EMD Serono. Dr. Ciotti has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Novartis. Dr. Ciotti has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Genentech. Dr. Ciotti has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Janssen Pharmaceuticals. The institution of Dr. Ciotti has received research support from National MS Society.
Swetha Renati, MD (University of South Florida) Dr. Renati has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Bayer. Dr. Renati has received personal compensation in the range of $500-$4,999 for serving as a NeuroSAE with 好色先生 .