Unraveling Susac Syndrome: A Case of Probable Diagnosis with Large Artery Disease
Gracie Little1, Ananya Vasudhar2, Muhammad Bhatti2, Lindsey Ladd3, Kunal Bhatia2
1University of Mississippi Medical Center, 2Neurology, University of Mississippi Medical Center, 3Neurology, University Of Mississippi Medical Center
Objective:
To demonstrate a unique presentation of probable Susac Disease in a patient with concomitant large vessel involvement
Background:
Susac syndrome (SuS) is a rare autoimmune vasculo-occlusive disease that affects the microvasculature of the brain, retina, and vestibulocochlear nerve in young women. Diagnostic criteria revolve around the classic clinical triad of encephalopathy, branched retinal artery occlusion (BRAO) and sensorineural hearing loss, as well as the presence of corpus callosal “snowball” lesions on MRI brain. Recent literature describes retinal microaneurysms and cotton wool spots as new ocular findings to suggest retinal ischemia in patients with SuS.
Results:
We present a 30-year-old African American female with a medical history of hypertension, diabetes mellitus, and recurrent ischemic stroke who presented with left leg weakness and gait imbalance. In addition, she described transient symptoms of headache, tinnitus, and an inability to ambulate which preceded each of her recurrent strokes. Brain MRI revealed several small acute infarcts involving the corpus callosum, right thalamus and cerebral peduncle, and right parietal lobe. CT angiogram was significant for chronic occlusion of the right ICA with reconstitution of the supraclinoid ICA. Diagnostic DSA was not suggestive of moyamoya-patterned revascularization. Laboratory workup revealed elevated ESR and CSF protein. Due to suspicion of possible SuS, ophthalmic evaluation was obtained to reveal bilateral retinal microaneurysms and cotton wool spots on fundoscopic exam. She was discharged on oral steroids with planned outpatient audiometry testing and ophthalmology follow up.
Conclusions:
SuS is notoriously challenging to diagnose given its broad symptomatology and presentation at various times over the course of a patient’s life. Although patients may not meet diagnostic criteria for definite Susac disease on initial presentation, it should be considered as the disease can have devastating effects such as dementia, blindness, and hearing loss.
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