Objective:

To detail a case report of a patient with Susac’s syndrome presenting with multifocal neurologic deficits. 

Background:

Susac’s syndrome is a rare, immune-mediated endotheliopathy characterized by microvascular injury leading to infarction within the brain, retina, and inner ear, producing a classic triad of symptoms: encephalopathy, branch retinal artery occlusions (BRAO), and sensorineural hearing loss. Key findings for the diagnosis include MRI brain showing FLAIR/T2 hyperintensities characteristically within the corpus callosum, fluorescein angiography demonstrating BRAO, and audiometry showing sensorineural hearing loss. Its presentation can vary given the multifocality of deficits. Another more common cause of multifocal neurologic deficits is patent foramen ovale (PFO) leading to embolic strokes.  

Design/Methods:

NA

Results:

We present a 32-year-old female who initially presented with vision loss as well as a remote history of transient left sided sensory deficits. Her deficits were thought to be due to a cardioembolic process in the setting of PFO which was then surgically corrected. She developed new symptoms of hearing loss followed by lightheadedness and imbalance which prompted re-evaluation. Additionally, she developed self-limited episodes of confusion lasting several weeks. MRI brain showed FLAIR/T2 hyperintensity involving the corpus callosum and left corona radiata. Suspicion for Susac’s syndrome led to further testing with fluorescein angiography revealing bilateral BRAOs and Audiometry revealing sensorineural hearing loss.  

Conclusions:

Given the multifocal neurologic signs and symptoms seen in Susac’s syndrome, it can easily be misdiagnosed as a more common cause of multifocal neurologic deficits such as embolic vascular disease. Characteristic MRI findings along with the triad of encephalopathy, recurrent BRAOs, and sensorineural hearing loss should raise suspicion for Susac’s syndrome. Importantly, the correct diagnosis of Susac's syndrome supports the use of immunotherapy for secondary stroke prevention. While it is a rare entity, Susac’s syndrome should remain in the differential diagnosis when a patient presents with multifocal neurologic deficits.