Abstract Details

Title More than a Stroke: When Hearing Loss and Vision Changes Reveal Susac Syndrome - A Case Report

Topic Cerebrovascular Disease and Interventional Neurology

Presentation(s) P2 - Poster Session 2 (11:45 AM-12:45 PM)

Poster/Presentation
Number 4-016

Objective To describe a case of Susac Syndrome and highlight the variability in its clinical presentation, emphasizing the role of early and intensive immunosuppressive therapy.

Background Susac syndrome is a rare autoimmune microangiopathy characterized by a triad of encephalopathy, branch retinal artery occlusion (BRAO), and hearing loss. This condition primarily affects women with a mean age between 29.5 and 31.6 years. The mainstay of treatment involves immunosuppressive therapies with methylprednisolone and IVIG. This case report highlights the use of plasma exchange therapy along with cyclophosphamide maintenance therapy as early treatment options.

Design/Methods N/A

Results A 42-year-old female presented with sudden onset right-sided hearing loss, tinnitus, and transient visual disturbances. MRI showed multifocal lesions in the periventricular white matter and corpus callosum and was prescribed prednisone. CTA head and neck was negative, and lumbar puncture was normal. Discharged with a presumed diagnosis of embolic stroke of undetermined source. After 3 weeks, the patient presented with an episode of generalized tonic seizure and was started on seizure management. The repeat brain MRI showed new punctate lesions with restrictive diffusion and post-contrast enhancement. Started a course of methylprednisolone and discharged on a tapering dose of prednisone. The patient had worsening symptoms with bilateral hearing loss and visual changes. Aggressive management with intravenous methylprednisolone, single dose of IVIG, therapeutic plasma exchange and cyclophosphamide resulted in symptomatic stabilization and marked reduction in disease activity. An ophthalmologic examination showed bilateral branch retinal artery occlusions (BRAO). The presentation, imaging findings and improvement with intense immunosuppressive treatment confirm the diagnosis of Susac syndrome.

Conclusions This case signifies the use of therapeutic plasma exchange coupled with cyclophosphamide maintenance therapy as an early treatment option in fulminant or treatment-resistant disease. Despite advancements in therapeutic strategies, the rarity and heterogeneity of Susac syndrome call for further research to optimize management protocols and improve treatment outcomes.

Authors/Disclosures
Suresh Reddy, MBBS (Osmania Medical College) PRESENTER	Mr. Reddy has nothing to disclose.
Sai Kumar Reddy Pasya, MD, MBBS	Dr. Pasya has nothing to disclose.
Natalie W. Byrne, MD (The University of Kansas Medical Center)	Dr. Byrne has nothing to disclose.
Virushnee Senthilkumar, MBBS	Dr. Senthilkumar has nothing to disclose.
Laith N. Maali, MD (University of Kansas Medical Center)	Dr. Maali has nothing to disclose.

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