Atypical Presentation of Susac Syndrome in 55-Year-Old: From Unremarkable Stroke Workup to Rapid Diagnosis of “Snowball Strokes” and Successful Immunosuppressive Treatment

TL;DR

A 55-year-old woman with atypical Susac syndrome symptoms was successfully treated with immunosuppressive therapy after a complex diagnostic process.

Contribution

This case highlights the diagnostic challenges and successful management of atypical Susac syndrome in an older patient.

Findings

The patient's symptoms progressed rapidly despite an initially unremarkable stroke workup.

Aggressive immunosuppressive treatment led to significant clinical improvement.

The case underscores the importance of considering rare autoimmune disorders in differential diagnoses.

Abstract

We report the case of a 55-year-old right-handed female with a medical history of hypothyroidism and gastric bypass surgery. The patient initially presented with cognitive impairment, dizziness, and unsteady gait. Despite an unremarkable stroke workup, her symptoms progressed rapidly within 2 days, leading to subsequent admissions and a complex diagnostic journey revealing Susac syndrome—a rare autoimmune disorder affecting the brain's microvasculature, retina, and cochlea. The patient's treatment involved aggressive immunosuppression with corticosteroids, IVIG, mycophenolate, and cyclophosphamide. The patient responded well and had progressive improvement, with discharge to home. This case highlights the diagnostic challenges and management strategies for Susac syndrome.

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