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Progressive Susac syndrome with bilateral visual loss and disability
Morteza Entezari1, Saeed Karimi1, Mohammadali Feizi2
1Department of Ophthalmology, Ophthalmic Research Center, Torfeh Medical Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Susac syndrome (SS) is a rare neurological disorder affecting the eyes, ears, and brain. Early diagnosis and treatment are crucial for preserving vision and improving patient outcomes.
Area of Science:
- Ophthalmology
- Neurology
- Vascular Medicine
Background:
- Susac syndrome (SS) is a rare autoimmune disease characterized by the triad of encephalopathy, retinal artery occlusions, and sensorineural hearing loss.
- The exact etiology of Susac syndrome remains unclear, posing diagnostic challenges.
Observation:
- A 35-year-old male presented with acute, painless vision loss in the right eye, followed by similar symptoms in the left eye two months later.
- Associated symptoms included hemiparesis, behavioral changes, and hearing loss, indicating a multi-systemic impact.
- Ophthalmic examination revealed multiple branch retinal artery occlusions (BRAOs) in both eyes.
Findings:
- Brain MRI demonstrated characteristic inflammatory changes with multiple "punched-out" lesions in the corpus callosum, confirming the diagnosis of Susac syndrome.
- Despite aggressive corticosteroid therapy, the patient experienced disease progression, including new BRAOs and persistent vision loss.
Implications:
- This case underscores the importance of prompt diagnosis and aggressive early treatment in Susac syndrome to prevent irreversible vision loss and neurological damage.
- Further research into the underlying mechanisms of Susac syndrome is needed to develop more effective therapeutic strategies.
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