Susac syndrome is a rare autoimmune microangiopathy affecting the brain, retina, and inner ear. It leads to a triad of encephalopathy, branch retinal artery occlusions, and hearing loss.
Clinical features
Symptoms may appear together or sequentially.
- Confusion, cognitive changes, and headaches from brain involvement
- Visual field defects from branch retinal artery occlusions seen on fluorescein angiography
- Sensorineural hearing loss, often low-frequency
- Characteristic ‘snowball’ lesions in the corpus callosum on MRI
Treatment
Early aggressive immunosuppression improves long-term outcome.
- High-dose steroids followed by steroid-sparing agents such as IVIG, mycophenolate, or cyclophosphamide
- Close collaboration between neurology, rheumatology, and ophthalmology
- Hearing and visual rehabilitation where deficits persist
- Long-term monitoring for relapses
