From the Research
The best treatment for Susac syndrome typically involves immunosuppressive therapy, with high-dose corticosteroids as the first-line treatment, as supported by the most recent study 1.
Treatment Approach
A common regimen starts with intravenous methylprednisolone 1000mg daily for 3-5 days, followed by oral prednisone (1mg/kg/day) with a slow taper over 6-12 months. For patients with severe or refractory disease, additional immunosuppressants are often added, including intravenous immunoglobulin (IVIG) at 2g/kg divided over 2-5 days, cyclophosphamide, mycophenolate mofetil, or rituximab 2, 3. Aspirin (81-325mg daily) is frequently prescribed to address the microangiopathic component of the disease.
Key Considerations
- Treatment should begin as early as possible after diagnosis to prevent permanent damage to the brain, retina, and inner ear.
- The therapy needs to be individualized based on disease severity, with regular monitoring of symptoms and organ involvement.
- Early aggressive treatment is crucial because Susac syndrome can cause permanent cognitive impairment, vision loss, and hearing deficits if left untreated.
- The goal is to suppress the autoimmune response that causes small vessel occlusions in the brain, retina, and cochlea.
Monitoring and Adjustment
- The response to treatment should be closely monitored by frequent clinical examinations, brain MRI, and fluorescein angiography 4.
- Once disease remission has been established, it appears prudent to continue maintenance treatment for at least two additional years, although the real long-term risk of future relapses remains unknown.