Susac syndrome is an autoimmune condition that affects the very small blood vessels in the brain, retina, and inner ear (cochlea). The condition is characterized by three main symptoms: brain disease (encephalopathy), hearing loss, and vision loss. Some people may not have all signs of Susac syndrome, but instead develop only specific combinations of the symptoms. Susac syndrome affects women more than men. The age at which symptoms begin is usually between 20 and 40 years, but some people have symptoms earlier or later than this age range.
The cause of Susac syndrome is still unknown. Diagnosis is based on a clinical exam and imaging tests to look for the specific signs of Susac syndrome. Treatment options include corticosteroids and cyclophosphamide as well as anticoagulation medications. Most people recover from Susac syndrome with treatment.
Last updated: 2/20/2017
How might Susac syndrome be treated?
Early, aggressive, and sustained treatment of Susac syndrome is recommended to avoid or minimize neurological damage, hearing loss, and vision loss. Treatment includes medications that suppress the activity of the immune system (immunosuppressive therapy) so that it does not attack the blood vessels in the brain, eyes, and ears. Immunosuppressive medications may include high doses of corticosteroids, cyclophosphamide, and intravenous immunoglobulins. Doctors may also recommend blood thinners such as aspirin in order to prevent clotting in the small blood vessels. Once treatment has improved symptoms, the medications can be slowly tapered off.
Alternative treatments such as mycophenolate mofetil may be helpful in cases that don’t improve with other treatment. Overall, medications for Susac syndrome can prevent more symptoms from developing and improve symptoms of the condition, but the treatment may not be able to repair neurological, hearing, or visual damage. In some cases, people with severe hearing loss may benefit from cochlear implants.
Last updated: 2/21/2017
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