Susac's syndrome is a rare autoimmune disorder that causes the body's natural defenses to behave as if there were an infection or injury when there is none.
The condition most often affects the smallest blood vessels in the brain, eye and inner ear, and can cause the blood vessels to become blocked. When a blood vessel is blocked, the blood and oxygen supply to that area of the body is limited, which can cause damage to the cells, tissues and organs.
Symptoms of Susac's syndrome vary based on the severity of the condition and the part of the body that is affected.
Symptoms related to the brain may include:
- Confusion or difficulty thinking (cognitive impairment)
- Short term memory loss
- Difficulty remaining focused and alert
- Changes in speech
- Changes in personality
- Migraine headaches
Symptoms related to the eye may include:
- Dark spots in vision
- Loss of side (peripheral) vision
- Darkening of vision
Symptoms related to the inner ear may include:
- Hearing loss
- Ringing in the ears
The condition is also associated with stroke when the blood vessels of the brain are affected. If you notice one or more of these signs in another person or in yourself, do not wait to seek help. Call 9-1-1 immediately.
The signs of a stroke are:
- Sudden numbness or weakness of the face, arm or leg, especially on one side of the body
- Sudden confusion
- Sudden trouble speaking
- Sudden trouble seeing in one or both eyes
- Sudden trouble walking
- Sudden dizziness, loss of balance or coordination
- Sudden, severe headache with no known cause
Causes and Risk Factors
The cause of Susac's syndrome is unknown and the condition is very rare. Susac's syndrome affects women three times more often, and between the ages of 20 and 40. However, the condition can occur in both men and women at any age.
Diagnosis of Susac's syndrome usually begins with a physical exam and a review of the patient's medical history and symptoms. A positive finding of Susac's syndrome is based on identifying symptoms and the findings of diagnostic tests.
Imaging tests, such as magnetic resonance imaging (MRI) or computed tomography (CT), are used to help diagnose the condition and better understand the patient’s symptoms. MRI or CT scans also may be used to look for areas of the brain that may show stroke damage. A cerebrovascular evaluation will look at the affected blood vessels and may include an angiography.
There is no cure for Susac's syndrome. Some cases may clear up over time without treatment; however, it is important that patients receive medical attention to minimize the possibility of permanent neurological damage. Treatment plans focus on managing the body's autoimmune response and addressing the symptoms.
Medications that suppress the immune system (immunosuppressants), such as corticosteroids, may be prescribed to manage the body's overactive immune system. Other medications may also be used to manage other symptoms.
If the patient has experienced hearing loss, a hearing aid may be used to help them regain some of their hearing.
For patients who have experienced a stroke, the Stroke Program at the Cedars-Sinai provides a multidisciplinary treatment approach through a personalized treatment plan tailored to each patient. Patient care is generally broken down into three categories: stroke prevention, treatment immediately after a stroke, and post-stroke rehabilitation.