What is Susac Syndrome? Let's talk about it simply.

Do you suddenly have a headache, memory loss, and a slight change in your vision and hearing? Perhaps these things can happen at the same time. It is normal for us to be scared when such symptoms suddenly appear. Today we are going to talk about a rare condition that can cause such symptoms, but is not very well known in our country. That is Susac Syndrome.

What is Susac Syndrome simply?

Simply put, Susac Syndrome is an autoimmune condition. Think of our body's immune system as like the army of our country. Its main job is to protect us from germs that cause disease. But sometimes this system makes a small mistake. Then, instead of attacking the enemies from outside, it starts attacking the good cells of our own body. This is what we call autoimmune diseases.

In Susac Syndrome, our immune system attacks the tiny blood vessels in the brain, retina, and cochlea . This attack causes those blood vessels to swell, reducing or completely blocking blood flow through them. That's what causes the symptoms of this disease.

Where is this disease mainly affected?

This condition mainly affects three parts of the body: the brain, eyes, and ears.

This is a very rare condition. Therefore, it is difficult to say exactly how many people develop it. Sometimes, the symptoms are similar to another disease called Multiple Sclerosis (MS), so even doctors can sometimes confuse the two diseases. Therefore, it can sometimes take a while to get the correct diagnosis.

What are the symptoms of Susac Syndrome?

Symptoms may vary from person to person, but they are related to the three main components mentioned earlier. Let's see what they are.

How do these characteristics affect you?

These symptoms do not occur in the same way for everyone. Some people develop this only once in their lifetime. For others, the symptoms come and go, then come back... and so on. Often, the symptoms can wax and wane over several years. Only a very small number of people have symptoms of all three at the same time when the disease is diagnosed.

Why does Susac Syndrome occur?

The exact cause of this is not yet known. But we do know that it is an autoimmune condition. As mentioned earlier, our own immune system attacks the cells inside the small blood vessels in our brain, retina, and inner ear. This attack causes those cells to swell. Then those blood vessels can become partially or completely blocked. When that happens, those parts of the body don't get the oxygen and nutrients they need. That's what causes all the symptoms mentioned earlier.

Is this a hereditary disease? Who is at higher risk?

No, there is no evidence that this is a hereditary disease. However , women are about three times more likely to develop this disease than men. Symptoms usually begin between the ages of 20 and 40.

What are the possible complications of this disease?

Damage to the eyes and ears caused by blocked blood vessels can sometimes be permanent. This means that vision and hearing can be permanently lost. And, although very rarely, a condition called dementia can occur, in which memory is completely lost.

How does a doctor diagnose this disease? (Diagnosis)

Your doctor will perform several tests to diagnose this disease.

• Physical exam
• Neurological exam
• Ophthalmological exam

In addition, you can also do tests like these to find out more about your symptoms:

• Magnetic Resonance Imaging (MRI) of the brain: This allows the doctor to look for white spots called 'snowball lesions' in the area where the two hemispheres of the brain connect (corpus callosum). These are a specific sign of Susac Syndrome.
• Fluorescein Angiography: This is a special test that examines the blood vessels in the retina of the eye. It can detect any blockages in the blood vessels.
• Audiogram: This is a hearing test. It checks whether you can hear low-frequency sounds and whether you have difficulty understanding speech.

What are the treatments for this?

The main goal of treatment is to control our immune system, which is malfunctioning. Immunosuppressants are used for this. Some examples are:

• Corticosteroids (e.g. prednisone)
• Intravenous immunoglobulin (IVIG)
• Mycophenolate
• Azathioprine
• Cyclophosphamide
• Rituximab

These medications can be given to the body in various ways. Some are taken as pills or capsules. Some are given as injections. Others are given intravenously (IV) through saline. These medications work by reducing the overactive immune system.

Learn about treatment, recovery, and outlook

Are there any side effects of the treatment?

Yes, like all medications, this one can have various side effects. So, talk to your doctor about this before starting treatment. If you experience any side effects, inform your doctor about them.

How long will it take to recover after treatment?

If this is recognized early and treatment is started early, symptoms can improve quickly. Some people only get sick once. Others may have recurring episodes over the years. However , with proper immunosuppressant therapy, the risk of recurrence is greatly reduced.

What will life be like with this disease? (Outlook)

The outlook for those who receive treatment as soon as symptoms appear is very good. This can prevent permanent damage to the brain, eyes, and ears. Permanent complications such as vision loss and hearing loss are rare. This is not a life-threatening disease, except in very rare cases. Although it may affect your quality of life, it does not directly affect your lifespan.

How to prevent the disease and when to see a doctor?

There is no specific way to prevent Susac Syndrome. However, by following the treatment plan given by your doctor exactly, you can minimize the recurrence of symptoms (flare-ups).

If you experience any of these changes, see your doctor immediately:

• Change in eyesight
• Hearing loss
• A change in the ability to think or remember
• A change in behavior or personality

The sooner the disease is recognized, the better you can stay healthy and prevent permanent damage.

Questions to ask your doctor

• "Can I come back after my symptoms improve?"
• "What kind of treatment do you recommend for me?"
• "How long should I use this medicine and how?"
• "What are the side effects of this medication?"
• "Will I need hearing aids or a cochlear implant?"

Susac Syndrome can affect your daily life. It's normal to feel scared and anxious when things you've always believed in, such as your vision or hearing, suddenly change. Sometimes talking to a mental health counselor can be a great relief. Remember, it's best to tell a doctor as soon as you notice symptoms, as complications can occur. Prompt treatment can prevent permanent damage.

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