Recognize Susac Syndrome, the Immune System that Wrongly Attacks Blood Vessels
This condition makes blood vessels clogged and causes interference with these organs.
This disorder, also known as retinocochleocerebral vasculopathy, is generally three times more common in women than men, especially women aged 20 to 40 years. However, children and adults, both boys and girls, outside this age group can also develop Susac syndrome.
What happens when the body’s immune system mistakenly attacks the blood vessels in the brain, eyes, and ears? This is what you need to know about Susac syndrome, including its symptoms, causes, diagnosis, and treatment.
1. Susac syndrome is an autoimmune condition that attacks the thin layer of cells in blood vessels
According to information from the National Organization for Rare Disorders (NORD), Susac syndrome is an autoimmune endotheliopathy condition. Autoimmunity is a condition where the immune system mistakenly attacks its own tissues.
Meanwhile, endotheliopathy is a disorder that causes injury to the endothelium (the thin layer of cells that lines the inside of blood vessels).
In Susac syndrome, the immune system mistakenly attacks the endothelial lining of the smallest blood vessels, such as the capillaries, venules, and arterioles, in the brain, retina, and inner ear (cochlea).
This attack causes the blood vessels to become injured, swollen, and blocked which blocks the flow of blood, oxygen and nutrients to the organ.
2. Researchers still don’t know the cause of Susac’s syndrome
The syndrome that was first reported by Dr. John Susac in 1979 is still not known for sure. It is not known why the immune system does not function properly, instead attacking blood vessel cells.
Launching Verywell Health, several autoimmune diseases occur because of inheritance or family inheritance. However, there is no evidence of cases of Susac syndrome being inherited from families or cases where more than one family member was affected.
3. Impaired brain function, vision and hearing are the main symptoms caused
The symptoms of Susac syndrome vary widely from person to person. Generally, this disorder is characterized by three main symptoms, namely impaired brain function (encephalopathy), partial or complete blockage (occlusion) of the branch retinal arteries (BRAO), and hearing loss.
However, these three symptoms may not appear at once. Some people may exhibit one or two of the three symptoms when evaluated by a doctor for the first time, while other symptoms appear later. In some cases, a person with Susac syndrome may not experience these three phases, but only develop certain combinations of symptoms.
In most cases, headaches, including migraines, usually precede the development of Susac syndrome. Some of the other symptoms that may occur in the brain can include:
- Headache, often accompanied by vomiting
- Cognitive dysfunction: short-term memory loss, confusion, and decreased executive functions of the brain such as problem solving
- Slurred walking and speech disorders
- Problems with focus and alertness
- Behavior or personality changes
- Mood problems, including depression, anxiety, anger, and aggression
- Psychosis, including delusions and hallucinations
- Meanwhile, branch retinal artery occlusion can cause a person to experience visual impairment, where they appear “dark spots” or “black areas” in their field of view. Some sufferers also described “curtains or shadows drawn” covering part of their vision. Medically, this condition is called a scotoma.
Meanwhile, disorders of the inner ear can cause a person to experience hearing loss, vertigo, and tinnitus (ringing in the ears). The severity can range from mild to severe.
Launching the National Institute of Health page, the symptoms of Susac syndrome can disappear and come back again for several years. Some other individuals may also experience the disorder only once, and recover after undergoing treatment
4. The diagnosis is usually made when three or two of the main symptoms of Susac’s syndrome are found
The diagnosis of Susac syndrome is made based on a physical examination, a detailed medical history and symptoms, as well as some special tests such as magnetic resonance imaging (MRI), fluorescence angiography, and audiogram.
If you find at least two of the three main symptoms and the presence of “snowball”, “finger”, or “hole” lesions in the brain, especially in the corpus callosum (the part of the brain that connects the left and right sides) on an MRI test, this will underlie the diagnosis. must be Susac syndrome.
A person who is suspected of having Susac syndrome will need to have an eye exam called fluorescence angiography, even if he or she has no eye symptoms. This test is used to measure blood flow through the retina.
In Susah syndrome, a fluorescent angiographic test shows: presence of BRAO, increased blood vessel wall stain (hyperflouresens of blood vessels), dye “leakage”, and chronic changes in the periphery (capillary rupture, neovascularization, microaneurysms), with potential for vitreous bleeding.
People with Susac syndrome should also have a hearing exam or audiogram to detect hearing loss. Everyone with suspicion of this syndrome should undergo this test even if they don’t have any inner ear symptoms.
5. Early and aggressive treatment is needed to overcome this autoimmune disorder
. Drugs such as immunosuppressants, corticosteroids, biologics, intravenous cyclophosphamide, and immunoglobulin drugs are usually used to suppress the activity of the immune system attacking blood vessels.
The use of a hearing aid or cochlear implant (for severe hearing symptoms) can also help reverse hearing loss.
That is an interesting fact of Susac syndrome. If you find changes in vision or hearing, or even find problems with gait or cognitive, immediately consult a doctor. Getting prompt and appropriate treatment can reduce or prevent other complications.