What is Susac syndrome?

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Living with Susac syndrome

Susac syndrome was named after Dr. John Susac and it is a rare autoimmune systemic or rheumatologic disease caused by occlusions of the microvessels in the brain, retina, and inner ear.

The damage can be reversible or permanent, depending on the severity and the duration of the blocked blood flow.

What causes Susac syndrome?

Susac Syndrome is an autoimmune disease and for this reason the underlying causes are still unknown. One of the hypotheses is that it is either due to a genetic predisposition or that it is caused by the exposure to external triggering factors.

What are the symptoms of Susac syndrome?

Susac syndrome symptoms can involve brain, retina, and cochlea. They can manifest at the same time, or surface years after previous symptoms. These symptoms include cognitive dysfunction, like headaches, severe and persistent migraines and sensory disturbance, such as loss of balance, vision, verbal fluency, or motor function.

Brain

Susac syndrome symptoms that involve the brain consist of headaches, migraines, mental confusion, disorientation, difficulty thinking, inattention, loss of memory, especially short-term memory, psychosis, behavioural and personality disorders. This condition causes the occlusion of the microvessels in the brain because the immune system mistakenly attacks endothelial cells that form in the interior surface of the arterioles and capillaries.

Eye

Susac syndrome symptoms at the level of the retina include Branch Retinal Arterial Occlusion (BRAO), scotomas, and peripheral vision loss. These symptoms may also include general eye diseases, visual field deficit with dark vision areas and the feeling of a dark patina in the eye. These symptoms occur because Susac syndrome is an autoimmune disease where the immune system attacks the microvessels of the retina causing their occlusion.

Inner ear

Susac syndrome affecting the inner ear is characterised by hypoacusis, and sudden hearing loss, intense tinnitus, vertigo, as well as other balance disorders and high frequency hearing loss. Similarly to its effects on the brain and in the eye, the immune system doesn’t recognise the cells of the inner surface of the vessels of the ear and it attacks them, causing their obstruction and occlusion.

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How is Susac syndrome diagnosed?

The diagnosis of Susac syndrome is rather difficult to pinpoint because its symptomatology is very similar to other pathologies such as multiple sclerosis, lupus, or meningitis.

Nevertheless, there are three examinations that can verify if the brain, retina, and cochlea are eventually compromised:

  • Nuclear Magnetic Resonance (NMR) of the brain;
  • Fluorescein Angiography;
  • Audiometry exam.

Can Susac syndrome be prevented?

Since the underlying causes of Susac syndrome are unclear, it is very difficult to understand how to prevent it. Nevertheless, closing monitoring its symptoms is always the best way to deal with the possible disease. 

How is Susac syndrome treated?

The treatment of Susac syndrome strictly depends on the symptoms observed. After the symptoms are confirmed by the doctor, they will determine the appropriate treatment.

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