Susac syndrome

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Susac Syndrome

Susac syndrome (/suːˈsæk/ soo-SAK) is a rare, non-inherited disorder characterized by a triad of symptoms: encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss. The condition was first described by Dr. John O. Susac in 1979.

Etymology

The syndrome is named after Dr. John O. Susac, who first described the condition in 1979.

Symptoms

The primary symptoms of Susac syndrome include:

  • Encephalopathy - A broad term used to describe brain disease, damage, or malfunction. In the context of Susac syndrome, it often presents as confusion, memory loss, or severe headaches.
  • Branch Retinal Artery Occlusion - This refers to a blockage in the small arteries that carry blood to the retina. It can cause sudden, painless vision loss.
  • Sensorineural Hearing Loss - This is a type of hearing loss in which the root cause lies in the inner ear or sensory organ (cochlea and associated structures) or the vestibulocochlear nerve (cranial nerve VIII).

Diagnosis

Diagnosis of Susac syndrome is often challenging due to the rarity of the condition and the variability of symptoms. It is typically diagnosed through a combination of clinical findings and imaging studies, including MRI and angiography.

Treatment

Treatment for Susac syndrome typically involves immunosuppressive therapy to reduce inflammation and prevent further damage to the brain, retina, and inner ear. This may include corticosteroids, cyclophosphamide, or mycophenolate mofetil.

Prognosis

The prognosis for individuals with Susac syndrome varies. Some individuals may experience a single episode and recover with minimal residual effects, while others may have recurrent episodes and significant long-term disability.

See Also

External links

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