Susac’s Syndrome: The clinical triad of encephalopathy, branch retinal artery occlusions and hearing loss
Susac’s Syndrome is named after Dr John Susac, of Winter Haven, FL, who first described it in 1979.
Susac’s Syndrome is a very rare disease, of still unknown etiology.
Susac’s Syndrome is an autoimmune condition that affects very fine blood vessels in the brain, retina, and the cochlea (inner ear).
It is characterized by three main symptoms: encephalopathy (which can include headache, mild memory losses, personality changes, and confusion); sensorineural hearing loss; and vision loss.
Most people do not have all the symptoms of Susac syndrome at the beginning of their illness but develop symptoms over the course of several years.
Some sufferers often experience a personality change and develop bizarre and paranoid behavior. Their speech can be affected, and many experience unrelenting and intense headaches and migraines, some form of hearing loss, and impaired vision.
In some cases, subjects can become confused, and believe they are re-living a time from their remembered past. According to Michael Hahn, a US Expert on the subject, the delusion of living in a foreign country occurs in a “fair percentage” of cases.
Susac’s Syndrome usually affects women around the age of 30 years old