Susac's syndrome, also known as Susac syndrome or retinocochleocerebral vasculopathy, is a rare autoimmune disorder that affects the small blood vessels in the brain, retina, and inner ear. It was first described by Dr. John Susac in 1979.
Susac's syndrome is characterized by a triad of symptoms:
These three hallmark symptoms may not all appear at the same time, and their severity can vary from person to person. Susac's syndrome predominantly affects young women between the ages of 20 and 40.
The exact cause of Susac's syndrome is still unknown, but it is believed to be an autoimmune disorder, where the body's immune system mistakenly attacks the blood vessels. It is not contagious or hereditary.
Diagnosis of Susac's syndrome can be challenging due to its rarity and similarity to other conditions. It often requires a combination of clinical evaluation, imaging tests (such as MRI and angiography), and hearing and vision tests.
Treatment for Susac's syndrome aims to control the inflammation and manage the symptoms. It typically involves a combination of immunosuppressive medications, such as corticosteroids, and supportive therapies to address specific symptoms like hearing loss or cognitive impairment.
While there is no cure for Susac's syndrome, many individuals experience spontaneous remission over time. However, some may have long-term complications, such as permanent hearing or vision loss.
It is important for individuals with Susac's syndrome to work closely with a multidisciplinary medical team, including neurologists, ophthalmologists, and audiologists, to monitor their condition and manage symptoms effectively.