Other Names for this Disease
- Retinocochleocerebral vasculopathy
- SICRET (small infarction of cochlear, retinal, and encephalic tissue) syndrome
What are the signs and symptoms of Susac syndrome?
What causes Susac syndrome?
How is Susac syndrome diagnosed?
How might Susac syndrome be treated?
The specific symptoms and severity of Susac syndrome can vary from one person to another. Headache is often one of the earliest symptoms of Susac syndrome. Recurrent headaches are almost always associated with encephalopathy. Other neurological symptoms may develop including walking difficulties, slurred speech (dysarthria), and cognitive changes including memory loss, confusion and personality or behavioral changes.
Patients may also have branch retinal artery occlusions (BRAO) usually in both eyes. Impairment of vision can occur in some cases; in other cases, people may have no symptoms with the presence of BRAO.
The three main symptoms (encephalopathy, branched retinal arterial occlusions, and hearing loss) are not always present at the onset of symptoms and all three do not necessarily develop in all cases.Susac syndrome can go away on its own, even without treatment. It usually runs its course in two to three years in which individuals experience recurrent episodes of symptoms. Although Susac syndrome may resolve on its own, some people can develop persistent neurological damage, hearing or vision loss.
The symptoms of this condition result from damage to these very small blood vessels, which in turn results in decreased or impaired blood flow. Impaired blood flow to the brain, retinas and inner ears (microinfarcts) results in damage to the tissue or organs in these areas.
In individuals with Susac syndrome, an MRI can show characteristic changes to the brain, especially the corpus callosum. A complete eye exam, including a flourescein angiography (angiogram) is necessary. Individuals suspected to have Susac syndrome should also have a hearing exam to detect any hearling loss.
Susac syndrome can mimic several diseases. These conditions may include multiple sclerosis, acute disseminated encephalomyelitis, chronic encephalitis, aseptic meningitis, Lyme disease, cardioembolic disorder, complicated migraine, systemic lupus erythematosus, Bechet syndrome, sarcoidosis, tuberculosis, syphilis, lymphomas, and Creutzfeldt-Jakob disease.
For those with significant hearing loss, a specific type of hearing aid called a cochlear implant may be an option.
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