What is Susac syndrome?

What is Susac syndrome?

Susac syndrome is a relatively rare disorder characterized by three main problems: impaired brain function (encephalopathy), partial or complete blockage (occlusion) of the small arteries and capillaries that supply blood to the retina (branch retinal artery occlusion, or BRAO), and inner ear disease (hearing loss.

How many cases of Susac syndrome are there?

Susac syndrome (SuS) prevalence is still unknown. To date more than 500 cases have been reported worldwide. Young females (20-40 years) are more affected (female: male ratio 3.5:1). The age at onset ranges from 8 to 72 years (mean age: 32 years).

How do you test for Susac syndrome?

A complete eye exam is also done to diagnose Susac syndrome. This eye exam will include a fluorescein angiography, which measures the blood flow through the retina. Other tests include a hearing exam to test for hearing loss and analysis of the cerebrospinal fluid, which may show high protein levels.

Who discovered Susac syndrome?

In 1979, John Susac, MD, and two colleagues reported the cases of two patients who presented with encephalopathy, hearing loss, and retinal microangiopathy. This condition was later named Susac syndrome (SuS). Today, despite continued research, little is known about its cause.

Is there a cure for Susac syndrome?

Treatment requires immunosuppression. High-dose corticosteroid therapy is the mainstay, but additional therapies such as intravenous immunoglobulin, mycophenolate mofetil, and cyclophosphamide are often necessary. Rituximab is the newest therapy to consider.

What are the symptoms of Susac syndrome?

What are the symptoms of Susac syndrome?

• Severe headache, often with vomiting.
• Confusion.
• Problems with thinking, such as short-term memory loss, slow thought processing and reduced ability to solve problems.
• Slurred speech.
• Walking difficulties.
• Changes in personality.

What autoimmune disease causes brain fog?

Lupus fog — the forgetfulness and fuzzy-headed feeling that can come with lupus (systemic lupus erythematosus, or SLE) – can be one of the most frustrating symptoms of the condition. The term lupus fog means more than memory problems.

Can autoimmune diseases cause memory loss?

Encephalopathies — typically characterized by confusion, seizures, memory loss and behavioral changes — have been well-recognized as having an autoimmune cause. In contrast, patients with progressive dementia without delirium have usually been classified as having neurodegenerative disease.

Can Cogan syndrome be cured?

Most patients with Cogan’s syndrome respond well to treatment, however in some cases, the disorder can lead to permanent hearing or vision loss. Early diagnosis and treatment can help minimize these risks. The long-term outlook also depends on whether vasculitis is present and its severity.

What does Covid brain fog feel like?

Key Takeaways. Brain fog is a common symptom reported by COVID long-haulers. People who have brain fog describe it as the inability to concentrate or to think clearly, as well as trouble remembering things.

Is Cogan syndrome fatal?

Prognosis. Many people with Cogan’s syndrome have permanent visual or hearing damage. The condition is fatal in less than 10% of patients. However, most patients are able to manage their symptoms and limit complications of the disease with treatment.

What does Susac syndrome mean?

Susac syndrome, usually seen in young females, is an angiopathy of unknown etiology that presents with various signs including ataxia, vertigo, pyramidal signs, and epileptic seizures due to extensive white and gray matter involvement.

What is Susac disease?

Susac’s syndrome is an autoimmune disease in which the smallest blood vessels in the brain, retina, and inner ear become blocked, causing these organs to suffer. Symptoms include headaches, slurred speech, and trouble focusing. Treatment includes drugs that suppress the immune system, such as steroids.

Is susacs syndrome hereditary?

Inheritance. To our knowledge, there is no evidence that Susac syndrome is inherited, and no familial cases of Susac syndrome have been reported. Cases described in the medical literature have occurred in people with no known history of the condition in their family.