Can you die from Susac syndrome?

Can you die from Susac syndrome?

In our patient, Susac syndrome was incomplete since hearing loss could never be demonstrated and atypical since consciousness rapidly worsened despite aggressive immunosuppressive therapy. Only one lethal case of Susac syndrome with severe encephalopathy and mutism has previously been reported [14] . …

What is the treatment for Susac syndrome?

Susac syndrome is best treated as soon as possible with an aggressive, long-term course of drugs that suppress the immune system. Medications may include: Prednisone or other corticosteroids. Intravenous methylprednisolone.

Is Susac syndrome a disability?

The disease usually self-limits after 2-4 years, Late relapses after decades have been reported. The final outcome shows a large variability of disability ranging from unimpairment to dementia, deafness and blindness. The majority of patients retains variable degrees of cognitive, visual and/or hearing deficits.

What autoimmune disease causes speech problems?

The first noticeable symptoms of myasthenia gravis may be weakness of the eye muscles, difficulty in swallowing, or slurred speech. Myasthenia gravis is an autoimmune disease because the immune system–which normally protects the body from foreign organisms–mistakenly attacks itself..

Is Meniere’s disease a neurological disorder?

Many other genetic neurological disorders can be associated with hearing and/or vestibular functional loss—that is, the syndromic audiovestibular diseases. In most, the site of the audiovestibular disturbance is unknown, but in some it is primarily in the end organ.

Can inner ear problems cause speech problems?

It may be harder to hear and understand speech if sound is muffled by fluid in the middle ear. Some researchers report that frequent hearing loss in children with middle ear fluid may lead to speech and language difficulties.

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.

What autoimmune causes tinnitus?

One cause of tinnitus in people with lupus is autoimmune inner ear disease (AIED). In AIED, the body’s immune system attacks the inner ear. This results in damage that can cause tinnitus. While it is possible to have AIED on its own, 15 to 30 percent of those with AIED have other autoimmune disorders.

How is the disease of Susac syndrome treated?

Susac syndrome is an autoimmune disease of the small arterioles of the brain, retina, and inner ear that has been successfully treated with immunosuppressants, such as cyclophosphamide or steroids. 3 Contrast enhancement of brain lesions and leptomeninges hints at an inflammatory process.

Can a person with Susac syndrome lose both eyes?

Both eyes can be affected in individuals with Susac syndrome. Permanent impairment of vision may occur. BRAOs may occur early in some individuals or later in the course of the disease in others. Many individuals with Susac syndrome develop hearing loss due to damage to the small, snail-shaped organ of the inner ear known as the cochlea.

What causes hearing loss in people with Susac syndrome?

Many individuals with Susac syndrome develop hearing loss due to damage to the small, snail-shaped organ of the inner ear known as the cochlea. The cochlea converts sound into nerve impulses to be sent to the brain. The damage to the cochlea is caused by the blockage of blood flow through the small vessels that supply blood to the cochlea.

How is Susac syndrome related to multiple sclerosis?

When Susac syndrome presents primarily with hearing loss, tinnitus, and vertigo, it is frequently misdiagnosed as Meniere’s disease. Multiple sclerosis is a chronic disorder of the central nervous system (CNS) that causes the destruction of the covering (myelin sheath) over the nerves.