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Devic’s Syndrome (Neuromyelitis Optica)

What is Devic’s syndrome?

Devic’s syndrome, also known as neuromyelitis optica, is an immune disorder that is sometimes confused with multiple sclerosis (MS). Devic’s syndrome is characterized by inflammation of the optic nerve of the eye and/or the spinal cord due to attacks by the immune system. Individuals with Devic’s syndrome may have one attack that lasts days or weeks or recurrent attacks separated by weeks to years of remission. The recurrent form of Devic’s syndrome is more common. The occurrence of Devic’s syndrome is estimated to affect approximately 1-5 in 100,000 individuals.

What are the symptoms of Devic’s syndrome?

The symptoms of Devic’s syndrome can often be confused with multiple sclerosis, so a correct diagnosis through MRI is important. Occurrence and severity of the symptoms of Devic’s syndrome depend on which optic nerve is affected, as well as where and how much of the spinal cord is affected. Symptoms of Devic’s syndrome include:
  • Loss of or blurred vision as well as problems with color recognition
  • Loss of bowel and/or bladder control
  • Weakness of legs and/or all four limbs ranging from mild to paralysis.
  • Abnormal sensations in legs such as burning, prickling and tingling
  • Muscle spasms
  • Headache
  • Fever
  • Loss of appetite
  • In rare cases, uncontrollable vomiting and hiccups when the brain stem is affected

What causes Devic’s syndrome?

Devic’s syndrome is thought to be an autoimmune disease in which the immune system suddenly begins to attack nerve tissue of the optic nerve and spine. This leads to inflammation and symptoms. The exact cause of this abnormal immune response is unknown. Some studies have linked the development of Devic’s syndrome to infections with varicella zoster virus, Epstein-Barr virus and HIV. Other studies have connected Devic’s syndrome to exposure to Clioquinol and anti-tuberculosis medications. Though not known to be an inherited disease, approximately 3% of patients with Devic’s syndrome report having family members affected as well.

Are there treatment options for Devic’s syndrome?

There is no known cure for Devic’s syndrome. A correct diagnosis of Devic’s syndrome versus MS is important because traditional medications used to treat MS do not appear to help with Devic’s syndrome. The treatment of Devic’s syndrome is directed at symptom management and the prevention of future attacks. Examples of treatments include:
  • Steroids to treat infection
  • Immune-suppression medications such as azathioprine to control the abnormal immune response
  • Medications to treat muscle spasticity

Where can I find more information about Devic’s syndrome?

Devic’s Syndrome (Neuromyelitis Optica) Articles