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Neuromyelitis Optica Spectrum Disorder

Overview

Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disorder that causes inflammation of the optic nerve and the spinal cord. It occurs when white blood cells and antibodies in the blood turn against the body — instead of protecting the brain and spinal cord, the immune system then attacks them. While for some patients NMOSD occurs only once, it is now recognized as a chronic condition that may flare up and then go into a period of remission. It is sometimes misdiagnosed as multiple sclerosis, and was previously considered a type of MS.

Symptoms

  • Abrupt vision changes
  • Eye pain
  • Fatigue
  • Inability to empty the bladder
  • Loss of bowel or bladder control
  • Nausea and vomiting
  • Paralysis
  • Shooting pain in the neck, back or abdomen
  • Sudden inability to see color
  • Uncontrollable hiccups
  • Vision loss or blurry vision in one or both eyes
  • Weakness or numbness

Prevention

No way to prevent neuromyelitis optica spectrum disorder is known.

Risk Factors

  • Age between 40 and 50
  • Family history of autoimmune disorders
  • Female sex

Diagnosis

Medical history and symptom review. First, your medical provider will carefully review your personal and family medical history, your lifestyle habits and any medications you may be taking. He or she will also ask questions related to the specific symptoms you are experiencing.

Physical examination. Following a symptom review, your provider will perform an exam that pays particular attention to the areas affected by symptoms. During this exam, he or she will also look for signs that could indicate a different neurological condition could be to blame. This will likely include an examination of your movements and muscle strength, along with your coordination and cognition.

Additional tests. To definitively diagnose NMOSD, your provider will order additional tests, which will likely include blood work, an MRI scan and a lumbar puncture. The blood work will look specifically for the autoantibody NMO-IgG, which helps differentiate NMOSD from other conditions, including multiple sclerosis.

Treatment

  • No curative treatment for NMOSD exists, so treatment will focus on alleviating symptoms and mitigating the risk of future attacks.
  • To reduce the inflammation related to an acute attack, your medical provider will likely prescribe an intravenous infusion of corticosteroid. This therapy typically lasts for up to five days and then will be tapered off.
  • Plasma exchange (plasmapheresis) is also often used in addition to steroids. During this procedure, blood is removed from the body and blood cells are separated from the plasma. Your blood cells are then mixed with a replacement solution and returned to the body.
  • To prevent future attacks, your medical provider may recommend you take a low dose of corticosteroids for a long period of time. This may be used in combination with other medications to suppress the immune system and prevent relapses.

Follow-up Care

  • Because NMOSD is a chronic disorder, treatment is ongoing. Your medical provider will recommend a regimen of care that includes how often follow-up appointments are needed.
  • In cases where vision impairment or movement-related symptoms linger, your provider may recommend the use of adaptive devices and equipment. Physical and occupational rehabilitation may also be helpful in recovering abilities.