How Neuromyelitis Optica Spectrum Disorder Is Treated

Treatment aims to improve comfort and prevent relapses

In This Article


Neuromyelitis optica spectrum disorder (NMOSD) is a rare condition involving the central nervous system (brain and spinal column). It is considered an autoimmune disease, because the underlying symptoms are caused by the immune system attacking its own cells and tissues (most commonly involving the spinal cord and the optic nerve). The most common form of this disorder—relapsing NMOSD—is characterized by flare-ups. The flare-ups (or relapses) can happen months, or even years apart. The other form of the disorder is called monophasic NMOSD, involving just one episode usually lasting around 30 to 60 days.

Although there is no cure for neuromyelitis optica spectrum disorder, there are many effective treatment modalities aimed at alleviating symptoms and preventing future relapses. Due to an increase in the recognition of this rare disease, there is a growing body of clinical research study evidence. This has resulted in a standard of care that is backed by research studies. The standard of care for NMOSD includes care for the treatment of acute (quick and severe) relapses, prevention of relapses, and therapies for treating symptoms of NMOSD.

Treatment of NMOSD involves two objectives:
1.   Suppression of acute inflammatory relapse
2.   Prevention of future relapses

Typical symptoms of NMOSD include muscle weakness (paraparesis), paralysis of the extremities (usually the legs, but sometimes the upper body as well) and visual disturbances or blindness in one or both eyes. Some people with NMOSD also have symptoms such as severe uncontrollable vomiting and hiccoughing. This results from an attack on brain tissue. 

In 70% of people with NMOSD, the person’s antibodies bind to a protein called aquaporin-4 (AQP4) autoantibody, also referred to as the NMO-IgG autoantibody. AQP4 is an autoantibody, produced by the immune system, that is directed against a person’s own tissue in the optic nerve and central nervous system.

Prescriptions

Medication for Prevention of Relapses

In 2019 the U.S. Food & Drug Administration announced the first approved treatment for NMOSD with the approval of Soliris (eculizumab) injection for intravenous (IV) administration. Soliris is for those who are anti-aquaporin-4 (AQP4) antibody positive. According to the FDA announcement, “This approval changes the landscape of therapy for patients with NMOSD.”

In a study of 143 people with NMOSD (who were AQP4 positive), the participants were randomly assigned to two groups; one group was given Soliris treatment and the other group received a placebo (sugar pill). The study discovered that over a 48-week course of the clinical research trial, those treated with Soliris experienced a 98% reduction in the number of relapses, as well as a reduction in the need for treatment of acute attacks and hospitalizations.

Acute Treatment

The goal of acute treatment is to subdue the acute inflammatory attack to help minimize the damage to the central nervous system, while improving long-term function. The first line of treatment for acute (sudden, severe) attacks is a high dosage (1 gram daily for three to five consecutive days) of methylprednisolone (a corticosteroid drug, given to suppress inflammation in acute relapses of NMOSD).

Other Medications for Acute Treatment

In some instances of acute treatment, high doses of corticosteroids and plasma exchange procedures are ineffective. Researchers have thus, experimented with alternative treatment approaches to acute NMOSD relapses. One such treatment is intravenous immunoglobulin (IVIg). Immunoglobulin therapy (also called normal human immunoglobulin) is the use of a mixture of antibodies to treat various health conditions such as Guillain-Barre syndrome and myasthenia gravis. Its effect on reducing inflammation in diseases of the central nervous system is not yet fully supported by medical research study evidence. But in a small study, five in ten study participants with NMOSD who were unresponsive to corticosteroids plus plasma exchange responded favorably to IVIg. IVIg may be given alone or in combination with an immune-suppressive drug called azathioprine. Another medication that may be given when a person with NMOSD who is not responsive to first-line treatment (during an acute inflammatory attack) include cyclophosphamides (an immunosuppressive drug often given to treat lymphoma), particularly if a person has NMOSD along with lupus erythematosus or other types of autoimmune diseases.

Long-term Treatment

There is no prescription drug that has been identified for long-term suppression of NMOSD attacks. But several drugs may be given with a goal of preventing future attacks that often result in chronic (long-term) disabilities. Immunosuppressive drugs (medications that suppress the immune system) that are commonly given for long-term treatment of NMOSD include:

Azanthioprine and mycophenolate mofetil are often given alone with low dosages of corticosteroids. Rituximab has been found to be effective for those who do not respond well to first-line immunosuppressant treatments such as AZA and MMF.
Common side effects of immunosuppressant medications may include:

  • Nausea
  • Vomiting
  • Diarrhea
  • An increase in susceptibility to infection

Studies on Preventative Prescriptions

Since 2008, the clinical research focused on immunosuppressive drugs including azathioprine, rituximab, and mycophenolate mofeitil. Nearly every study has reported benefits from these medications.

Symptoms Treatment

Prescriptions to treat symptoms of NMOSD may include:

  • Tegretol (carbamazepine) is an anti-convulsant that decreases nerve impulses. It may be given in low doses to control spasms that commonly result from attacks.
  • Baclofen or tizanidine are antispasmodics. These may be given for long-term symptoms of spasticity that frequently occurs as a result of permanent motor (muscle movement) deficits in NMOSD.
  • Amitriptyline or Cymbalta (duloxetine) are anti-depressants that may be recommended to treat depression that commonly occurs in chronic debilitating diseases such as NMOSD.
  • Tramadol and opiates are analgesics that might be prescribed for pain control.

Specialist-Driven Procedures

Plasma Exchange (PLEX)

Some people who are having an acute attack of NMSDO do not respond favorably to methylprednisolone (the first line of treatment for acute attacks of NMSDO).
Those who do not respond well to corticosteroids may be given a procedure called plasma exchange (a procedure that involves removing some of the plasma (the fluid portion of the blood) from the blood. Next, blood cells are extracted from the plasma and then, the blood cells are mixed with a replacement solution and returned to the body.

The primary goal of the plasma exchange is to lower the level of NMO-IgG (anti-AQP4 antibody) in the blood.

Plasmapheresis

An autoimmune disease involves a malfunction of the immune system. Normally the body develops proteins called antibodies that identify foreign invaders (such as viruses) and destroys them. In those with NMOSD, the antibodies attack normal cells and tissues of the spinal cord, optic nerve and certain areas of the brain, instead of attacking foreign invaders. One type of treatment, called plasmapheresis is able to stop the malfunction of the immune cells by removing the blood plasma that contains the malfunctioning antibodies.

Plasmapheresis is also a procedure aimed at removing anti-AQPR antibodies from the blood. Plasmapheresis differs from plasma exchange in that it removes a smaller amount of plasma from the blood (usually less than 15% of the total blood volume. It does not require a person to get replacement fluid.

A 2013 study found that plasmapheresis was well tolerated and 50% of the study participants who received plasmapheresis had a signficant improvement immediately after the procedure was complete. Plasmapheresis also resulted in a significant decline in serum levels of anti-AQP4.

Home Remedies and Lifestyle

There are no proven home remedies or lifestyle improvements for the treatment of NMOSD. However, a diet high in vitamin D and fatty acids is thought to help to suppress the immune system. But, no one should ever employ a diet in place of standard treatment modalities that are backed by clinical research studies.

Vitamin D (calcitriol) is considered a steroid-like hormone, that is produced in the kidneys. Steroids (short for corticosteroids) are synthetic drugs that closely resemble cortisol, a hormone that your body produces naturally. Steroids work by decreasing inflammation and reducing the activity of the immune system; they are synthetic (man-made) drugs used to treat a variety of inflammatory diseases and conditions. Steroids are commonly used to decrease inflammation and reduce the activity of the immune system in the treatment of NMOSD

Studies on Vitamin D

There are very few research studies on vitamin D for the treatment of NMOSD. A 2018 study of vitamin D (that did not focus on NMOSD) notes that it regulates immune cell function. A study conducted in 2014 discovered a link between vitamin D deficiency and NMOSD. The study authors wrote, “patients with NMOSD can be of high risk for vitamin D deficiency and we recommend the screening of vitamin D levels in these patients.”

The study authors further wrote, “The association of vitamin D levels and disease disability implies that vitamin D may have a modulating effect on disease course in NMOSD, although the causal-effect relationship is not certain.“

Study on Fatty Acids

Researchers from Isfahan University of Medical Sciences in Isfahan, Iran, examined brain scans of 126 patients with MS and 68 patients with NMOSD who underwent MRI assessments of the brain and spinal cord. The study participants were given a questionnaire about dietary intake of fatty acids; they were also given an Expanded Disability Status Scale (EDSS) test and a fatigue questionnaire.

The study authors concluded that there was a link between the intake of saturated fatty acids (SFA’s)—considered bad fats—in people with MS (multiple sclerosis) and NMOSD.  The study authors wrote, “Dietary intakes of PUFAs [polyunsaturated fatty acids/good fats] can decrease EDSS in all patients with MS or NMOSD and decreases fatigue scale in NMOSD patients.”

The study also found that eating healthy polyunsaturated fats, such as those found in salmon, avocados, olives, olive oil and more, and limiting saturated fatty acids (such as those found in animal fat and other sources) resulted in lower levels of fatigue and less incidence of disability in people with NMOSD.

A person with NMOSD should always consult with their health care provider before starting any type of home remedy—including diet or lifestyle changes.

A Word From Verywell

Neuromyelitis optica spectrum disorder is a chronic, debilitating disease that has no cure. But, just like in other incurable diseases, there is still some hope. People with NMOSD are encouraged to become educated on available treatment options that are effective in providing palliative (comfort promoting) and preventative effects.

 It’s also vital to develop new coping skills and reach out to as many support people/systems as possible. For those who are newly diagnosed with NMOSD,an important part of your treatment plan is to start building a support network. Attending support groups and becoming involved in online support resources will help to equip you with the armor needed to effectively cope with the disease, on a daily basis. 

Open communication with the healthcare team will enable your healthcare provider/s to offer treatment options (such as pain or anti-depressant medications) that can be a vital tool for dealing with NMOSD on a long-term basis. 

Keep in mind that new medications that help to prevent relapses, such as Soliris, are on the horizon, so try not to give up hope. Last, but not least, although the future may hold a promise for a cure, don’t focus intensely on the future, instead, try to live each day in the here and now. Letting go of the things you cannot control (such as future attacks) and taking control of those you can (like reaching out to a support network) can help enable people with NMOSD to live the highest possible quality of life.

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Article Sources

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