Neuromyelitis optica spectrum disorder (NMOSD) is a rare disease that affects the brain and spinal cord. It is caused by the body’s immune system attacking a protein called aquaporin-4 (AQP4), which leads to inflammation and damage to the nervous system.
The disease mostly affects women between the ages of 20 and 50. Symptoms of NMOSD can include pain behind the eye, vision loss, weakness, numbness or tingling in the limbs, loss of bladder or bowel control, and uncontrollable vomiting or hiccups.
Doctors diagnose NMOSD by looking at the patient’s symptoms and doing laboratory tests. Treatment for NMOSD usually involves taking medications that help to reduce inflammation and prevent further attacks. Some of the common treatment options for NMOSD are:
- Corticosteroids: These medications help to reduce inflammation in the body and are often used to treat acute attacks of NMOSD.
- Immunomodulators: These medications work by modifying the immune system’s response and reducing inflammation. Off-label medication examples include azathioprine, mycophenolate mofetil, and methotrexate.
- Plasma exchange (plasmapheresis): This procedure involves removing the patient’s blood plasma, which contains the harmful antibodies that are attacking the nervous system. The plasma is then replaced with healthy donor plasma.
- Rehabilitation: People with NMOSD may need physical therapy or occupational therapy to help with their symptoms and improve their quality of life.
- Monoclonal antibodies: These are targeted medications that help to prevent NMOSD attacks. They work by binding to specific cells or proteins in the body. Rituximab is an example of a monoclonal antibody that in the past was commonly used to treat NMOSD before there were any FDA approved medications. FDA approved medications include Uplizna (inebilizumab-cdon), Soliris (eculizumab), and Enspryng (satralizumab-mwge).
While rituximab and Uplizna both work by targeting B cells in the immune system, Uplizna specifically targets a type of B cell called a CD19+ B cell, whereas rituximab targets CD20+ B cells. Uplizna has been shown to be effective in reducing the frequency of NMOSD attacks in clinical trials. Uplizna infusion times are at lot shorter than rituximab infusions. Uplizna only takes 90 minutes and is given twice a year. Uplizna was designed to lower the risk of infusion reactions. If you are still treated with rituximab, you should inquire with your neurologist, whether you are a candidate for the FDA approved medication Uplizna instead.
Soliris (eculizumab) works by blocking a protein in the blood called complement component 5 (C5). This protein is part of the body’s immune system, and it helps to fight off infections and other harmful things. By blocking the C5 protein, Soliris helps to reduce inflammation and prevent further damage to the nerves in the brain and spinal cord. Soliris is given as an infusion, which means it is injected into the person’s vein. It is usually given every two weeks and takes about 1-2 hours.
Enspryng (satralizumab) works by blocking a protein in the body called interleukin-6 (IL-6). This protein is part of the immune system, which helps to protect the body from infections and other harmful things. By blocking the IL-6 protein, satralizumab helps to reduce inflammation and prevent further damage to the nerves in the brain and spinal cord. Satralizumab is given as an injection under the skin, similar to how people with diabetes inject insulin. It is usually given every four weeks.
There is no cure for NMOSD, but early diagnosis and treatment can help people with the disease to maintain a good quality of life and independence.
Our neurology experts specialize in the treatment of people living with NMOSD. Please contact our office for an appointment (appointments@inirehab.com) or call us at 1-410-828-4629.