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Neuromyelitis Optica (NMO)

What is Neuromyelitis Optica (NMO)?

NMO is an autoimmune condition that typically leads to recurrent episodes of optic neuritis and transverse myelitis. It is caused by a confused immune system that mistakenly attacks the optic nerve and spinal cord. It can also attack the brain but this occurs less than the optic nerve and spinal cord. Most patients have elevated levels of anti-aquaporin-4 antibodies (anti-AQP4). NMO can present in both children and adults. 

What are the signs and symptoms of Neuromyelitis Optica (NMO)?

The signs and symptoms of NMO are based on which part of the central nervous system is affected. Patients typically develop optic neuritis and/or transverse myelitis. Optic neuritis, which is inflammation of the optic nerve, typically leads to eye pain and vision loss. Transverse myelitis, which is inflammation of the spinal cord, can cause paralysis, sensory disturbances or changes involving bladder and bowel functions.

How is Neuromyelitis Optica (NMO) diagnosed?

Children presenting with symptoms consistent with NMO will be evaluated in a detailed neurological exam. Further diagnostic testing can include and MRI of the optic nerve, brain and spinal cord to identify possible abnormalities as well as a lumbar puncture to obtain cerebrospinal fluid (CSF) to evaluate for signs of inflammation or infection. Blood work is also performed to look for anti-AQP4 antibodies. Other testing will be done to rule out other potential causes as well. 
 

How is Neuromyelitis Optica (NMO) treated?

Treatment during the acute episode is aimed at reducing the ongoing inflammation. Potential therapies include high doses of steroids or plasma exchange (PLEX). Steroids work by reducing the number of inflammatory cells in the brain. They can be administered orally or intravenously (IV). Most children do not have side effects, but some may experience mood changes, appetite changes, difficulty sleeping or irritability. Rarely, patients can experience infection or bone abnormalities. PLEX is used to clean the blood of inflammatory components. It requires special IV access and a portable machine to clean the blood. A patient’s blood is circulated through the machine and proteins such as antibodies are removed. A therapy session takes about 90 minutes and is usually well tolerated. A patient usually receives five to seven treatments to complete a PLEX course. 

Due the relapsing nature of NMO, patients will also need to start a preventative medication that works to suppress the immune system to prevent future attacks. This decision will be made after discussing all the options with your child’s health care provider. These medications cause more profound immune suppression and should be used by centers that have experience in prescribing these medications.

In addition to evaluation by a neuropsychologist for cognitive, emotional and/or behavioral problems, patients may also require psychosocial support or other therapies to manage the impacts of NMO.

Neuromyelitis Optica (NMO) Doctors and Providers