Neuromyelitis optica

Diagnosis and treatment

To diagnose neuromyelitis optica, a healthcare professional first reviews your medical history and symptoms and does a physical exam. That may include a neurological exam, which involves checking movement, muscle strength, coordination, sensation, memory, thinking, vision and speech.

If your healthcare professional suspects NMO based on symptoms, then you may have various tests. The diagnosis process includes ruling out other nervous system conditions that have similar symptoms.

Tests to diagnose neuromyelitis optica may include:

• Blood tests. A blood test can be done to look for the antibody that binds to proteins and causes NMO. That antibody is called aquaporin-4-immunoglobulin G (AQP4-IgG). Testing for this antibody can tell the difference between NMO and MS and make an early diagnosis of NMO.

  Blood tests also may be done to diagnose an NMO relapse. Those tests look for substances, called biomarkers, such as serum glial fibrillary acidic protein (GFAP) and serum neurofilament light chain. A test for myelin oligodendrocyte glycoprotein immunoglobulin G (MOG-IgG) antibody also might be used to check for another inflammatory condition that has symptoms like those of NMO.

• MRI. This imaging test uses a magnetic field and radio waves to create a detailed view of organs inside the body. The results may show lesions or damaged areas in the brain, optic nerves or spinal cord.
• Lumbar puncture, also called a spinal tap. During this test, a needle is put into the lower back to remove a small amount of spinal fluid. This test shows the levels of immune cells, proteins and antibodies in the fluid. The test can help show whether symptoms are being caused by NMO or by MS.
• Optical coherence tomography. This test looks at the retinal nerve fiber layer and its thickness. People who have inflamed optic nerves from NMO have more vision loss and retinal nerve thinning than do people with MS.

• Stimuli response test. To learn how well the brain responds to stimuli such as sounds, sights or touch, a test called an evoked potentials test or evoked response test may be used.

  For this test, wires called electrodes are attached to the scalp and, sometimes, the earlobes, neck, arms, legs and back. Equipment attached to the electrodes records the brain's responses to stimuli. These tests help find lesions or damaged areas in the nerves, spinal cord, optic nerve, brain or brainstem.

Neuromyelitis optica can't be cured. But treatment sometimes can lead to long periods with no symptoms, called remission. NMO treatment involves therapies to ease symptoms and lower the risk of relapse.

• Easing symptoms. In the early stage of an NMO attack, a healthcare professional might give a corticosteroid medicine such as methylprednisolone (Solu-Medrol) to reduce symptoms. It's given through a vein in the arm. The medicine is taken for about five days. Typically, it's then tapered off slowly over several days.

  Plasma exchange often is recommended as treatment, usually in addition to corticosteroids. In this procedure, some blood is taken from the body, and blood cells are mechanically separated from the fluid in the blood called plasma. The blood cells are mixed with a replacement fluid, and the blood is returned to the body. This process can remove harmful substances from the blood.

• Lowering the risk of relapses. A lower dose of corticosteroids taken over time may help prevent future NMO attacks.

  Medicines called monoclonal antibodies have been shown to be effective in lowering the risk of NMO relapses in adults. These medicines include:

  • Eculizumab (Soliris).
  • Inebilizumab (Uplizna).
  • Ravulizumab (Ultomiris).
  • Rituximab (Rituxan).
  • Satralizumab (Enspryng).

  Medicines that suppress the immune system also may help prevent relapses. Those medicines include:

  • Azathioprine (Imuran, Azasan).
  • Mycophenolate mofetil (Cellcept, Myhibbin).
  • Methotrexate (Trexall, Xatmep, others).
  • Tocilizumab (Actemra).

  Medicine called intravenous immunoglobulins (IVIg) also may lower the number of NMO relapses.

You're likely to start by seeing your primary healthcare professional. But when you call to make an appointment, you may be referred to a doctor trained in brain and nervous system conditions, called a neurologist.