Neuromyelitis optica

Overview

Neuromyelitis optica (NMO) is a central nervous system disorder that primarily affects the eye nerves (optic neuritis) and the spinal cord (myelitis). NMO is also known as neuromyelitis optica spectrum disorder or Devic's disease. It occurs when your body's immune system reacts against its own cells in the central nervous system, mainly in the optic nerves and spinal cord, but sometimes in the brain.

The cause of neuromyelitis optica is usually unknown, although it sometimes appears after an infection, or it can be associated with another autoimmune condition. Neuromyelitis optica is often misdiagnosed as multiple sclerosis (MS) or perceived as a type of MS, but NMO is a distinct condition.

Neuromyelitis optica can cause blindness in one or both eyes, weakness or paralysis in the legs or arms, painful spasms, loss of sensation, uncontrollable vomiting and hiccups, and bladder or bowel dysfunction from spinal cord damage. Children can have confusion, seizures or coma with NMO. Neuromyelitis optica flare-ups might be reversible, but they can be severe enough to cause permanent visual loss and problems with walking.

Diagnosis

Your doctor will perform a thorough evaluation to rule out other nervous system conditions that have signs and symptoms similar to neuromyelitis optica.

To diagnose your condition, your doctor will review your medical history and symptoms and perform a physical examination. You might also undergo:

  • Neurological examination. A neurologist will examine your movement, muscle strength, coordination, sensation, memory and thinking (cognitive) functions, and vision and speech. An eye doctor (ophthalmologist) also might be involved in your exam.
  • MRI. This imaging test uses powerful magnets and radio waves to create a detailed view of your brain, optic nerves and spinal cord. Your doctor might be able to detect lesions or damaged areas in your brain, optic nerves or spinal cord.
  • Blood tests. Your doctor might test your blood for the autoantibody NMO-IgG, which helps doctors distinguish NMO from MS and other neurological conditions. This test helps doctors make an early diagnosis of NMO. A myelin oligodendrocyte glycoprotein (MOG-IgG) antibody test also might be used to look for another inflammatory disorder that mimics NMO.
  • Lumbar puncture (spinal tap). During this test, your doctor will insert a needle into your lower back to remove a small amount of spinal fluid. Doctors test the levels of immune cells, proteins and antibodies in the fluid. This test might help your doctor differentiate NMO from MS.

    In NMO, the spinal fluid might show markedly elevated white blood cells during NMO episodes, greater than normally seen in MS, although this doesn't always happen.

  • Stimuli response test. To learn how well your brain responds to stimuli such as sounds, sights or touch, you'll undergo a test called evoked potentials or evoked response tests.

    Doctors attach small wires (electrodes) to your scalp and, in some cases, your earlobes, neck, arm, leg and back. Equipment attached to the electrodes records your brain's responses to stimuli. These tests help your doctor find lesions or damaged areas in the nerves, spinal cord, optic nerve, brain or brainstem.

Treatment

Neuromyelitis optica can't be cured, though long-term remission is sometimes possible with the right management. NMO treatment involves therapies to reverse recent symptoms and prevent future attacks.

  • Reversing recent symptoms. In the early stage of an NMO attack, your doctor might give you a corticosteroid medication, methylprednisolone (Solu-Medrol), through a vein in your arm (intravenously). You'll be given the medication for about five days, and then the medication will be tapered off slowly over several days.

    Plasma exchange is frequently recommended as the first or second treatment, usually in addition to steroid therapy. In this procedure, some blood is removed from your body, and blood cells are mechanically separated from fluid (plasma). Doctors mix your blood cells with a replacement solution and return the blood to your body.

    Doctors can also help manage other possible symptoms, such as pain or muscle problems.

  • Preventing future attacks. Doctors might recommend that you take a lower dose of corticosteroids over time to prevent future NMO attacks and relapses.

    Your doctor might also recommend taking a medication that suppresses your immune system. Immunosuppressive medications that may be prescribed include azathioprine (Imuran, Azasan), mycophenolate (Cellcept) or rituximab (Rituxan).


Content From Mayo Clinic Updated:
© 1998-2020 Mayo Foundation for Medical Education and Research (MFMER). All rights reserved. Terms of Use