• NINDS Neuromyelitis Optica Information Page

    Information about Neuromyelitis Optica from the National

    Institute of Neurological Disorders and Stroke (NINDS)


    NMO or Devic Disease

    Devic’s Syndrome

    Table of Contents (click to jump to sections)

    What is Neuromyelitis Optica ?


    MRI in NMO

    Neuromyelitis optica (NMO) is an uncommon disease syndrome of the central nervous system (CNS) that affects the optic nerves
    and spinal cord. Individuals with NMO develop optic neuritis, which causes pain in the eye and vision loss, and transverse
    myelitis, which causes weakness, numbness, and sometimes paralysis of the arms and legs, along with sensory disturbances and
    loss of bladder and bowel control. NMO leads to loss of myelin, which is a fatty substance that surrounds nerve fibers and
    helps nerve signals move from cell to cell. The syndrome can also damage nerve fibers and leave areas of broken-down tissue.
    In the disease process of NMO, for reasons that aren’t yet clear, immune system cells and antibodies attack and destroy myelin
    cells in the optic nerves and the spinal cord.

    Historically, NMO was diagnosed in patients who experienced a rapid onset of blindness in one or both eyes, followed within
    days or weeks by varying degrees of paralysis in the arms and legs. In most cases, however, the interval between optic neuritis
    and transverse myelitis is significantly longer, sometimes as long as several years. After the initial attack, NMO follows
    an unpredictable course. Most individuals with the syndrome experience clusters of attacks months or years apart, followed
    by partial recovery during periods of remission. This relapsing form of NMO primarily affects women. The female to male ratio
    is greater than 4:1. Another form of NMO, in which an individual only has a single, severe attack extending over a month or
    two, is most likely a distinct disease that affects men and women with equal frequency. The onset of NMO varies from childhood
    to adulthood, with two peaks, one in childhood and the other in adults in their 40s.

    In the past, NMO was considered to be a severe variant of multiple sclerosis (MS) because both can cause attacks of optic
    neuritis and myelitis. Recent discoveries, however, suggest it is a separate disease. NMO is different from MS in the severity
    of its attacks and its tendency to solely strike the optic nerves and spinal cord at the beginning of the disease. Symptoms
    outside of the optic nerves and spinal cord are rare, although certain symptoms, including uncontrollable vomiting and hiccups,
    are now recognized as relatively specific symptoms of NMO that are due to brainstem involvement.

    The recent discovery of an antibody in the blood of individuals with NMO gives doctors a reliable biomarker to distinguish
    NMO from MS. The antibody, known as NMO-IgG, seems to be present in about 70 percent of those with NMO and is not found in people with MS or other similar conditions.


    NMO Antibody test

    Is there any treatment?

    There is no cure for NMO and no FDA-approved therapies, but there are therapies to treat an attack while it is happening,to reduce symptoms, and to prevent relapses. Therapies used in the U.S. include a corticosteroid drug (methylprednisolone)to stop the initial attack, immunosuppressent drugs such as mycophenolate mofetil (CellCept) and azathioprine (Imuran), andthe monoclonal antibody ritixumab (Rituxan). If frequent relapses occur, some individuals may need to continue a low doseof steroids for longer periods. Plasma exchange (plasmapheresis) is a technique that separates antibodies out of the bloodstream and is used with people who are unresponsive to corticosteroid therapy. Pain, stiffness, muscle spasms, and bladderand bowel control problems can be managed with the appropriate medications and therapies. Individuals with major disabilitywill require the combined efforts of occupational therapists, physiotherapists, and social services professionals to address their complex rehabilitation needs.


    What is the prognosis?


    Most individuals with NMO have an unpredictable, relapsing course of disease with attacks occurring months or years apart.
    Disability is cumulative, the result of each attack damaging new areas of myelin. Some individuals are severely affected by
    NMO and can lose vision in both eyes and the use of their arms and legs. Most individuals experience a moderate degree of
    permanent limb weakness from myelitis. Muscle weakness can cause breathing difficulties and may require the use of artificial
    ventilation. The death of an individual with NMO is most often caused by respiratory complications from myelitis attacks.


    What research is being done?


    The National Institute of Neurological Disorders and Stroke (NINDS) conducts research related to NMO in laboratories at the
    NIH and also supports additional research through grants to major medical institutions across the country. Much of this research
    focuses on finding better ways to prevent, treat, and ultimately cure rare neurological syndromes such as NMO.


    NIH Patient Recruitment for Neuromyelitis Optica Clinical Trials


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    Multiple Sclerosis Foundation
    6520 North Andrews Avenue
    Ft. Lauderdale,
    http://www.msfocus.orgTel: 954-776-6805
    888-MSFOCUS (673-6287)Fax: 954-351-0630
    National Eye Institute (NEI)
    National Institutes of Health, DHHS
    31 Center Drive, Rm. 6A32 MSC 2510
    http://www.nei.nih.govTel: 301-496-5248
    National Organization for Rare Disorders (NORD)
    55 Kenosia Avenue
    http://www.rarediseases.orgTel: 203-744-0100
    Voice Mail 800-999-NORD (6673)Fax: 203-798-2291

    Publicaciones en Español

    Prepared by:

    Office of Communications and Public Liaison

    National Institute of Neurological Disorders and Stroke

    National Institutes of Health

    Bethesda, MD 20892

    NINDS health-related material is provided for information purposes only and does not necessarily represent endorsement by
    or an official position of the National Institute of Neurological Disorders and Stroke or any other Federal agency. Advice
    on the treatment or care of an individual patient should be obtained through consultation with a physician who has examined
    that patient or is familiar with that patient’s medical history.

    All NINDS-prepared information is in the public domain and may be freely copied. Credit to the NINDS or the NIH is appreciated.

    Last updated June 4, 2014


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