Skip to main content
U.S. Department of Health & Human Services
National Institutes of Health
NCATS

COVID-19 is an emerging, rapidly evolving situation.

Get the latest public health information from CDC: https://www.coronavirus.gov (link is external)
Get the latest research information from NIH: https://www.nih.gov/coronavirus (link is external)

National Center for Advancing and Translational Sciences National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences
1-888-205-2311
Menu Search
1-888-205-2311
Menu
  • Home
  • Diseases expand submenu for Diseases
    • Browse A-Z
    • Find Diseases By Category expand submenu for Find Diseases By Category
      • Autoimmune / Autoinflammatory diseases
      • Bacterial infections
      • Behavioral and mental disorders
      • Blood Diseases
      • Chromosome Disorders
      • Congenital and Genetic Diseases
      • Connective tissue diseases
      • Digestive Diseases
      • Ear, Nose, and Throat Diseases
      • Endocrine Diseases
      • Environmental Diseases
      • Eye diseases
      • Female Reproductive Diseases
      • Fungal infections
      • Heart Diseases
      • Hereditary Cancer Syndromes
      • Immune System Diseases
      • Kidney and Urinary Diseases
      • Lung Diseases
      • Male Reproductive Diseases
      • Metabolic disorders
      • Mouth Diseases
      • Musculoskeletal Diseases
      • Myelodysplastic Syndromes
      • Nervous System Diseases
      • Newborn Screening
      • Nutritional diseases
      • Parasitic diseases
      • Rare Cancers
      • RDCRN
      • Skin Diseases
      • Viral infections
    • List of FDA Orphan Drugs
    • GARD Information Navigator
    • FAQs About Rare Diseases
  • Guides expand submenu for Guides
    • Patients, Families and Friends expand submenu for Patients, Families and Friends
      • How to Find a Disease Specialist
      • Tips for the Undiagnosed
      • Support for Patients and Families
      • Tips for Finding Financial Aid
      • Help with Travel Costs
      • How to Get Involved in Research
      • FAQs About Chromosome Disorders
      • Medical and Science Glossaries
    • Healthcare Professionals expand submenu for Healthcare Professionals
      • Caring for Your Patient with a Rare Disease
      • ICD Coding for Rare Diseases
      • FindZebra Diagnosis Assist Tool
    • Researchers expand submenu for Researchers
      • Finding Funding Opportunities
    • Teachers and Students expand submenu for Teachers and Students
      • Teaching Resources
  • News expand submenu for News
    • In The Spotlight
    • News Archive
    • Connect with GARD
    • Media Requests
  • About GARD expand submenu for About GARD
    • Contact GARD
    • About GARD
    • Videos
    • Brochures and Publications
  • En Español expand submenu for En Español
    • Enfermedades
    • Contacte GARD
    • Guías de Información expand submenu for Guías de Información
      • Preguntas Más Frecuentes Sobre Enfermedades Raras
      • Como Encontrar un Especialista en su Enfermedad
      • Consejos Para una Condición no Diagnosticada
      • Consejos Para Pacientes y Familias
      • Consejos Para Obtener Ayuda Financiera Para Una Enfermedad
      • Participe en Estudios de Investigación
      • Preguntas Más Frecuentes Sobre los Trastornos Cromosómicos
    • Sobre GARD
  • Home
© Positive Exposure
banner showing people with disabilities and/or their relatives
  1. Home
  2. Diseases
  3. Neuromyelitis optica spectrum disorder
print
  • Table of Contents expand submenu for Table of Contents
    • Summary
    • Symptoms
    • Cause
    • Diagnosis
    • Treatment
    • Statistics
    • Related Diseases
    • Research
    • Organizations
    • Learn More
    • GARD Answers
    • References
  • Browse A-Z
  • Find Diseases By Category expand submenu for Find Diseases By Category
    • Autoimmune / Autoinflammatory diseases
    • Bacterial infections
    • Behavioral and mental disorders
    • Blood Diseases
    • Chromosome Disorders
    • Congenital and Genetic Diseases
    • Connective tissue diseases
    • Digestive Diseases
    • Ear, Nose, and Throat Diseases
    • Endocrine Diseases
    • Environmental Diseases
    • Eye diseases
    • Female Reproductive Diseases
    • Fungal infections
    • Heart Diseases
    • Hereditary Cancer Syndromes
    • Immune System Diseases
    • Kidney and Urinary Diseases
    • Lung Diseases
    • Male Reproductive Diseases
    • Metabolic disorders
    • Mouth Diseases
    • Musculoskeletal Diseases
    • Myelodysplastic Syndromes
    • Nervous System Diseases
    • Newborn Screening
    • Nutritional diseases
    • Parasitic diseases
    • Rare Cancers
    • RDCRN
    • Skin Diseases
    • Viral infections
  • List of FDA Orphan Drugs
  • GARD Information Navigator
  • FAQs About Rare Diseases
You can help advance
rare disease research!
You can help advance rare disease research!
toolbox
Find out how with the NCATS Toolkit.

Neuromyelitis optica spectrum disorder


Información en español Title


How can we make GARD better? We want to hear from you.
Provide Feedback

Other Names:
Devic syndrome; NMO; Devic's neuromyelitis optica; Devic syndrome; NMO; Devic's neuromyelitis optica; Devic disease; NMO spectrum disorder; Neuromyelitis optica; Neuromyelitis optica spectrum disorders See More
Categories:
Eye diseases; Nervous System Diseases
GARD Information Navigator

GARD Information Navigator

Try our interactive tool for help finding information, services, experts, financial aid, and more!

Summary Summary


Listen
Neuromyelitis optica spectrum disorders (NMOSD) affect the spinal cord and optic nerves (nerves that carry visual messages to and from the brain). Symptoms include pain, weakness, bowel and bladder problems, and temporary vision loss. NMOSD usually occurs in adulthood, but symptoms may start at any age. Some people have a single attack of symptoms lasting months, but in most people the symptoms come and go over time. People with NMOSD may develop permanent muscle weakness and vision loss. The cause of NMOSD is unknown. It occurs when the body's immune system mistakenly attacks healthy cells in the spinal cord and eyes. It can be diagnosed by a clinical exam, MRI looking for specific signs, and blood tests looking for certain antibodies. Treatment is focused on managing the symptoms and preventing relapses.[1][2][3][4]
Last updated: 6/18/2020

Symptoms Symptoms


Listen
The following list includes the most common signs and symptoms in people with neuromyelitis optica spectrum disorders (NMOSD). These features may be different from person to person. Some people may have more symptoms than others and symptoms can range from mild to severe. This list does not include every symptom or feature that has been described in this condition.

Signs and symptoms may include:[1][2]
  • Inflammation of the optic nerve (optic neuritis)
  • Temporary vision loss
  • Inflammation of the spinal cord (acute transverse myelitis)
  • Pain
  • Abnormal sensations
  • Weakness in the arms and legs
  • Bladder and bowel control problems
  • Episodes of nausea and vomiting
Symptoms typically begin in adulthood but can start at any age. Optic neuritis tends to occur suddenly and causes eye pain and varying degrees of vision loss. Transverse myelitis develops over hours or days. Most people with NMOSD have episodes or "attacks" of symptoms months or years apart (the relapsing form), while others have a single episode lasting several months. NMOSD may cause permanent disability. 
Last updated: 6/18/2020

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Showing of 15 |
Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Autoimmune antibody positivity 0030057
Functional abnormality of the bladder 0000009
Myelitis
Inflammation of spinal cord
0012486
Neuronal loss in central nervous system
Loss of brain cells
0002529
Ocular pain
Eye pain
0200026
Optic neuritis 0100653
Paraplegia
Leg paralysis
0010550
Peripheral demyelination 0011096
Sensory impairment 0003474
Visual loss
Loss of vision
Vision loss
[ more ]
0000572
30%-79% of people have these symptoms
Abnormality of brain morphology
Abnormal shape of brain
Abnormality of the brain
[ more ]
0012443
5%-29% of people have these symptoms
CSF pleocytosis 0012229
Nausea 0002018
Recurrent singultus
Recurrent hiccup
0100247
Respiratory failure 0002878
Showing of 15 |
Do you have more information about symptoms of this disease? We want to hear from you.
Last updated: 1/1/2021

Cause Cause


Listen
The cause of neuromyelitis optica spectrum disorders (NMOSD) is unknown. It is considered an autoimmune disease in which the immune system mistakenly attacks cells in the spinal cord and optic nerves. Many people who develop NMOSD have another autoimmune disease. It is also possible that genetic factors may be involved.[1][3]

Last updated: 6/18/2020

Diagnosis Diagnosis


Listen
A diagnosis of neuromyelitis optica spectrum disorder (NMOSD) is based upon a clinical examination looking for the presence of characteristic symptoms and imaging studies (MRI) of the brain, spinal cord, and eyes.[4] Additional testing may include a blood test looking for a specific type of antibody and a spinal tap to collect a small amount of fluid that surrounds the brain and spinal cord to look for white blood cells.[3]
Last updated: 6/18/2020

Treatment Treatment


Listen
Treatment for neuromyelitis optica spectrum disorders is focused on managing the symptoms and preventing relapses. High-dose steroids, medications that suppress the immune system, and pain medications may be used. Plasma exchange, a procedure for removing excess proteins from the blood, may also be used for severe symptoms.[2][3]

Specialists involved in the care of someone with a neuromyelitis optica spectrum disorder may include:
  • Neurologist
  • Ophthalmologist
  • Physical therapist
  • Occupational therapist
Last updated: 6/18/2020

FDA-Approved Treatments

The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition. Learn more orphan products.

  • Satralizumab (Brand name: Enspryng) - Manufactured by Genentech
    FDA-approved indication: Enspryng is indicated for the treatment of neuromyelitis optica spectrum disorder (NMOSD) in adult patients who are anti-aquaporin-4 (AQP4) antibody positive.
    National Library of Medicine Drug Information Portal
  • Eculizumab (Brand name: Soliris) - Manufactured by Alexion Pharmaceuticals, Inc
    FDA-approved indication: June 2019, eculizumab (Soliris) was approved for the treatment of neuromyelitis optica spectrum disorder (NMOSD) in adult patients who are anti-aquaporin-4 (AQP4) antibody positive.
    National Library of Medicine Drug Information Portal
    Medline Plus Health Information
  • Inebilizumab (Brand name: Uplizna) - Manufactured by Viela Bio
    FDA-approved indication: Uplizna is indicated for the treatment of neuromyelitis optica spectrum disorder (NMOSD) in adult patients who are anti-aquaporin-4 (AQP4) antibody positive.
    National Library of Medicine Drug Information Portal

Statistics Statistics


Listen
It has been estimated that between 0.5 - 4.4/100,000 people has a neuromyelitis optica spectrum disorder. It has been reported to be more prevalent in Asians and Africans.[1][2][3]
Last updated: 6/18/2020
Do you have updated information on this disease? We want to hear from you.

Related Diseases Related Diseases


Listen

Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.

Conditions with similar signs and symptoms from Orphanet
Differential diagnoses include MS; idiopathic, viral, paraneoplastic and connective tissue disease (e.g. SLE)-associated myelitis; ischemic and connective tissue associated optic neuropathies (see these terms).
Visit the Orphanet disease page for more information.

Research Research


Listen

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Neuromyelitis optica spectrum disorder. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Patient Registry

  • The Autoimmune Registry supports research for Neuromyelitis optica spectrum disorder by collecting information about patients with this and other autoimmune diseases. You can join the registry to share your information with researchers and receive updates about participating in new research studies. Learn more about registries.

Organizations Organizations


Listen

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

  • Guthy-Jackson Charitable Foundation
    10525 Vista Sorrento Pkwy, #210
    San Diego, CA 92121
    Telephone: 858-638-7638
    Fax: 858-638-7698
    E-mail: info@guthyjacksonfoundation.org
    Website: http://www.guthyjacksonfoundation.org/
    The Foundation also provides a list of additional support groups for neuromyelitis optica that can be filtered by topic or type of group: https://guthyjacksonfoundation.org/support-groups/
  • Siegel Rare Neuroimmune Association (SRNA)
    1787 Sutter Parkway
    Powell, OH 43065-8806
    Toll-free: 1-855-380-3330 (Helpline)
    Telephone: +1-614-317-4884
    E-mail: https://www.surveymonkey.com/r/MyelitisHelpLine
    Website: https://wearesrna.org/
    (Formerly the Transverse Myelitis Foundation)

Social Networking Websites

  • Visit the following Facebook groups related to Neuromyelitis optica spectrum disorder:
    MOG Antibody Support And Info

Organizations Providing General Support

  • American Autoimmune Related Diseases Association (AARDA)
    19176 Hall Road, Suite 130
    Clinton Township, MI 48038
    Toll-free: 800-598-4668
    Telephone: 586-776-3900
    Fax: 586-776-3903
    E-mail: aarda@aarda.org
    Website: https://www.aarda.org/
Do you know of an organization? We want to hear from you.

Learn More Learn More


Listen

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • The Mayo Clinic Web site provides further information on Neuromyelitis optica spectrum disorder.
  • Genetics Home Reference (GHR) contains information on Neuromyelitis optica spectrum disorder. This website is maintained by the National Library of Medicine.
  • The National Institute of Neurological Disorders and Stroke (NINDS) collects and disseminates research information related to neurological disorders. Click on the link to view information on this topic.
  • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

  • The Merck Manual for health care professionals provides information on Neuromyelitis optica spectrum disorder.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Neuromyelitis optica spectrum disorder. Click on the link to view a sample search on this topic.

GARD Answers GARD Answers


Listen

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

Have a question? Contact a GARD Information Specialist.

References References


  1. Huda S, Whittm D, Bhojak M, Chamberlain J, Noonan C, Jacob A. Neuromyelitis optica spectrum disorders. Clin Med (Lond). 2019; 19(2):169-176. https://pubmed.ncbi.nlm.nih.gov/30872305.
  2. Wu Y, Zhong L, Geng J. Neuromyelitis optica spectrum disorder: Pathogenesis, treatment, and experimental models. Mult Scler Relat Disord. 2019; 27:412-418. https://pubmed.ncbi.nlm.nih.gov/30530071.
  3. Bruscolini A, Saccetti M, La Cava M, et al. Diagnosis and management of neuromyelitis optica spectrum disorders - An update. Autoimmun Rev. 2018; 17(3):195-200. https://pubmed.ncbi.nlm.nih.gov/29339316.
  4. Wingerchuk DM, Banwell B, Bennett JL, et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 2015; 85(2):177-189. https://pubmed.ncbi.nlm.nih.gov/26092914.
  5. Weinshenker B. Neuromyelitis Optica Spectrum Disorder. NORD. Updated 2018; https://rarediseases.org/rare-diseases/neuromyelitis-optica/.
Do you know of a review article? We want to hear from you.
You can help advance
rare disease research!
You can help advance rare disease research!
toolbox
Find out how with the NCATS Toolkit.

Share this content:

Share this content:

twitter-icon facebook-icon contact-icon link-icons

Copy Link

Link copied to your clipboard.

printer-icon

You May Be Interested In

How to Find a Disease Specialist

How to Find a Disease Specialist

Tips for the Undiagnosed

Tips for the Undiagnosed

Support for Patients and Families

Support for Patients and Families

Tips for Finding Financial Aid

Tips for Finding Financial Aid

Help with Travel Costs

Help with Travel Costs

How to Get Involved in Research

How to Get Involved in Research

FAQs About Chromosome Disorders

FAQs About Chromosome Disorders

Medical and Science Glossaries

Medical and Science Glossaries

Caring for Your Patient with a Rare Disease

Caring for Your Patient with a Rare Disease

ICD Coding for Rare Diseases

ICD Coding for Rare Diseases

FindZebra Diagnosis Assist Tool

FindZebra Diagnosis Assist Tool

Finding Funding Opportunities

Finding Funding Opportunities

Teaching Resources

Teaching Resources

placeholder for the horizontal scroll slider Back to top
GARD Home NCATS Home Site Map Browse Glossary A-Z Privacy Notice Disclaimer Accessibility FOIA OIG

If you have problems viewing PDF files, download the latest version of Adobe Reader

For language access assistance, contact the NCATS Public Information Officer

Genetic and Rare Diseases Information Center (GARD) - PO Box 8126, Gaithersburg, MD 20898-8126 - Toll-free: 1-888-205-2311

contact gard Office of Rare Disease Research Facebook Page Office of Rare Disease Research on Twitter
Listen