Brain & Nervous System More Neurological Diseases Neuromyelitis Optica Spectrum Disorder Guide Neuromyelitis Optica Spectrum Disorder Guide Overview Symptoms Causes Diagnosis Treatment An Overview of Neuromyelitis Optica Spectrum Disorder By Sherry Christiansen Sherry Christiansen is a medical writer with a healthcare background. She has worked in the hospital setting and collaborated on Alzheimer's research. Learn about our editorial process Sherry Christiansen Medically reviewed by Medically reviewed by Claudia Chaves, MD on February 09, 2020 Claudia Chaves, MD, is board-certified in cerebrovascular disease and neurology with a subspecialty certification in vascular neurology. Learn about our Medical Review Board Claudia Chaves, MD Updated on February 12, 2020 Print Table of Contents View All Symptoms Causes Diagnosis Treatment Next in Neuromyelitis Optica Spectrum Disorder Guide Symptoms of Neuromyelitis Optica Spectrum Disorder Neuromyelitis optica spectrum disorder (NMOSD) is a rare disease of the brain and spinal column. The primary symptoms involve inflammation of the optic nerve (optic neuritis) and spinal column (myelitis). 1:34 Myelin Sheath and the Role It Plays in MS The condition is chronic. It often begins during childhood, but it can also begin in adulthood (often during a person’s 40s). It is characterized by severe, debilitating symptoms that can result in blindness and paralysis. These severe symptoms result from what is called “demyelinating autoimmune inflammatory process” which affects the central nervous system (the brain and spinal column). Other common names for NMOSD include:NMODevic diseaseOptic neuromyelitisNeuromyelitis optica (NMO)Opticomyelitis andresr / Getty Images What Is Demyelinating Autoimmune Inflammatory Process? Demyelinating autoimmune inflammation occurs when the immune system attacks the covering of the nerves, called the myelin sheath. The myelin sheath is designed to protect the nerves. When this protective layer is damaged, inflammation and injury to the nerve fibers occur, eventually leading to multiple areas of scarring (called sclerosis). In NMOSD, the nerves that are involved are primarily the optic nerves (nerves that carry signals from the eyes to the brain) and the spinal cord. Symptoms Common symptoms of NMOSD may include: Eye painLoss of visionWeakness in the upper and lower extremities (arms and legs)Paralysis of the upper and lower extremitiesLoss of bladder controlLoss of bowel controlUncontrollable hiccups and vomiting The condition usually involves episodes that may occur months or even years apart. Between flare-ups, some people are symptom-free. This asymptomatic period is referred to as being in remission. The cardinal symptoms of NMOSD come from one of two processes, including: Optic neuritis: This is inflammation of the optic nerve, leading to pain inside the eye and a disturbance in clear vision (acuity). Either one eye is affected (unilateral optic neuritis) or both eyes could be symptomatic (called bilateral optic neuritis).Myelitis: This is sometimes referred to as “transverse myelitis” because of the involvement of the motor, sensory, and autonomic function (the nerves that govern bowel and bladder control). Transverse myelitis can cause sudden impairment of sensation, motor control, and autonomic function (sometimes within 24 hours). Symptoms of NMOSD may be identical to those of transverse myelitis with an unknown cause. Symptoms of myelitis may include: Headaches and back painPain in the arms, legs or spineMild to severe paralysis (called paraparesis when it’s partial paralysis and paraplegia when it’s a complete loss of sensation and motor function)Loss of bladder or bowel controlSensory loss In the initial stages of the disorder, NMOSD can be easily confused with multiple sclerosis (MS) and is often misdiagnosed. Medical experts used to believe that NMOSD was simply a form of multiple sclerosis, but today they think that perhaps it is a different condition altogether. Although some of the symptoms are very similar, symptoms are more severe in those with NMOSD than in those with MS. Another difference is that those with NMOSD often have vision problems in both eyes, while multiple sclerosis is more commonly known to cause vision problems in just one eye. Types There are two types of NMOSD, including: Relapsing form: This involves flare-ups that occur with periods of recovery between episodes. This type is the most common form of NMOSD, with women more likely to be affected than men.Monophasic form: This consists of a single episode lasting 30 to 60 days. Men and women are equally apt to get this type of NMOSD. Complications Possible complications of neuromyelitis optica spectrum disorder include: Blindness or visual impairmentParalysis of one limbStiffness, weakness, or muscle spasmsLoss of bladder or bowel controlDepressionChronic fatigue Causes Although the exact cause of NMOSD has not yet been discovered, over 90% of those with NMOSD report that they have no known relatives with the condition. In half the cases, people diagnosed reported having a family history of some type of autoimmune disease. This indicates a strong possibility that NMOSD is linked with a genetic predisposition. But the underlying cause of autoimmunity is not well understood. NMOSD is one of a diverse group of diseases which involve a specific protein that functions as an antibody (called the aquaporin-4 [AQP4] autoantibody). While antibodies normally attack and kill pathogens such as bacteria and viruses, when a person has an autoimmune disease such as NMOSD, some of the antibodies attack the person’s own proteins instead of attacking pathogens. Experts feel that autoimmune diseases such as NMOSD are a result of the body’s immune system guarding against an invading organism, such as a virus or a disease. The body then begins to mistakenly attack healthy tissue for some reason that is not well understood. Diagnosis There are several tests aimed at diagnosing NMOSD. Your health care provider may perform several diagnostic tests if NMOSD is suspected, including: A magnetic resonance imaging (MRI) scan of the brain and spinal cordVarious tests to evaluate the function of the optic nervesBlood tests to check for biomarkers (measurable substances in which, when present, can predict the presence of a disease)Spinal fluid samples to check for a biomarker called an AQP4-Ab (this biomarker can differentiate between multiple sclerosis and NMOSD) Treatment NMOSD is considered incurable, but according to a 2016 study, there are several treatment modalities that can be tried including: Corticosteroid medication: It is given intravenously during an acute attack for approximately five days to help improve symptomsImmunosuppressants: Prevent further relapses by slowing down the immune system’s attack on the nerves. These agents include Azathioprine, Rituximab, and the recently FDA-approved Eculizumab.Plasmapheresis: A procedure found to effectively remove anti-AQP4 antibodies from the blood.Pain medicationsSpasticity (stiffness) medicationsMedications to help bladder control A Word from Verywell The debilitating symptoms that can occur in those with NMOSD can be devastating, particularly considering this is a relatively young person’s disease. The symptoms could progress to paralysis, blindness, or they may even involve the muscles that control breathing. Because of the wide range of different symptoms of NMOSD, each person’s prognosis and treatment will be different. Taking control of the things you can do, such as building a support network—as well as learning to let go of the things you cannot control—are vital coping skills for those with NMOSD who aim to maintain the highest quality of life possible. Symptoms of Neuromyelitis Optica Spectrum Disorder Was this page helpful? Thanks for your feedback! Sign up for our Health Tip of the Day newsletter, and receive daily tips that will help you live your healthiest life. Sign Up You're in! Thank you, {{form.email}}, for signing up. There was an error. Please try again. What are your concerns? Other Inaccurate Hard to Understand Submit Article Sources Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy. Johns Hopkins Medicine. Neuromyelitis optica. Updated 2020. National Organization of Rare Diseases (NORD). Neuromyelitis optica spectrum disorder. Updated September 12, 2018. Additional Reading Shaygannejad MV, Azarbayejani R, Mirmosayyeb O. Assessment of coping strategies in neuromyelitis optica spectrum disorder and multiple sclerosis patients. Multiple Sclerosis and Related Disorders. November 2018;26:253-254. doi:10.1016/j.msard.2018.10.076