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If you’ve been diagnosed with neuromyelitis optica spectrum disorder (NMOSD), it’s natural to wonder what the future holds. You may have fears about whether your symptoms will get worse or how the condition could affect your life expectancy. In this article, we’ll explore the outlook for people living with NMOSD, including common challenges and new hope offered by modern treatment options.
NMOSD, formerly known as Devic’s disease, is a rare autoimmune condition. In most cases, it occurs when certain immune proteins, called antibodies, mistakenly attack a protein called aquaporin 4 (AQP4). AQP4 helps manage water levels in the central nervous system (CNS). In some cases, these antibodies are not found, leading to what is called seronegative NMOSD. This disease primarily damages the optic nerves, spinal cord, and brain, often leading to further harm to myelin (the protective coating around nerve fibers). Myelin is essential for transmitting signals that control sensation and movement in the body.
Although NMOSD and multiple sclerosis (MS) both involve myelin damage, they’re different diseases with unique causes and treatments.
Unfortunately, NMOSD is typically a lifelong condition. NMOSD occurs in one of two forms — relapsing or monophasic. Around 90 percent of people with NMOSD experience the relapsing form, in which relapses (periods of attacks) alternate with remission (periods with no attacks) over months or years.
The less common monophasic form causes a single, severe attack without a subsequent relapse. However, the definition of this form is uncertain, as relapses can occur many years later. A minimum of five years without relapse is typically used to confirm a monophasic form, but some people relapse 10 years after their attack.
Although neither form goes away on its own, NMOSD can be managed with treatments that reduce relapses and limit long-term damage.
The course of NMOSD is different for everyone. Relapses most commonly involve optic neuritis (painful loss of vision) and transverse myelitis (swelling in the spinal cord). Optic neuritis happens more often in people under 30, whereas transverse myelitis is more common in older adults. For those with AQP4 antibodies, around 60 percent of people who have a myelitis episode will experience another attack within a year. Quick treatment is important to prevent serious, lasting damage.
NMOSD tends to come and go in cycles, with periods of flares (attacks) and remission. Doctors often call this a relapsing-remitting pattern. Some people may go months or years between episodes of active disease.
Repeated attacks can damage the CNS. Injuries called lesions often form on the brain or spinal cord and lead to serious disabilities, such as vision loss, difficulty walking, and even paralysis. A 2017 study found that about 25 percent of people with NMOSD had less mobility within two years of their disease starting. However, new treatments may help lower this risk.
Although attacks themselves don’t worsen, the damage they cause can lead to a gradual decline in neurological (brain and movement) function. Generally, the higher the number of relapses, the greater the risk that the disease will get worse. This is why preventing the attack is key in NMOSD.
During an NMO attack, especially one affecting the spinal cord or brainstem, weakness, numbness, or trouble moving the arms and legs can occur. This type of attack can even lead to breathing problems, which may be a life-threatening emergency.
If left untreated, NMOSD can lead to permanent disabilities that may shorten your lifespan. Early studies in people with untreated NMOSD showed mortality rates as high as 33 percent within five years of diagnosis. (“Mortality rate” refers to the percentage of people who die from a condition.) However, life expectancy for people with NMOSD has improved a lot in recent years. Mortality rates are now estimated to be 5 percent or lower within 10 years of diagnosis, thanks to early and ongoing treatment that can also make a big difference in quality of life.
Vision loss is a common concern in NMOSD because the optic nerves, which carry signals from your eyes to your brain, can be damaged during relapses. Without treatment, repeated attacks can lead to severe, sometimes permanent vision loss, including blindness. About 50 percent of people with untreated NMOSD may become legally blind in at least one eye within five years of diagnosis. However, advances in treatments for preventing relapses have reduced the risk of blindness.
Certain factors can increase the chances of having a more severe course of disease. These risk factors include your age when NMOSD is diagnosed, certain comorbidities (co-occurring medical conditions), and delays in diagnosis.
Studies show that NMOSD most commonly starts in a person’s 30s or 40s, though it can occur in a wide age range. People diagnosed later in life often experience more intense relapses and have a harder time recovering.
Many people with NMOSD also have comorbidities. About 30 percent have immune-related conditions such as systemic lupus erythematosus, rheumatoid arthritis, Sjögren’s syndrome, or myasthenia gravis. Others may have nonimmune conditions. Researchers believe that having multiple health issues at the same time may make it harder for the body to recover from relapses and could increase the risk of complications.
Getting an early and accurate diagnosis is crucial to prevent severe complications, as untreated attacks can damage the CNS. Diagnosing NMOSD can be challenging, especially because it can be mistaken for multiple sclerosis. The two conditions require different treatments, and some MS treatments can make NMO worse.
Treatment of NMOSD has advanced a lot in recent years, with clinical trials leading to the approval of medications designed specifically for seropositive NMOSD. One major breakthrough is the use of targeted therapies, which can significantly lower the frequency and severity of relapses, reducing the risk of long-term disability from attacks. These medications work by focusing on the specific immune pathways involved in NMOSD.
Targeted medications for NMOSD include:
Access to these newer drugs can vary, but other treatment options are available off-label (not for their approved purpose). These medications include traditional immunosuppressants, such as rituximab and tocilizumab. During attacks, corticosteroid treatment and plasma exchange may be used to help limit damage. With more treatment options than ever before, many people with NMOSD are likely to find something that works.
Read more about treatment options for NMOSD.
The outlook for people with NMOSD has improved tremendously, thanks to advances in research and treatment. Although NMOSD is a lifelong autoimmune disorder, many people can manage their symptoms well and enjoy a high quality of life. If you develop new or worsening symptoms, talk to your healthcare team right away. Early diagnosis and consistent treatment are key to improving your outlook and receiving the best possible care.
MyMSTeam is the social network for people with multiple sclerosis and their loved ones. On MyMSTeam, more than 218,000 members come together to ask questions, give advice, and share their stories with others who understand life with MS.
Have you been diagnosed with neuromyelitis optica spectrum disorder? Do you worry about your outlook? Share your experience in the comments below, or start a conversation by posting on your Activities page.
Chiara Rocchi, M.D.
completed medical school and neurology residency at Polytechnic Marche University in Italy.
Learn more about her here.
Zoe Owrutsky, Ph.D.
earned her Bachelor of Science from the University of Pittsburgh in 2014 and her Ph.D. in neuroscience from the University of Colorado Anschutz Medical Campus in 2023.
Learn more about her here.
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