Symptoms of neuromyelitis optica spectrum disorder are almost identical to multiple sclerosis. But this rare disease has challenges all its own.

Maybe you wound up in the ER because you suddenly couldn’t see out of one or both eyes. Or perhaps you felt painful muscle spasms just trying to move your arms or legs. Or maybe you woke up with uncontrollable hiccups, nausea, and vomiting that wouldn’t let up. Regardless of which scenario is like yours, we bet your thinking was the same: *Something is seriously wrong here, and I need help.*

That something has a name: __neuromyelitis optica spectrum disorder (NMOSD)__. And, while rare, this condition, which affects approximately 15,000 people in the U.S., is known for symptoms such as vision loss or even blindness, weakness, muscle spasms, serious mobility issues, and, sometimes, permanent paralysis. “Symptoms usually come on over 24 to 48 hours and last for days to weeks, if not months,” says Oliver Tobin, M.B., associate professor of neurology at Mayo Clinic College of Medicine in Rochester, MN. 

While this autoimmune disorder can appear out of the blue, NMOSD can have long-lasting health ramifications. Here’s what to expect if you’re being screened for NMOSD, or are newly diagnosed. 

## Before Diagnosis: Testing, Testing, Testing 

If your doctor suspects NMOSD, he or she first needs to rule out multiple sclerosis (MS). Both diseases involve a haywire immune response that attacks the central nervous system (CNS), which is comprised of your brain, optic nerves, and spinal cord. That means both conditions can cause serious eye pain and vision problems from __optic neuritis__—when the optic nerve is attacked by rogue immune cells and becomes inflamed, causing vision loss and eye pain—as well as serious, and sometimes permanent, mobility problems.

Ruling out MS early on is essential “because the overwhelming majority of MS drugs do not work for NMOSD, and some of them can make NMOSD worse,” says Florian P. Thomas, M.D., director of the Multiple Sclerosis Center at Hackensack University Medical Center in New Jersey. “We suspect NMOSD essentially in every patient who presents with conditions that could be MS because it’s absolutely crucial to treat NMOSD with drugs of dramatic effectiveness from the get-go.”

To determine which condition you have, your doctor will likely conduct a series of tests. They include:

- __Bloodwork__, which screens for a specific antibody that signals NMOSD (more on this in a moment).

- __Magnetic resonance imaging (MRI)__, which delivers detailed scans of the CNS, detecting specific locations of scarring. In MS, lesions tend to be smaller and are commonly found on brain tissue. In NMOSD, the target is usually the spinal cord or optic nerve (or a related area called the optic chiasm), with the brainstem being attacked in just 10% of cases. Lesions from NMOSD are usually elongated and cover a cross-section of the nerve, one of reasons why symptoms tend to affect both limbs, or both eyes, whereas in MS, symptoms generally occur in a single eye, arm, or leg. 

- A __spinal tap__, which is performed to examine cerebrospinal fluid that runs through the spinal column. In NMOSD, this may reveal AQP4 antibodies or high levels of inflammatory proteins called cytokines. If you have high levels of a protein called immunoglobulin instead, you probably have MS.

- __Optical coherence tomography (OCT)__, which often shows much more thinning of the nerve fiber in the retina in people with NMOSD than in people with MS.

- __Evoked potentials test__, also called an evoked responses test, which allows your doctor to review how your brain and optic nerves are responding to sounds, sights, and touch, and helps detect other damaged areas in your CNS. 

## While You Wait for Results: Inflammation Is Treated

An official NMOSD diagnosis can take a few weeks as you wait for results from bloodwork to come back, says Dr. Thomas. Until then, he says that—just as if you’d had a serious MS flare—you’ll be given a high dose of __intravenous (I.V.) corticosteroids__ for about five days to tame inflammation and reduce symptoms. (That’s because while preventative MS drugs can harm people with NMOSD, many common symptoms of both conditions are treated the same.)

Steroids are the first line of defense in this initial or any future attack—which 90% of people with this condition do go on to experience. That’s because there are two main types of NMOSD:

- __Relapsing-remitting__ is the most common form, meaning symptoms come on for weeks or months—called a relapse—then remit, or go into remission, only to reappear again down the line.

- __Monophasic__, which is a one-and-done form of the disease, affects just 10% of people with NMOSD, with symptoms generally being more severe during the single attack, but then never happening again. However, since doctors won’t be able to tell for months or even years which type you have, you’ll be treated as if you have relapsing-remitting.

“One of the biggest differences between MS and NMOSD is that, as far as we know, there is no progressive phase of NMOSD,” says Dr. Tobin. While MS symptoms flare and then abate during remissions, and sometimes (but not always) worsen over time, each NMOSD attack can cause accumulative or even permanent damage. Which is why powerful new treatments are focused on preventing the next attack from ever occurring.

After you receive I.V. steroids, if your symptoms are severe enough your doctor may order a few sessions of __plasma exchange__—a treatment that removes inflammatory antibodies from your blood to help curb symptoms. In 75% of people with NMOSD, the immune system produces antibodies that target the protein __aquaporin-4__, which is responsible for transporting water in certain nerve cells. Those antibodies, which are not present in MS, appear to be the main cause of the damaging inflammation. 

The presence of aquaporin-4 antibodies, also known as __NMO-IgG__, can help confirm the diagnosis.

“MS can actually be more difficult to diagnose than NMOSD, because once the blood test for aquaporin-4 is positive, NMOSD is diagnosed,” explains Mary Rensel, M.D., a neurologist at Cleveland Clinic’s Mellen Center for MS Treatment and Research in Ohio. 

Still, she says that some people with NMOSD actually receive negative antibody tests. Somewhere around 20% of people with NMOSD don’t have AQP4-IgGA—but about half of this group do have antibodies for a different protein called __myelin oligodendrocyte glycoprotein (MOG)__, which is found in the outer layer of the myelin sheath and can result in similar attacks on the optic nerve and spinal cord. Since their symptoms and other test results fit the disease, they’re given the diagnosis of NMOSD (although there is ongoing debate in the research community about whether the presence of MOG antibodies might be a different disease altogether). 

## Day 1 of Diagnosis: Learn About NMOSD (and Have Hope)

If your doctor just told you have NMOSD, you’re probably feeling a wide range of emotions. Maybe you’re relieved to have answers, even as you’re feeling shocked that your life has taken such a sudden turn. Try to give yourself time to process the news and all it means, says Dr. Tobin. And, remember, while there are general trends seen in a typical NMOSD disease course, you are an individual. No one can definitively predict your future, or how you will respond to treatment.

Still, if you’re like most others with a new diagnosis, chances are good you’ve already Googled NMOSD—and are feeling pretty alarmed by what you’ve found. “Historical accounts of this disease are very, very bad,” Dr. Tobin acknowledges. But, he adds, “The advent of newer disease-modifying therapies in the last few years will significantly impact that history. I think the future looks very promising.” So try to bank on hope.

## First Week: Discuss Medications (and Consider Joining a Clinical Trial)

There’s never a good time to get NMOSD—but the good news is that your diagnosis has come now. “The landscape for NMOSD has changed dramatically just in the last two years, in terms of what treatments are available,” Dr. Tobin says. 

Once your attack has been stopped with I.V. steroids (and maybe plasma exchange), the next step is to prevent future attacks and pre-empt more damage. While no treatment yet available is 100% effective, these drugs have been to shown to reduce the severity and frequency of relapses:

- __More steroids:__ Your doctor may prescribe a low dose of a corticosteroid like Medrol (methylprednisolone) or Prednisone Intensol (prednisone) to help prevent future NMOSD attacks and to tame inflammation. 

- __Rituximab:__ You’ll get two courses of this I.V. infusion, a monoclonal antibody, in the first two weeks, and then one every six months. In a 2014 study, researchers took a 10-year retrospective look at 90 NMOSD patients and found that those taking rituximab cut their relapse rate by 88%, with two out every three patients achieving full remission. In fact, it’s been a mainstay of NMOSD treatment for more than a decade, says Dr. Thomas. “It’s a very effective drug. I’ve had patients with NMOSD who’ve been stable on it for 15 years.” However, rituximab does have potential side effects, he says, including fever, chills, rash, headache, nausea, vomiting, shortness of breath, and more.

- __Off-label Immunosuppressants:__ These medications help shut down the rogue and inflammatory immune response, and may prevent the next NMOSD attack from happening. They include:

  -   CellCept (mycophenolate mofetil)
  -   Imuran (azathioprine)

- __NOMSD-specific Drugs:__ For the 75% or so of people who test positive for NMO-IgE, the FDA has approved two immunosuppressants: __Soliris__ (eculizumab) and __Uplizna__ (inebilizumab) that have been shown in clinical trials to help prevent future attacks. A third drug, __Enspryng__ (satralizumab) is widely believed to be approved soon. They are infusions with different delivery methods (every two weeks for Soliris versus every six months for Uplizna) and hefty price tags, though insurance may cover most of these costs. 

## 1 to 2 Weeks After Diagnosis: Assemble Your Care Team

As soon as you're strong enough, it’s time to get strategic: Get ready for quite a few medical appointments in the coming weeks and months as you and your doctor come up with a treatment plan going forward.

Since NMOSD is so rare, it’s a good idea to __find a neurologist__ who’s familiar with the condition, advises Dr. Rensel. If possible, she recommends reaching out to an MS center for recommendations. You may be able to do some of your appointments virtually if you don’t live near a center.

Your neurologist will help you put together a __care team__ to address your individual issues. Depending on your needs and symptoms, this care team is similar to one someone with MS might have, bringing together a wide range of practitioners that can include:

- an __ophthalmologist__ for eye pain and vision loss from optic neuritis
- a __nurse practitioner__ who you can see more frequently for symptom management, and to answer questions
- a __psychologist__ to address the emotional stress of this life-changing condition
- a __neuropsychologist__ to help with any cognitive issues or decline
- a __physical therapist__ for walking, balance, and gait issues
- an __occupational therapist__ to help you figure out strategies and tools to maintain your independence at home and in the community
- a __urologist__ for bladder and constipation problems

It’s probably going to take a while to adjust to this huge life change, so be kind to yourself during this raw time, advises Dr. Rensel. She relays how at Cleveland Clinic they connect people who are newly diagnosed with NMOSD to patient advocacy groups such as the [Guthy-Jackson Charitable Foundation](https://guthyjacksonfoundation.org/) and the [Siegel Rare Neuroimmune Association](https://wearesrna.org/)) for support and education.

## 3 to 4 Weeks After Diagnosis: Focus on Making Healthy Changes

At this point, your initial symptoms should be getting better, but you may have some long-lasting or even permanent injury, including vision loss or walking issues. 

Dr. Tobin suggests you embrace self-care, starting with a __healthy lifestyle__. Since obesity has an impact on inflammatory diseases, he says neurologists recommend all patients maintain a normal weight. A heart-healthy diet full of fruits, vegetables, and whole grains and low on sugar, processed foods, and red meat, can help you control your weight. It also gives you energy and helps prevent heart disease. 

Getting daily exercise is important, too. Any kind is beneficial, as long as you’re doing it every day, Dr. Thomas says. So is [quitting smoking](https://www.heart.org/en/healthy-living/healthy-lifestyle/quit-smoking-tobacco/the-benefits-of-quitting-smoking-now). If you still light up, do everything in your power to quit.

## 1 to 5 Months After Diagnosis: Arrange for a New Schedule and Join a Support Group 

Hopefully by now your symptoms are well-controlled and you haven’t had a second relapse, though these can happen at any point. Some people with NMOSD only have relapses of optic neuritis (pain, vision loss, or blurring), while others only have relapses of myelitis (weakness, numbness, paralysis, spasticity, uncontrollable vomiting and nausea, uncontrollable hiccups, fatigue, and loss of bladder/bowel control). Others have both. It’s impossible to know what course your disease will take, so try to take it one day at a time.

Your emotional well-being affects your immune response, so if you’re feeling anxious, it’s important to protect yourself and get help if you need it, Dr. Rensel says. Be sure to alert your care team.

Prepare yourself for __frequent MRIs__. “We get MRIs fairly regularly on patients to monitor that the drug (they’re on) is effective in terms of preventing a relapse,” Dr. Thomas says. With both MS and NMOSD, people tend to have more relapses that are visible on an MRI (new lesions or areas of inflammation without symptoms) than ones that create symptoms (clinical relapse). “The MRI relapses are just as much tell-tale signs for disease activity as the clinical relapses, so that’s something we follow very, very closely,” says Dr. Thomas.

Dr. Thomas also notes that you may need to make some adjustments at work or school to allow for your condition. For instance:

- College students may need a specialized learning plan to give them more time to take exams if memory has been affected, which he says can occur.

- At work, you can ask to move closer to a restroom if you have bladder or bowel control problems. 

- If your neurologist put you on an NMOSD-specific medication, you may need to talk to your employer about getting time off of work or rearranging your schedule, since you’ll be getting infusions that last 35 to 90 minutes (plus about an hour of observation afterward to make sure you don’t have an allergic reaction). If you run into obstacles with your boss, head to the [Job Accommodation Network](https://askjan.org/soar.cfm) to find out more about accommodations and your rights under the [Americans with Disabilities Act](https://www.ada.gov/) (ADA). 

You may want to investigate joining a [support group](https://www.nationalmssociety.org/Resources-Support/Find-Support) with others battling this disease, whether it’s online or in-person. Talking to other people who are going through similar things can make a big impact on your emotional health. 

If you have another relapse during this time, you’ll start the process of stopping the attack with high doses of corticosteroids and possibly plasma exchange all over again. Your neurologist will reevaluate whether you’re on the best treatment and maybe make a change. You’ll also need treatment for any new or worsening symptoms.

## 6 Months After Diagnosis: Ask About Vitamin D and Do Brain Games

Provided you haven’t already had another attack, your neurologist will review all of your symptoms and ask you about any new or worsening ones. Dr. Tobin says your neurologist might start you on __vitamin D supplements__ if your levels are low. Studies have found that low levels of vitamin D may increase your risk for relapses.

You could still be experiencing residual symptoms from your initial attack—from vision changes to muscle weakness, spasticity, or paralysis—and if you’ve had another attack, your symptoms will likely get even worse since the effects of each relapse tend to build up. You’ll need to keep addressing all symptoms with your care team.

If your memory has been affected, keep your mind sharp with __apps and online brain games__. Read a novel, do a crossword puzzle, get good sleep, and move your body every day—all help prevent cognitive decline..

## 1 Year After Diagnosis and Beyond: Celebrate What’s Ahead

If all goes well and you avoid another relapse, you’ll continue with treatments, plus do six-month or yearly follow-ups to monitor your disease, says Dr. Tobin. Relapses can happen really fast, though, so if you notice any symptom changes at all, contact your neurologist right away. 

In the meantime, it’s important to stick with your treatment plan and be proactive about your mental and physical health, says Dr. Tobin. Keep all your appointments, report all problems to your care team, and make sure you have lots of support.

If it all feels overwhelming, keep in mind that new and innovative treatments are changing the forecast for NMOSD. “We’re doing a clinical trial of one of the medicines that is at the FDA now, and I have patients who are clinically stable with this condition, which is not how it used to be,” says Dr. Rensel. “It’s a bright new day or this group.”

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From diagnostic testing, lifestyle changes, plus immunosuppressant drugs and drugs to help prevent future relapses, here’s your future forecast after being told you have NMOSD, which can sometimes be mistaken for MS.

NMOSD: What to Expect

Getting diagnosis of neuromyelitis optica spectrum disorder can be scary. Knowing what’s ahead can dispel some of that fear.

Your First Week, Month, Year, and Beyond With NMOSD

- __Americans with NMOSD:__ National Multiple Sclerosis Society. (n.d.) “Neuromyelitis Optica (NMO).” [nationalmssociety.org/What-is-MS/Related-Conditions/Neuromyelitis-Optica-(NMO)](https://www.nationalmssociety.org/What-is-MS/Related-Conditions/Neuromyelitis-Optica-(NMO))

- __Rituximab Side Effects:__ Mayo Clinic. (2020.) “Rituximab (Intravenous Route): Side Effects.” [mayoclinic.org/drugs-supplements/rituximab-intravenous-route/side-effects/drg-20068057](https://www.mayoclinic.org/drugs-supplements/rituximab-intravenous-route/side-effects/drg-20068057) 

- __Eculizumab Side Effects:__ *UpToDate*. (2019.) “Neuromyelitis optica spectrum disorders: Treatment.” [uptodate.com/contents/neuromyelitis-optica-spectrum-disorders#H203479463](https://www.uptodate.com/contents/neuromyelitis-optica-spectrum-disorders#H203479463)

- __Eculizumab Cost:__ *The American Journal of Managed Care*. (2019.) “Soliris Gains FDA Approval for Neuromyelitis Optica Spectrum Disorder.” [ajmc.com/newsroom/soliris-gains-fda-approval-for-neuromyelitis-optica-spectrum-disorder](https://www.ajmc.com/newsroom/soliris-gains-fda-approval-for-neuromyelitis-optica-spectrum-disorder)

- __Preventative Medications:__ National Multiple Sclerosis Society. (n.d.) “Treatments of NMO.” [nationalmssociety.org/What-is-MS/Related-Conditions/Neuromyelitis-Optica-(NMO)/Treatments](https://www.nationalmssociety.org/What-is-MS/Related-Conditions/Neuromyelitis-Optica-(NMO)/Treatments)

- __Immunosuppressant Availability:__ *UpToDate*. (2019.) “Neuromyelitis optica spectrum disorders: Treatment.” [uptodate.com/contents/neuromyelitis-optica-spectrum-disorders#H203479463](https://www.uptodate.com/contents/neuromyelitis-optica-spectrum-disorders#H203479463)

- __Types of Relapses:__ National Organization for Rare Disorders. (2018.) “Neuromyelitis Optica Spectrum Disorder: Signs & Symptoms.” [rarediseases.org/rare-diseases/neuromyelitis-optica/](https://rarediseases.org/rare-diseases/neuromyelitis-optica/)

- __Vitamin D:__ *Journal of Clinical Neuroscience*. (2019). “Low levels of vitamin D and the relationship between vitamin D and Th2 axis-related cytokines in neuromyelitis optica spectrum disorders.” [pubmed.ncbi.nlm.nih.gov/30692037/](https://pubmed.ncbi.nlm.nih.gov/30692037/)


Shaheen Lakhan, M.D.

Medical Reviewer

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NMOSD: Mistaken for MS

Getting a diagnosis of neuromyelitis optica spectrum disorder can be scary. Knowing what’s ahead can dispel some of that fear.

It’s no wonder neuromyelitis optica spectrum disorder (NMOSD) is frequently mistaken for multiple sclerosis (MS). “Both NMOSD and MS are autoimmune disorders that damage the nervous system,” says Eric Lowell Singman, M.D., associate professor of ophthalmology at the Johns Hopkins Wilmer Eye Institute in Baltimore. With both conditions, your immune system, which normally defends you against bacteria and viruses, decides to attack your own tissues. And people with either neurological condition may experience blurred vision, numbness or tingling in their legs. 

With such similar symptoms, it took more than a century (really!) for scientists and physicians to realize that NMOSD wasn’t just a spoke of the MS umbrella. Turns out, NMOSD is distinctly different.  

## 1. NMOSD Targets a Specific Protein

Though both MS and NMOSD involve the body attacking itself and both result in inflammation, what’s doing the attacking and what’s being primarily attacked differs between the two conditions. In MS, T-cells (a.k.a. T-lymphocytes) attack myelin, the sheaths that wrap around long nerve fibers (normally these immune cells destroy invading bacteria and viruses). However, with NMOSD, it’s not T-cells that have gone awry. Instead, specialized proteins called antibodies attack AQP4, a key protein that regulates how water moves in and out of cells. And they don't attack all the AQP4 proteins in your body, only the ones present in nerve cells called astrocytes. 

Astrocytes are nerve-cell caretakers that live in the brain and spinal cord. One of their most important functions is to deliver energy to neurons and to kickstart the release of neurotransmitters, chemicals that allow the neurons to communicate with each other. If something were to damage the astrocytes, depending on the location and the extent of the damage, it can result in anything from difficulty walking to blindness.
	
## 2. Gastrointestinal Symptoms May Be Severe

“The first symptom of NMOSD may be uncontrollable nausea and vomiting for weeks,” says Dr. Pittock. (Or you may have hiccups that don’t abate for weeks on end.) These gastrointestinal (GI) symptoms are not your run-of-the-mill indigestion, reflux or nausea that can readily be resolved with an OTC drug. Rather, they are extremely severe and constant, and affect your ability to go about your daily activities. With MS, having nausea, vomiting or hiccups that won't go away is rare.

## 3. The Optic Nerve Is Hit Hard

Vision loss can occur with [NMOSD](https://www.healthcentral.com/condition/neuromyelitis-optica-spectrum-disorder-nmosd) because of severe inflammation of the optic nerve (optic neuritis), which impairs the proper transmission of nerve impulses from the back of the eye (retina) to the visual center of the brain. Often NMOSD affects the part of the brain where the two optic nerves—one from each eye—connect, referred to as the optic chiasma. That’s why the damage to vision can be bilateral. 

Your vision may be blurry, colors may appear washed out, you may lose vision in the upper or lower half of your visual field, or you may temporarily or permanently go blind in one or both eyes. You can also develop severe pain when you move your eyes, or a stabbing pain in the back of your eye. With MS, vision difficulties usually occur in just one eye, and they often improve once inflammation abates. Visual field defects, like spots, are rare with MS.

## 4. Damage Tends to Cluster in Certain Parts of the CNS

“MS damages the *entire* central nervous system,” says, Dr. Singman. This includes the optic nerves, spinal cord, and all parts of the brain: the cerebellum (which manages balance and coordination); the brainstem (the lower part of the brain that regulates numerous vital functions, including swallowing and breathing); and the cerebrum (which controls everything from memory, problem solving, and learning to  walk), continues Dr. Singman. 

Meanwhile, the damage associated with NMOSD is focused on the optic nerve, brainstem, and spinal cord. The reason people with NMOSD have uncontrolled nausea, vomiting or hiccups, for instance, is because there's damage to the area postrema on the brainstem, explains Dr. Singman.

## 5. Lesions on the Spinal Cord Are Much Larger

People with NMOSD have lesions on their spinal cord that are longer than those found in MS, often extending along several segments of the spinal cord, says Dr. Pittock. In MS, the areas of damage are generally short. These larger lesions contribute to more severe symptoms. 

## 6. Diagnosis Techniques Vary

Doctors diagnose NMOSD by taking a blood sample, looking for the antibody to AQP4 as well as one called myelin oligodendrocyte glycoprotein (MOG). “If you have this antibody, you have the disease; it is not found in the blood of patients with MS, which has no biomarker substance that tells you that you have the disease,” says Dr. Pittock. Testing for this antibody is now standard. 

Typically the diagnosis for MS is made based on symptoms, an MRI, and sometimes the results of a spinal tap to look for various immune cells and antibodies in the cerebrospinal fluid. Though your doctor might also do an MRI and spinal fluid test if they suspect NMOSD, the blood test is the definitive one.  

## 7. Standard MS Treatments Make NMOSD Worse

MS is typically treated with disease-modifying therapies (DMTs), which operate by suppressing the immune system. However, some of the DMTs are either ineffective in treating NMOSD or can actually make the condition worse. Among the DMTs that can worsen NMOSD are the new generation drugs that successfully treat MS, including Gilenya (fingolimod) and Tysabri (natalizumab). 

Unlike with MS, NMOSD has fewer treatment options. Up until 2019, doctors treated the condition with off-label drugs that had been approved for other autoimmune disorders like lupus. The FDA recently approved two immunosuppressants, Soliris (eculizumab) and Uplizna (inebilizumab), that suppress your immune system to prevent future attacks. Physicians believe a third immunosupressant drug called Enspryng (satralizumab) will also be approved by the FDA. 

## 8. Disease Progression Looks Very Different

With MS, most people do not experience permanent disability from the relapses; they, in fact, make a good recovery. When disability does occur, it’s usually with the more advanced progressive form of the disease: SPMS (secondary progressive multiple sclerosis). With SPMS, symptoms come on, but they either don’t improve or worsen without a relapse. 

“It’s different with NMOSD; the progression is related to the attacks,” says Dr. Pittock. “Between the attacks there’s not much progression.” But with each attack there is accumulated nerve damage, resulting in sensory losses (including worsening vision difficulties), problems with bladder and bowel control as well as the ability to move your muscles properly. 

For example, with MS, 90% of people recover from optic neuritis, but not so with NMOSD, which has a high likelihood of vision loss. That’s why it’s so important to stop the attacks. “If you stop the attacks, you stop the disability,” adds Pittock. 


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This head-to-toe guide features the documented differences between NMOSD and MS—including how the two conditions affect the optic nerve, spinal cord and the area postrema.

Ways NMOSD Differs From MS

Sure, on paper NMOSD sounds super similar to MS. But, in the body, the two conditions look and feel very different. 

8 Important Ways NMOSD Is Different From MS

- __NMOSD History:__ *Journal of Neuroinflammation*. (2013). “The history of neuromyelitis optica.” [ncbi.nlm.nih.gov/pmc/articles/PMC3599417/](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3599417/)

- __MS Eye Disorders vs. NMOSD Eye Disorders:__ *Neural Regeneration Research*. (2016). “Multiple sclerosis and neuromyelitis optica spectrum disorders: some similarities in two distinct diseases.”
- [ncbi.nlm.nih.gov/pmc/articles/PMC4828999/](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4828999/)

- __MS Pain vs. NMOSD Pain:__ *Journal of Clinical Neurology*. (2020). “Comparison of Neuropathic Pain in Neuromyelitis Optica Spectrum Disorder and Multiple Sclerosis.” [ncbi.nlm.nih.gov/pmc/articles/PMC6974826/](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6974826/)

- __Evolution of our Knowledge of NMOSD:__ *Current Opinion in Neurology*. (2019). “Neuromyelitis optica spectrum disorders: still evolving and broadening.” [journals.lww.com/co-neurology/fulltext/2019/06000/neuromyelitis_optica_spectrum_disorders__still.11.aspx](https://journals.lww.com/co-neurology/fulltext/2019/06000/neuromyelitis_optica_spectrum_disorders__still.11.aspx)


Main Differences Between NMOSD and MS

If you’re suddenly experiencing blurred vision, numb limbs, relentless vomiting, or hiccups that last for days on end, you probably—no, you definitely—want to know what’s going on, *stat*. But getting an accurate diagnosis for these unsettling symptoms isn't always straight forward. While they’re the classic signs of the autoimmune disease __neuromyelitis optica spectrum disorder__ (NMOSD), they can ring true for other conditions, too. So, it may take some sleuthing work before your doctor can put all the pieces together and get you on the right treatment path. Here’s why:

## 1. NMOSD is super rare—only 15,000 people in the U.S. have it. So your doctor might not suspect it at first.

NMOSD, a.k.a. Devic’s disease, accounts for just 2% of all demyelinating diseases—those that damage the myelin sheath, the protective covering on your nerves—so there’s a good chance your primary care physician has never had a single patient with the disease. NMOSD attacks myelin on the optic nerve (which carries visual info from the eyes to the brain), the spinal cord, and sometimes the brainstem, leading to a wide range of symptoms that affect vision, balance, and even bladder and bowel function. 

“Symptoms develop over a period of a few days and peak within a week to 10 days,” says Elias Sotirchos, M.D., director of the Neuromyelitis Optica Clinic at Johns Hopkins University in Baltimore. But if you were to google your symptoms (and we suspect you already have), you’ll see that there’s a lot of overlap with other conditions including multiple sclerosis (MS), lupus, and even a brain tumor. So, before your doc lands on an NMOSD diagnosis, he may consider—and potentially try to rule out—these other, more common issues first. 

## 2. Some tissues may appear normal during an exam. 

If you have blurry vision and throbbing eye pain—which can happen because NMOSD often causes a condition called optic neuritis (ON), leading to inflammation of the optic nerve—you’d expect your doc to find some evidence of it when looking into your eyes during a regular eye exam. But with NMOSD, that’s not always the case. 

“Optic disc swelling may not be present,” says Dr. Sotirchos. Getting an ON diagnosis, then, comes from other parts of an eye exam, a thorough patient history, and an optical coherence tomography (OCT) scan. This non-invasive diagnostic test can help spot swelling and damage of nerves in the back of the eye, and can also help nail an ON diagnosis, he adds. An MRI can also be done to ID which part of the nerve is involved. 

## 3. If you *do* get an ON diagnosis, multiple sclerosis (MS) may be suspected first.

ON happens in about half of all MS cases. About 20% of the time, it’s one of the first symptoms of that condition, according to research published in the journal *Eye and Brain*. So, if you go to your doctor with sudden vision loss or pain behind one or both eyes, your doctor may initially suspect MS. 

And to confuse matters more, NMOSD was once considered a variant of MS, not a completely separate disease. “Up until about 15 years ago, if a patient came in with [these symptoms], they would be diagnosed with MS,” says Patricia Coyle, M.D., interim chair of the department of neurology at Stony Brook University in Stony Brook, NY. 

## 4. If you have weakness or numbness in your limbs, injury to the spinal cord must first be ruled out.

Waking up to one or both limbs feeling weak, numb, or even paralyzed can be pretty scary. Or maybe you can’t control your bladder, or urinate at all. These are things that can happen with you have transverse myelitis (TM). TM is diagnosed through a medical history and a neurological exam, as well as an MRI to look for spinal cord lesions that are typical with TM. 

But your physician isn’t finished there. He has to figure out *why* you have it. TM can signal NMOSD or MS, but it can also stem from other autoimmune disorders, vascular causes, and infections, as well as compression from a mass or even unexplained injury. So these other possibilities may need to be considered—and then rejected—before NMOSD emerges as the likely cause of your TM symptoms.  

## 5. Seemingly benign symptoms like hiccups or vomiting (even if severe) may initially be dismissed. 

If your brainstem is the target of an NMOSD attack—a rare occurrence in an already rare disease—your symptoms, while intense, may not immediately point to this condition.

NMOSD brainstem attacks aren’t as common as ON and TM. They happen in just 10% of cases. Nausea and vomiting might be dismissed as the flu or even food poisoning. It’s only after you’ve been throwing up for days—or even weeks—that your doctor may start looking for other causes. 

Hiccups are usually benign and can be triggered from carbonated beverages, alcohol, stress, eating too much, or swallowing air. But when they linger for more than 48 hours, your doctor may suspect nerve damage or irritation caused by reflux or even a cyst or tumor, central nervous system conditions including MS and stroke, as well as metabolic diseases (such as diabetes and kidney disease) before determining they’re actually the result of an NMOSD attack.

## 6. Bloodwork to identify NMOSD-specific antibodies can take weeks to come back.

Fortunately, there are some highly specific blood tests that can tell docs if you have NMOSD. Back in 2004, researchers discovered that about 75% of people with NMOSD have antibodies in their blood called aquaporin-4 antibodies (AQP4-IgG). These antibodies target water channels (aquaporins) located on astrocytes, a type of cell in the central nervous system. “We now realize that NMOSD is [usually] an immune attack on aquaporin-4,” says Dr. Coyle. 

About 20% of people with NMOSD don’t have AQP4-IgG, but they may have another kind of antibody: myelin oligodendrocyte glycoprotein (MOG-IgG), a protein in the outer layer of the myelin sheath. Identifying these two antibodies have helped docs get to an NMOSD diagnosis faster than before (and rule out MS in one fell swoop). 

Even so, getting your results can take weeks, leaving you, well, a bundle of nerves. And a small percentage of people with NMOSD won’t have either antibody, even though they have all the classic NMOSD symptoms. If this happens to you, after your bloodwork comes back your doctor may schedule more tests, including a spinal tap and an MRI. 

## 7. You might test positive for lupus, confusing matters more.

There is some association between NMOSD and the autoimmune disorder lupus. Some people with NMOSD will test positive for antibodies associated with lupus, even if they don’t have any signs of that condition, says Dr. Sotrichos. “The overlap between NMO and lupus likely has to do with a shared genetic tendency to developing these two autoimmune diseases,” he says. 

Interestingly, those who *do* have lupus *and* symptoms of ON and TM often carry AQP4-1gG antibodies—as many as 60%, suggests a study in *Lupus*. “People with lupus with transverse myelitis, optic neuritis, or other neurological symptoms consistent with NMOSD should be tested for AQP4-IgG at that time,” Dr. Sotirchos advises. In most cases, those who are positive for AQP4-IgG have actual signs of NMOSD, suggesting it may be co-existing with lupus. 

## 8. Once you get a diagnosis, it will likely be years before your doctors can tell you definitively which type you have.

As if this rare disease isn’t complex enough, there are two types: __relapsing__, which is when you get repeated attacks with months or even years of remission in between, and __monophasic__, a single attack that lasts for at least a month or two. Your doctor won’t know which one you have right away, but your bloodwork holds some clues: AQP4-IgG-positive NMOSD is typically treated as relapsing. 

Those with MOG-IgG antibodies may have a monophasic attack, but it’s a wait-and-see game. “If patients [with MOG-IgA antibodies] are followed long enough, the vast majority will have a relapse,” says Dr. Sotirchos. So, doctors treat most cases as if they are the relapsing type. 

Now that you have an official diagnosis of NMOSD, you might sigh with relief to finally have a name for what you’ve been experiencing—even as you battle worry, or even fear. Deep breath. Know that in the past few years powerful new treatment options have emerged to reduce the likelihood of another attack occurring—to help keep you symptom-free in the future.


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Symptoms of neuromyelitis optica spectrum disorder can be confused for other autoimmune disorders—and even bloodwork can pose more questions. Here are all the reasons patience is often required to get the answers you want and need when it comes to this condition.

NMOSD Diagnosis

Even though there's a blood test for neuromyelitis optica spectrum disorder, diagnosis isn't always a snap. 

Think You Might Have NMOSD? Diagnosis May Take a While

- __General info on NMOSD:__ National Organization for Rare Disorders. (n.d.). “Neuromyelitis Optica Spectrum Disorder.” [rarediseases.org/rare-diseases/neuromyelitis-optica/](https://rarediseases.org/rare-diseases/neuromyelitis-optica/)

- __Prevalence of NMOSD:__ NYU Langone Health. (n.d.). “Neuromyelitis Optica Treatment & Research Program.” [nyulangone.org/locations/multiple-sclerosis-comprehensive-care-center/neuromyelitis-optica-treatment-research-program](https://nyulangone.org/locations/multiple-sclerosis-comprehensive-care-center/neuromyelitis-optica-treatment-research-program)

- __Diagnosis:__ The Transverse Myelitis Association. (2018). “Neuromyelitis Optica Spectrum Disorder (NMOSD).” [wearesrna.org/wp-content/uploads/2018/06/About_NMOSD_2018.pdf](https://wearesrna.org/wp-content/uploads/2018/06/About_NMOSD_2018.pdf)

- __MS and ON:__ *Eye and Brain*. (2016). “Optic Neuritis as an Early of Multiple Sclerosis.” [ncbi.nlm.nih.gov/pmc/articles/PMC5398757/](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5398757/)
 
- __Prevalence of NMOSD:__ NYU Langone Health. (n.d.). “Neuromyelitis Optica Treatment & Research Program.” [nyulangone.org/locations/multiple-sclerosis-comprehensive-care-center/neuromyelitis-optica-treatment-research-program](https://nyulangone.org/locations/multiple-sclerosis-comprehensive-care-center/neuromyelitis-optica-treatment-research-program)

- __Diagnosis:__ The Transverse Myelitis Association. (2018). “Neuromyelitis Optica Spectrum Disorder (NMOSD).” [wearesrna.org/wp-content/uploads/2018/06/About_NMOSD_2018.pdf](https://wearesrna.org/wp-content/uploads/2018/06/About_NMOSD_2018.pdf)

- __Lupus and NMOSD:__ *Lupus*. (2017). “195 Systemic Lupus Erythematosus and Neuromyelitis Optica Spectrum Disorders.” [lupus.bmj.com/content/4/Suppl_1/A91](https://lupus.bmj.com/content/4/Suppl_1/A91)

- __Monophasic vs. Relapsing NMOSD:__ *Neurology*. (2015). “International Consensus Diagnostic Criteria for Neuromyelitis Optica and Spectrum Distorders.” [ncbi.nlm.nih.gov/pmc/articles/PMC4515040](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4515040)



Guillaume Lamotte, M.D.

8 Reasons Why an NMOSD Diagnosis Might Take Some Time 

After treatment for NMOSD, it can take six months or more for nerves to recover. During that time you can experience “positive symptoms," like pain or tingling, which are actually good signs that you're healing. Experts explain how to cope with this phenomenon.

NMOSD Positive Symptoms

When you are newly diagnosed with neuromyelitis optica spectrum disorder, you’ll first need a good neurologist. But there are other healthcare professionals you’ll want to consider reaching out to. These include an ophthalmologist to keep your eyes healthy, an occupational therapist to help you adapt your life to any neurological changes, and a urologist to help with bladder symptoms. 

NMOSD Healthcare Team

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When you’re diagnosed with neuromyelitis optica spectrum disorder, these specialists can help you get back to living.

Meet Your NMOSD Healthcare Dream Team

These HealthCare Professionals Can Help With NMOSD

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When you're recovering from an attack, you may experience new symptoms that are actually positive signs that you're healing.

NMOSD Symptoms: When Not to Worry

The Positive Symptoms of NMOSD that Signal You’re Healing

Healing That Feels Anything But

Halos or Light Sensitivity

A Hyperactive Bladder

Spastic Muscles

Intense Pain

Expect Many Tough Days

Help Recovery Along

Maybe you wake up one morning with throbbing pain behind one or both eyes. Maybe you suddenly have a loss of central or color vision. Or, perhaps you have a relentless ache in your eye that gets worse with every passing minute, as your vision goes blurry. While you might be tempted to pop a pain reliever and see if things improve over the next day or two, please follow this advice: Don't wait. First of all, we're talking about your eyes here. Sudden changes to your vision should be checked out ASAP. Among the many reasons why: A serious but rare disorder called __neuromyelitis optica spectrum disorder__ (NMOSD) could be to blame. And if it's not caught and treated quickly, you're at risk for permanent damage to your eyesight. 

Sudden, severe eye pain and vision loss are often the first signs of NMOSD, an often disabling autoimmune disease that targets the optic nerves, which send information from the retina to the brain, allowing you to see. An estimated 250,000 people around the world, including about 15,000 people in the U.S., have been diagnosed with the condition, and as many as 50% experience severe visual disturbances in at least one eye from a condition called __optic neuritis__ (ON). 

ON occurs when there's damage to the optic nerves' protective coating (called myelin), usually from inflammation. Those lesions interrupt the flow of information, causing symptoms.  

“After multiple attacks, if the patient's not on appropriate treatment, the chance of developing permanent legal blindness is quite high,” says Brian Weinshenker, M.D., professor of neurology at the Mayo Clinic in Rochester, MN. “Many patients are permanently blind even after a single attack,” he adds. 

So, that's terrifying to hear, especially when the condition can seem to strike out of the blue. But know this: Treatment can help. The key is to get the right diagnosis as quickly as possible. 

## NMOSD Is Often Mistaken for MS

NMOSD, also known as __Devic's disease__, can be confused with multiple sclerosis (MS). Both conditions can cause ON, as well as serious mobility issues from damaging inflammation in the central nervous system (CNS), which is comprised of the optic nerves, brain, and spinal cord. There's so much overlap that until quite recently, NMOSD was actually considered a form of MS. 

“The older recognition of the disease was based on having both involvement of the spinal cord and the optic nerves,” explains Michael Morgan M.D., director of neuro-ophthalmology at University Hospitals Cleveland Medical Center in Ohio. 

NMOSD and MS are now recognized as distinct conditions, says Dr. Morgan. And both require specific and often different treatments to manage attacks and prevent the most serious symptoms from occurring, including blindness.

## How NMOSD Impacts the Eyes

Like multiple sclerosis, NMOSD is a relapsing-remitting disease—meaning it causes repeated relapses, called __flares__, followed by periods of __remission__, when the immune system calms down and many symptoms, or even all in the case of MS, abate.

However, NMOSD attacks are often much more severe from the get-go, so damage done to the optic nerves can be permanent with each flare—making preventing future flares all the more urgent. Those diagnosed with this condition often experience significant disabilities that never remit, even during a remission. 

Along with the increased severity of flares affecting the optic nerves, here are four other ways NMOSD affects the eyes:

- __Aquaporin-4 antibodies drive an NMOSD diagnosis:__ Dr. Weinshenker notes that in NMOSD, the immune system produces antibodies that target a specific protein, aquaporin-4, which is responsible for transporting water on the surface membrane of the eyes. Those antibodies, which are not present in MS, appear to be the main cause of the eye pain and inflammation associated with optica neuritis, leading to vision loss. Dr. Morgan notes in an estimated 70% of cases, those with NMOSD have aquaporin-4 antibodies in their blood, which helps confirm the diagnosis.
   
   Dr. Weinshenker adds, “If a patient has severe optic neuritis with poor recovery, it would be important to get these blood tests done because they would have very important treatment implications if they were positive.” 

- __MOG antibodies offer another clue:__ A second antibody, myelin oligodendrocyte glycoprotein (MOG), may also be present in NMOSD, creating a similar but distinctive variation of the disease. (If you have MOG antibodies, you won’t have aquaporin-4 antibodies; it seems no one has both.) 

[Research](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3937859/) shows that those with MOG antibodies tend to have serious optic (and less spinal) involvement, including ON in both eyes, but also have fewer attacks and better recovery than those with aquaporin-4 antibodies. It’s also important to note that the presence of MOG antibodies doesn’t always mean you have NMOSD. In fact, scientists are trying to determine if this specific disease course should be categorized as its own, separate disease. But, for now, it's still considered NMOSD.

- __Both eyes are attacked in NMOSD:__ Optic neuritis from MS tends to affect one eye at a time (and may only ever affect one eye and never the other), while ON in NMOSD has a much stronger tendency to attack both eyes at the same time.

- __Vision loss from MS is usually temporary:__ While ON symptoms can come on suddenly in both conditions, Dr. Weinshenker says that vision loss associated with NMOSD is distinct—and may be permanent, even with treatment, while people with MS generally regain their full vision within a few weeks after a relapse that includes an ON attack.
 
   “An NMOSD patient might say, ‘I notice some pain when I move my eye,’ and, by the next morning, they get up and say, ‘I can barely read,’ or, ‘There's a black spot in front of my vision in the center. When I look at your face, I can see around the edges, but I can't see the center.’ [Without treatment], over several days, it could progress even to the point of losing all ability to [see] light and dark, or maybe just a small crescent of vision [is all] that's preserved.” 
  

## It’s Difficult to Detect NMOSD Nerve Damage

The sudden onset of vision symptoms in NMOSD, combined with the severity of episodes, make early diagnosis and treatment especially important. What's confounding is that NMOSD damage is rarely visible during a conventional eye exam. In fact, the eyes can look normal.

“With this condition, you look in the eye and don't see anything wrong,” Dr. Weinshenker says. “You may see swelling of the optic nerve if it is very close to the eyeball, but, more often than not, the eye looks healthy. There is no macular degeneration, no diabetic retinopathy, no venous thrombosis. Once you rule out those other causes, it raises concerns that there is a disease involving the optic nerve.”

The most common diagnostic tests that ophthalmologists use to confirm NMOSD, in addition to antibody testing, include:

- __Ocular coherence tomography (OCT):__ This is a clinical test that measures the thinning of the retinal nerve fiber layer to assess the degree of damage. 
- __Magnetic resonance imaging (MRI):__ “The MRI shows intense inflammation along one or both optic nerves, or also potentially involving that optic chiasm,” says Dr. Morgan. “It's one of the ways that we help become more suspicious that this disease might be neuromyelitis optica. And if the inflammation involves a very long segment of the nerve, involves both nerves, stretches all the way back to the optic chiasm, or involves the optic chiasm, tan NMO spectrum disease is more likely than another kind of optic neuritis.”

## NMOSD Treatments Tame Symptoms and Prevent Relapses

The sooner an NMOSD diagnosis is made, the sooner treatments can begin. However, treating NMOSD with MS medications can actually worsen symptoms, not make them better—another reason getting the correct diagnosis is so important. 

“It's quite different than in MS. The treatment we use to prevent attacks of MS don't work in NMO, but other treatments do,” Dr. Weinshenker says. Those treatments include:

- __Injectable and oral steroids__ in high doses are often the first line of defense against an NMOSD attack. A 2019 study in the journal *Neurology, Neuroimmunology and Neuroinflammation* showed that those who received treatment with injectable steroid medications within seven days of an attack had a greater likelihood for restoring their vision than those who delayed treatment. 
- __Preventative, NMOSD-specific medications__ were first approved in June 2019 by the U.S. Food and Drug Administration (FDA) to treat people with aquaporin-4 antibodies. Soliris (eculizumab) was initially approved, followed by Uplizna (inebilizumab), with Enspryng (satralizumab) widely expected to gain approval in 2020. Clinical trials for all three showed significant reductions in NMOSD relapses and hospitalizations. 
- __Plasma exchange__ is also a treatment option. The process involves filtering out the part of the blood that carries inflammation-causing antibodies. This can help shorten an attack, Dr. Morgan explains. To be effective, the plasma exchange process must be repeated in multiple sessions, often five times over a period of 10 days. The treatment has good success rates for restoring vision, the doctor notes.
- __Corticosteroids and monoclonal antibody therapies__ are additional treatments used for NMOSD to quell the haywire immune system response. Because more than 90% of those living with this disease experience multiple, severe attacks, these drugs are used to help prevent relapse.

## Early Detection Delivers the Best Prognosis

“Almost everyone gets some improvement with these treatments, but the degree of response varies,” Dr. Morgan says. “For some patients, vision is never restored. Even for patients who do get good recovery of their vision, and even if they are able to see colors or read 20/20 in the clinic, they still [notice] that something about their vision is different following an NMOSD attack.”

The best prognosis comes with early treatment, he stresses. “In the event of sudden or very rapid vision loss and eye pain, see an eye doctor as soon as possible. We wouldn’t want someone to go several days without getting good evaluation and treatment.” It just might save your sight.


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In this rare condition, also known as Devic’s disease, your immune system mistakenly attacks your optic nerves. Sudden and permanent blindness can result when NMOSD is left untreated.

NMOSD Vision Loss

One of the scariest possible effects of neuromyelitis optica spectrum disorder is losing your sight. Here's what to know.

A Clear Look at Vision Loss Caused by NMOSD

- __NMOSD and MS (1):__ National Multiple Sclerosis Society. (n.d.). “Neuormyelitis Optica (NMO).” [nationalmssociety.org/What-is-MS/Related-Conditions/Neuromyelitis-Optica-(NMO)/Symptoms-and-Diagnosis](https://www.nationalmssociety.org/What-is-MS/Related-Conditions/Neuromyelitis-Optica-(NMO)/Symptoms-and-Diagnosis)
- __NMOSD and MS (2):__ *Korean Journal of Ophthalmology*. (2019). “Association of Optic Neuritis with Neuromyelitis Optica Spectrum Disorder and Multiple Sclerosis in Korea.” [ncbi.nlm.nih.gov/pmc/articles/PMC6372377/](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6372377/)
- __NMOSD and MS (3):__ *Neural Regeneration Research*. (2016). “Multiple sclerosis and neuromyelitis optica spectrum disorders: some similarities in two distinct diseases.” [ncbi.nlm.nih.gov/pmc/articles/PMC4828999/](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4828999/)
- __NOMSD Treatment (1):__ U.S. Food and Drug Administration. (2019). “FDA approves first treatment for neuromyelitis optica spectrum disorder, a rare autoimmune disease of the central nervous system.” [fda.gov/news-events/press-announcements/fda-approves-first-treatment-neuromyelitis-optica-spectrum-disorder-rare-autoimmune-disease-central](https://www.fda.gov/news-events/press-announcements/fda-approves-first-treatment-neuromyelitis-optica-spectrum-disorder-rare-autoimmune-disease-central)
- __NOMSD Treatment (2):__ *Neurology, Neuroimmunology and Neuroinflammation*. (2019). “Does time equal vision in the acute treatment of a cohort of AQP4 and MOG optic neuritis?” [nn.neurology.org/content/6/4/e572](https://nn.neurology.org/content/6/4/e572)


Let's Look at Vision Loss Caused by NMOSD

This severe but treatable autoimmune disorder is often mistaken for multiple sclerosis. Get the facts on what makes it unique.

NMOSD Myths and Facts

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Myths and Facts: Getting to Know NMOSD

- __An Overview of NMOSD:__ *Clinical Medicine*. (2019). “Neuromyelitis Optica Spectrum Disorders.” [ncbi.nlm.nih.gov/pmc/articles/PMC6454358/](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6454358/)
- __Clinical Presentations:__ *Rheumatic Diseases Clinics of North America*. (2017). “Neuromyelitis Optica.” [pubmed.ncbi.nlm.nih.gov/29061244/](https://pubmed.ncbi.nlm.nih.gov/29061244/)
- __Differences Between NMOSD and MS:__ National MS Society. (2019). “Neuromyelitis Optica (NMO).” [nationalmssociety.org/What-is-MS/Related-Conditions/Neuromyelitis-Optica-(NMO)](https://www.nationalmssociety.org/What-is-MS/Related-Conditions/Neuromyelitis-Optica-(NMO))


Your Guide to the Myths and Facts of NMOSD

Fact: NMOSD Is Not MS

Myth: NMOSD Is a Progressive Condition

Fact: Attacks Can Leave Lasting Damage

Myth: Vision Loss Is Always Temporary

Fact: Vomiting and Hiccups Are Symptoms

Myth: The Cause of NMOSD Is a Mystery

Fact: Spinal Cord Inflammation Is Common

Myth: NMOSD Is an Isolated Disease

Fact: Effective Treatments Exist

Myths and Facts: What to Know About NMOSD

*Maybe you woke up one morning with pain in both eyes or numbness in your leg and had no idea what was happening to you. Or, perhaps your GP referred you to a neurologist after suspecting NMOSD. Whatever the scenario, you’re probably nervous, confused, and maybe even a little scared. That’s normal! Here, we share the realities and challenges of this rare condition, but also the best treatments, helpful lifestyle changes, where to find your NMOSD community, and all the crucial information you need right this minute. We’ve got you, so keep reading.*

## What Is Neuromyelitis Optica Spectrum Disorder, Anyway?

You’d be forgiven for thinking that neuromyelitis optica spectrum disorder—NMOSD for short—was a neurological disorder. This group of inflammatory diseases is actually an autoimmune condition that affects your central nervous system (CNS), which is comprised of the spine, optic nerves, and brain. 

NMOSD is like [multiple sclerosis](https://www.healthcentral.com/condition/multiple-sclerosis) (MS) in that way. In fact, for many years, doctors thought NMOSD was a variation of MS—and some still misdiagnose people who have NMOSD with the better-known condition. But NMOSD (also sometimes called neuromyelitis optica, or just plain old NMO, its official name before 2007) is a different thing altogether. 

Similar to MS, your immune system suddenly goes haywire and attacks healthy tissues in your body. But unlike that condition, NMOSD targets a specific water-channel protein known as __aquaporin-4 (AQP4)__, which is responsible for transporting water in and out of the brain and spinal cord. Even though AQP4 is also in other parts of your body, including your lungs, the immune system focuses its attack on the protein in the CNS, carpet-bombing it with all its disease-fighting might as if it were a foreign pathogen, like invading bacteria or a virus. 

There is a variation of NMOSD that involves a different antibody. In a small subset of people—just 10% to 11%—with this condition, the immune system goes rogue on an altogether different protein called the __myelin oligodendrocyte glycoprotein, or MOG__. MOG is found on the protective coating that surrounds nerve cells, called myelin. Those who have this form of the disease tend to have less severe attacks with better recoveries.

Regardless of which protein is the target, the attack produces swelling (a.k.a. inflammation), which, 90% of the time in NMOSD, damages tissue in one of two places: 

- __The optic nerve:__ This is the nerve that connects the eye to the brain. Inflammation here can lead to blurry or obstructed vision, or even blindness—sometimes temporary, sometimes permanent.

- __The spinal cord:__ Inflammation here can lead to numbness and an inability to pee, with paralysis—again, sometimes temporary, sometimes permanent—a real possibility. 

Damage to the brain, in particular the brainstem, occurs in just 10% of cases, and it may cause uncontrollable hiccupping and horrible nausea, which usually (and thankfully) subside after a few weeks.

Vision loss and/or major mobility issues are super scary symptoms, to be sure, and most of the time you have no idea what hit you. That’s understandable. NMOSD is rare—you’ve likely never heard of it before. Here are some more quick facts:

- Roughly 15,000 people in the U.S. have NMOSD, with between one and four cases per 100,000 people being diagnosed every year. 
- Women are nine times more likely to have the most common form of NMOSD then men. 
- On average, people have their first attack at age 39.
- Children as young as 3 can have the disease (though this is incredibly rare). Children account for about 4% of NMOSD cases. 
- Seniors can get it, too, with late onset occurring any time after 55.


Basics

NMOSD stats by the numbers, including average age at diagnosis, women vs. men, and U.S. incidence.

NMOSD Stats by the Numbers

## Is the Damage From NMOSD Temporary or Permanent?

There’s no cure for NMOSD, but unlike the different types of MS, this disease doesn’t follow a progression path to more serious stages of disability.

Instead, the degree of your injuries from NMOSD depends on two things:

- The damage that’s done after the initial attack
- Whether or not you have additional attacks after the first one

With each relapse, typically more damage occurs—either to the same part of the CNS or to a different one. For instance, your first attack can leave you unable to see clearly out of one eye, or reliant on a walker to get around. But subsequent attacks can leave you mostly blind in that same eye, or forever confined to a wheelchair. Or, alternatively, a subsequent attack can target your spinal cord rather than your optic nerve (or vice versa). 

That means reducing the likelihood of another attack is key, especially since you can usually recover much of your sight and mobility between relapses—if you get treatment in time. But recovery varies from person to person, ranging from none to full, and most likely depends on the severity of the attack, how quickly you received treatment, what type of treatment you got, and even your age. This is true of repeat attacks, too, though when they happen again in the same place (say, the spinal cord) your ability to bounce back is usually pretty limited. 

Luckily, there are more treatments for NMOSD than there were even two short years ago—and doctors are finding out new things about this disease every day. While you may have to live with NMOSD from now on, we’ve got the answers you need to help you live your best life.

## What Are the Types of NMOSD?

There are two forms of NMOSD, and the difference between them comes down to whether you suffer a single attack, or whether you go to have two or more. At the outset doctors cannot tell which kind you have (so treatment is essentially the same). They are:

### Relapsing NMOSD

This is the most common type, affecting 90% of patients, according to the Guthy-Jackson Foundation, a nonprofit in Beverly Hills, CA, dedicated to funding basic and clinical research on the disease.

If you're diagnosed with relapsing NMOSD, you have a roughly 60% chance of having another attack in the first year, and a 90% chance of having one within three years. The good news is that preventative treatments can cut your chances of having relapses, and probably lessen the severity of an attack if you do have one.

### Monophasic NMOSD

This is the least common form, affecting between 5% and 10% of patients, men and women equally. This is essentially a one-and-done form of the disease—you have one bad attack that progresses quickly (in hours or days), and then you do not typically have a relapse. But doctors won’t be able to tell if you’ve had a singular event until years have passed, so they treat you as if you have the relapsing kind. 

This initial attack is usually more severe, leaving 20% of patients mostly blind in one or both eyes, and in 30% of cases, permanently paralyzed in one or both legs, too. That’s scary to read, we know. But it’s the rarer form of a very rare disease, and you’re much less likely to experience an attack again.

Types

## What Are the Risk Factors for NMOSD?

So what causes NMOSD, you ask? The short answer: It’s a mystery, as it often is for inflammatory autoimmune disorders. Scientists suspect people with NMOSD may have a genetic predisposition for the condition that gets triggered by something—a virus, say, that mimics the composition of the aquaporin-4 channel protein. After your immune system eradicates the original virus, it confuses AQP4 with the original virus and attacks the spinal cord or the optic nerve, thinking the virus is still lurking there. 

That’s the theory, anyway. Having said that, there may be other risk factors that may trigger your immune system to go into overdrive and turn on AQP4 (or the alternative protein, MOG).

Such risk factors include: 

### Family History

NMOSD isn’t a genetic condition—so far, no one has isolated the genes that could predispose you to this, and it’s extremely rare to find multiple family members who have the same condition. But many NMOSD patients have a family history of autoimmune disorders such as lupus, psoriasis, and/or Sjogren’s Syndrome. In fact, there’s some confirmed overlap with lupus—you could have NMOSD and test positive for lupus, even though you have no signs of the latter condition.

### Ethnicity 

If you’re African American, you have a greater risk of developing NMOSD. When researchers from Johns Hopkins looked at the demographics of 187 patients from three NMOSD clinics across the U.S., they found that while African Americans comprise just 13.4% of the U.S. population, they made up 37% of NMOSD cases, compared to patients who were white, who made up 48%. The remaining 15% of NMOSD cases were a combination of Latinos, Asian Americans, and Native Americans, more in keeping with general population trends, according to the U.S. Census Bureau. 

### Biological Sex

As previously noted, relapsing NMOSD, which the vast majority of people with this condition get, is like many autoimmune disorders: It strikes women far more often than it does men—in this case, nine times more, according to some estimates. 

### Gut Bacteria

You need something to trigger your immune system, and people with NMOSD seem to have more of the bacteria *C. perfringens* in their GI system than people normally do—which may mean that the immune system is constantly trying to eradicate it (and mistakenly targeting AQP4, too). The bacteria also spurs production of pro-inflammatory cells that are linked to other autoimmune disorders, as well.

### Lifestyle Factors

It’s really tough to establish cause and effect with diet and exercise—people always overestimate the servings of fish they eat or underestimate their junk food consumption. Still, Iranian researchers looked at 100 NMOSD patients and discovered that they were more sedentary than average and consumed less fish and dairy, along with fewer fruits and vegetables. This is also true for many autoimmune disorders—eating high-fat, high-salt foods, coupled with less physical activity, can up your risk for developing such diseases.

### Vitamin D Deficiency

There’s some evidence to show that NMOSD patients have low levels of vitamin D—in fact, even lower than those levels found in people with MS. The vitamin is known for regulating the immune system by keeping inflammation at bay, which is why some doctors recommend Vitamin D supplements for NMOSD patients.


Risk Factors

## How, Exactly, Does an NMOSD Attack Do Damage?

Most of the injury is done to the AQP4 water-channel protein and __astrocytes__—the special cells where most AQP4 is found. Astrocytes perform a variety of useful functions—they feed and water other brain cells and clean out brain debris (like excess proteins). So when the astrocytes are damaged, nearby neurons no longer can work properly.    

A secondary casualty of the AQP4 attack is the loss of myelin. When this protective sheath gets damaged or destroyed—as it can when your body attacks MOG proteins—your nerves can’t function as well. If your optic nerve is attacked, for example, myelin damage can slow down or even block the brain from sending signals to your eyes. If your spinal cord is the target, signals to your limbs may be affected. Similar to MS, you can also develop scars, known as lesions, on your spinal cord, optic nerve, and brain where healthy tissue used to be, and such scarring is visible on a MRI scan—another reason NMOSD was so often confused for multiple sclerosis.  

Often, the damage is so bad you usually develop symptoms in a matter of days, and sometimes even hours (which is faster than what can happen if you have MS, say). You can go to bed with a tingling in your leg and wake up the next morning to discover that it’s so numb you can’t even move it. Or you open your eyes to blurred vision. Or you’re horribly nauseous and can’t stop hiccupping, if the haywire immune response damaged to your brainstem.


## What Are the Symptoms of NMOSD?

The signs of NMOSD aren’t at all subtle. They usually happen quickly—and there’s no soft-pedaling it, the symptoms can be alarming. Still, they range in degree of seriousness and are different for everyone. Milder symptoms tend to be the exception, but they also can occur. 

Most of the time you absolutely know that something serious has happened. That’s because the optic nerve and spinal cord aren’t that thick, so any injury done to the tissue can wreak havoc on the pathways that communicate with the brain.

### If your optic nerve was targeted...

The inflammation can happen anywhere along the optic nerve (which goes from the back of your eye through the skull to the brain), and you typically have the following symptoms in one eye (though sometimes both can be involved):

- Your eye feels sore, as if someone has punched you, or it hurts when you move it up and down or side to side.
- Your vision gets obscured—it could look like there's a giant gray spot in the middle of your field of vision, or like a black curtain has come down halfway.
- Your vision is blurry or cloudy.
- You lose your vision completely in one or both eyes or are legally blind (20/200, meaning you see at 20 feet what others can spot at 200 feet). Such vision loss may be temporary or permanent, depending on the severity of the attack and how soon you receive the right treatment.

Inflammation of the optic nerve (also known as optic neuritis, or ON) tends to be more common in people younger than 35, and when children and teens develop NMOSD, it usually involves the optic nerve.

### If your spinal cord is targeted...

The inflammation can happen along any part of it, and the location influences which part of your body experiences symptoms. Symptoms are primarily __motor__ (affecting your ability to move) or __sensory__ (what you feel):

- Numbness, or a lack of sensation, in one or both of your limbs
- Weakness in your arms or legs, ranging from mild to an inability to move them
- Tingling sensations in your arms or legs, or itchiness, or burning sensations
- Inability to pee or move your bowels for hours, even when you have to go 
- Feelings of tightness, either around your stomach, legs, or arms, or around your neck and into your scalp
- Pain in the back or neck
- Paralysis in one or both limbs

Spinal cord attacks (also known as transverse myelitis, or TM) are more common among those over 35, although no one knows why.

### If your brain is targeted...

An attack here is super rare, but when it does occur, it usually affects a small area called the postrema that controls vomiting. Those symptoms last two or three weeks and can be intense, but they go away (even on their own) and usually don’t come back. They include:

- Terrible nausea
- Vomiting
- Uncontrollable hiccupping


Symptoms

6 ways NMOSD can affect you, including complications like eye problems, muscle spasms, and fatigue.

INFO NMOSD revCOMPLICATION-1

6 Ways NMOSD Can Affect You

## When Should I See a Doctor for NMOSD?

It won’t even be a question in your mind—if you cannot see out of one eye or are unable to pee for a day you’ll want to get yourself checked out STAT. If you have ON, an ophthalmologist will perform an exam called an ophthalmoscopy to check for swelling at the back of the eye and may give you steroids before referring you to a neurologist. 

If you can’t go to the bathroom or are paralyzed and go to the ER, you may get an MRI that will show signs of inflammation in the spinal cord. Again, you’ll probably be referred to a neurologist for treatment and further testing (though you may be given a course of steroids at the hospital to get the inflammation down).

Some people don’t get lucky, though—NMOSD is a rare disease, many doctors are unaware of it, and some may not even think you need an MRI. That’s a problem, since the faster you get treatment, the greater your chances of recovering most of your mobility or vision and preventing another attack. 

Your best bet is to find an NMOSD-savvy neurologist by going to the Guthy-Jackson Foundation’s [Connect the Dots](https://guthyjacksonfoundation.org/tools-for-download/) tool.  After you’ve been diagnosed, that person can work with your primary provider, since NMOSD patients need a healthcare team to manage their disease. 


When to See a Doctor

## How Do Doctors Diagnose NMOSD?

The most reliable test to diagnose NMOSD if you have the symptoms is a simple blood test. There are NMOSD-specific antibodies in nearly 90% of people with NMOSD, so that’s the easiest way to confirm a diagnosis, even though you have to wait a couple of weeks to get the official results. 

In the meantime, your symptoms from an NMOSD attack will be treated aggressively, often with medications used to relieve MS symptoms, like steroids to bring down inflammation. (Remember, it’s *preventative* drugs for MS that don’t work well for NMOSD, but these two conditions do sometimes treat *symptoms* from a haywire immune response with the same approach.) If you don’t have antibodies, doctors look for other clues to make sure you don’t have MS or some other neurological disease. 

The diagnostic tests for NMOSD include:

### Physical Exam

Your doctor will measure your vital signs—heart rate, body temperature, and blood pressure—and check on how well the major systems of your body are functioning. You’ll also be asked questions about your medical history, as well as the health history of your family.

### Blood Tests

Your blood will be drawn to check for specific NMOSD-related antibodies—the test is called an NMO IgG antibody test. Around 75% will test positive for AQP4. About half who test negative for AQP4 go on to test positive for MOG, the protein on the myelin sheath. There’s ongoing debate whether having MOG antibodies means you have a different disease altogether (called MOG Antibody Disease). While you may have the same symptoms as NMOSD, if you have MOG antibodies you tend to heal better and make a fuller recovery. For now, treatment of MOG and AQP4 NMOSD is similar, and both disease courses are classified as variations of NMOSD. Then, there is a small group of patients that don’t test positive for either antibody, which then makes the condition tougher to diagnose.

### Magnetic Resonance Imaging (MRI)

This is an imaging test that uses powerful magnets and computer waves to make a detailed picture of your brain, optic nerve, and spinal cord. The lesions of NMOSD tend to be much longer and more inflamed than those in MS (but not always). The lesions can be found on the optic nerve, the spinal cord, and the brain stem, though sometimes there are lesions in other parts of the brain. 

### Spinal Tap

This is performed to examine cerebrospinal fluid that runs through the spinal column. In NMOSD, this may reveal AQP4 antibodies or high levels of inflammatory proteins called cytokines. If you have high levels of a protein called immunoglobulin instead, you probably have MS. 

### Optical Coherence Tomography (OCT)

This is a photograph of the back of the eye, including the retina. In many NMOSD patients, the retinal nerve layer has thinned out because of optic nerve damage.


Diagnosis

Common treatments for NMOSD, including Enspryng, Soliris, Uplizna, and plasma exchange.

INFO NMOSD revTREATMENTS

Common Treatments for NMOSD

## What Are the Best Treatments for NMOSD?

There are two stages of treatment. The first comes as soon as possible after an attack, and the goal is to put out the fire—to tame inflammation and stop further damage from occurring. Then, you’re put on a regimen of medications designed to prevent (or at least cut down) the number of relapses. Every person with NMOSD—monophasic and MOG-antibodies included—gets preventative meds, which you’ll take for the rest of your life. 

There are also treatments for specific symptoms. While you can go into remission, there is always some residual damage that can cause old symptoms to flare up. The triggers can be anything from an infection, like a urinary tract infection, or hot, humid weather, or stress. And for nearly every NMOSD patients, there’s nearly constant pain from nerves misfiringe—though there are ways to lessen that pain, so keep reading. 


Treatment

### Acute Treatment 

While you’re waiting for your antibody testing to come back, you’ll receive acute treatment—meaning urgent interventions—to relieve your symptoms. These include:

- __Corticosteroids:__ A high dose of these anti-inflammatory medications, via an I.V., will suppress your immune system so it’ll stop attacking itself. If your attack was relatively mild, then this will probably be the only treatment you need. What happens next depends on the response: If your symptoms don’t get better, or an MRI still shows inflammation, then doctors will move onto the next method. 

- __Plasmapheresis or plasma exchange:__ Think of this as a type of dialysis for your blood. Your blood goes through an I.V. and into a machine, which is designed to take out the proteins causing all that damage. Those include: 
  -  the antibodies
  -  the complement proteins, which normally clear up cell damage but  attack healthy cells during inflammation
  -  the cytokines, those inflammatory proteins 

  This process takes two weeks and is usually done for two hours every other day (sometimes all in the hospital, sometimes on an outpatient basis). Not everyone gets this treatment, but if you’ve had a bad attack with serious symptoms—you’ve lost most of your vision in one eye or can’t move your leg—doctors will do both treatments without waiting for results from the steroids.


### Preventative, NMOSD-Specific Drugs

Before 2019, there were no NMOSD-specific drugs. Instead, doctors treated the condition with off-label drugs that had been approved for other autoimmune disorders like lupus. But in 2019, the results of three NMOSD-specific drugs in clinical trials were published. Those studied included:

- Soliris (eculizumab)
- Uplizna (inebilizumab)
- Enspryng (satralizumab)

All three drugs are known as immunosuppressants—they suppress your immune system so you’re less likely to get another attack. And they all have the same basic side effect: Because your immune system is tapped down, you are more vulnerable to infection, which can be frightening during, say, a novel coronavirus pandemic. Some keep your immune system suppressed longer than others, so you may want to take that into consideration when discussing your options with a doctor.

In addition, the cost of these drugs can be sky-high (think in the hundreds of thousands of dollars per year, though insurance may cover most of these costs). Convenience, too, is a deciding factor, as Soliris is a bimonthly infusion, so you may prefer another approach, like taking an oral pill daily at home.

Now, onto the specifics:

- __Soliris (eculizumab__): This is a 35-minute infusion that you get every two weeks via an I.V. at the doctor’s office. It’s specifically designed for those patients who have AQP4 antibodies. It works by blocking two specific complement proteins, but it can also up your risk for meningitis, so you need to get a meningococcal vaccine before you start treatment. Still, Soliris has shown efficacy in preventing relapses. In clinical trials of 143 patients, it reduced the number of relapses by 94% over the 48-week trial. The negatives include some side effects, including respiratory infections, diarrhea, and headaches.

- __Uplizna (inebilizumab)__: This is a 90-minute I.V. infusion that you get every six months (after getting the first two infusions over two weeks) at your doctor’s office. Like Solaris, it’s also designed for patients with AQP4 antibodies and in trials, lowered the risk of another relapse by 77%. Side effects include UTIs and hepatitis B, so you must be screened for hepatitis B before you start the drug.

- __Enspryng (satralizumab)__: Like the other two drugs, this medication is only for people who have AQP4 antibodies. But unlike the other two, Enspryng is not an I.V. infusion. Instead, it’s an injection you can give yourself every four weeks— your doctor will show you how—after the initial dose, which is three shots in the first month. In one of the clinical trials, about two-thirds of the 63 people taking just Enspryng were relapse-free after two years. In another, 41 people took Enspryng along with another treatment (like Immuran or CellCept). Of these folks, only 11% had relapses. Side effects include a sore throat, runny nose, fatigue, and joint pain, but affected roughly 15% of those in the trial. You must be screened for both hepatitis B and tuberculosis before you start the drug.   

If you do have a relapse, you’ll be given the steroids and the plasma exchange again, and your doctor may discuss switching you to another preventative medication.







### Off-Label Drugs

There are three immunosuppressant medications that have had the best results for NMOSD, even though the evidence for them is based on studies that look at small number of patients. Please note that none of these drugs is 100% effective—at best, each lowers the odds you’ll have another attack.

- __Rituxan (rituximab):__ This, too, is an I.V. infusion. You get two courses in the first two weeks, and then one every six months. In one study, researchers took a 10-year retrospective look at 90 NMOSD patients and found that those taking rituximab cut their relapse rate by 88%, with two out every three patients achieving full remission.

- __CellCept (mycophenolate) and Immuran (azathioprine):__ These are tablets you take every day. Some doctors will also suggest you take the steroid prednisone for some time along with these meds, although this powerful steroid can cause serious side effects, so it’s not a long-term solution. Of the two, CellCept seems to cut the relapse rate more effectively than Immuran, according to one retrospective study.


## What Happens During NMOSD Remission? 

After an attack—and once treatment has brought down inflammation—your body begins to repair the damaged tissue. Some patients heal better than others, and make full recoveries, and others don’t (though doctors think having the MOG antibodies is a sign that you may do better long-term), and they don’t exactly know how this process works.  

What they do know is that the healing process often feels worse than the attack itself, because neurons, once blocked by damage, start communicating with the brain again. So if you first felt numbness in your leg, as nerves heal you might feel tingling all the time, or have nearly constant (and painful) muscle spasms. 

Or, if you couldn’t empty your bladder at all, you now have a hyperactive one that forces you to up two or three times a night to go to the bathroom. Believe it or not, these are good signs—the reason why they’re called positive symptoms. 

Healing usually takes six months or more, and it’s never linear—you’ll have good days and bad ones. Something as benign as a bitter cold or hot, humid day can trigger your old symptoms, and you might have blurrier vision for a short period of time or need a walker to get around for a few hours or days.

Physical therapy definitely helps you regain your mobility, though it may never go back to what it once was. Depending on the severity of your first attack, your vision problems from optic neuritis may improve, but you may have long-term effects like halos, trouble seeing in the dark, or even some permanent vision loss. 

Chances are, you’ll slowly get better—you may start out in a wheelchair but after a few months just need a cane for longer walks. But if you wake up not being able to see out of one eye again or find you cannot move a leg (if your previous attack only involved your eyes), then call your doctor immediately. Your physician will determine if these are signs of a new attack.

Because NMOSD is a lifelong condition that needs to be managed well, you’ll probably have a treatment team that includes:

- An __NMOSD specialist__, usually a neurologist that has experience treating related diseases (like MS or TM), who you may see a couple of times a year and who can keep your primary care provider in the loop about new treatments. This may be someone at an MS center or hospital affiliated with a medical school or someone from one of the two NMO centers (one at Johns Hopkins and one at the University of Texas) who will collaborate with your primary care doctor or neurologist. 

- A __primary care doctor__ or __local neurologist__, who can manage symptoms and medications

- A __physical therapist__, who will work with you to recover your strength

- An __occupational therapist__, to help with daily living

- A __social worker__ or __therapist__, who can give you coping strategies to help with mood

Remission

## Does NMOSD Have Complications?

Most of the time, yes—including emotional ones. Even if you make a nearly full recovery, you don’t really know when you might have another attack. And that uncertainty can take its toll.
Many NMOSD patients report one or more of the following complications:

### Neuropathic Pain

At least two-thirds of NMOSD patients—and possibly as many as 91% of them—have constant, often debilitating pain, making it the number-one complaint. This can include:

* Constant, disruptive tingling
* Feeling like you’re on fire when something touches you
* Stabbing sensations

Again, it seems all wrong but such symptoms are a good sign—it means the nerves in your CNS are communicating again, even if they have an increased response, perhaps to make up for the nerve cells that were destroyed. 

Doctors can treat this pain with meds, including anti-epileptics like gabapentin, antidepressants, and opioids. But because the NMOSD lesions in the spinal cord are broad and long, they disrupt more connections between the pain pathways going to and from the brain, leading to more severe pain. One result: Medications don’t work as well for people with NMOSD as well as they do for, say, MS patients. Plus, they can lead to medication overload, which can add other side effects.

Pain is complex, and involves the actual physical discomforts as well as the way it affects your thoughts and emotions. Reducing pain has to involve both medications and non-pharmaceutical treatments, including:

* __Mindfulness meditation__: Focusing on your breath may not always lessen the intensity of the discomfort (studies on this for other chronic pain patients are mixed), but it can improve your quality of life by decreasing stress and boosting a sense of calm and acceptance.  

* __Cognitive-behaviorial therapy (CBT)__: This short-term therapy aims to give you a way to reframe thoughts as well as strategies to help you cope with your pain. And while it may not lessen the severity (again, studies are mixed), like mindfulness, it can make pain more bearable.

*	__Yoga and tai chi__: These mind-body exercises can improve mindfulness and build up your muscles, preventing the wear and tear on your joints that can lead to more pain. 

*	__Electrical stimulation__:  This can include older devices like TENS, which uses electrodes placed on your skin to deliver low-voltage currents that block nerves from transmitting pain signals to the brain. Another device is the newer Scrambler therapy, a specially designed machine that reorganizes the inappropriate signaling of the nerves by directly stimulating sensory pain receptors called C delta fibers via pads put on the areas where you feel the most pain. Scrambler therapy has to be done by a specially trained therapist, but preliminary evidence for NMOSD looks promising—in one small study, patients were able to cut their pain in half after 10 days of therapy.













### Muscle Spasms or Stiffness

Muscle spasms or tightening are another source of pain. This can range from feeling very stiff in the morning to really bad Charlie horses that happen 10 times an hour. Or, muscles that are so tight you can’t move your arm because it’s flexed or frozen in place. Physical therapy is key with these symptoms, as well as daily stretching to help you regain flexibility.

### Depression

If your life is upended and you suddenly can’t walk or see well, then it makes sense that you feel down much of the time—and one study found that 40% of NMOSD patients were either moderately or severely depressed. But scientists also suspect the inflammation in your brain from NMOSD may also affect your mood (as it does in MS), though more studies need to be done on this aspect. What is true is that depression can make your pain and exhaustion worse, so it needs to be treated. Medications can help, but so can things like exercise (even chair yoga) and CBT.

### Sexual Dysfunction

Spinal cord lesions can affect your genitals too, and that can lead to problems that interfere with pleasure. So if you’re a guy, you may not feel any sensations in your penis, or you could have problems ejaculating, or even becoming erect. If you’re a woman, extra-sensitive nerve endings and/or a lack of lubrication can make sex painful—or, you may be too numb to feel any kind of stimulation (vaginal or clitoral). 

Plus, other symptoms like pain or fatigue can put the kibosh on your libido, as can medications. This lack of intimacy is a huge issue—and it may be affecting nearly all NMO patients, though no one really knows—but you may be too embarrassed to bring it up with your doctor. Don’t be—speak up so you can get help. Vaginal lubricants or estrogen-based creams can help with dryness and pain, as can meds for erectile dysfunction.

### Bladder and Bowel Issues

If NMOSD affected your ability to go to the bathroom during an initial attack, your bladder can come back during the healing process with a vengeance. That means more trips to the bathroom, and (if you’re a woman) more UTIs and trouble emptying out the bladder. Sometimes Botox shots can help with a hyperactive bladder, as can other medications. So this is another issue you must bring up with your provider, no matter how embarrassing. 

### Fatigue

It’s unclear whether exhaustion is a primary symptom of NMOSD or a secondary one, a result of pain, too many medications, and just the physical energy it takes to get around when you have to learn new ways to walk. Finding strategies to deal with pain can help you feel less tired, as can gentle forms of exercise, like yoga, tai chi, and stretches. 

Complications

## What Is Life Like for People With NMOSD?

When you’re left with residual weakness, fatigue, pain, sexual and bladder dysfunction, and problems seeing it can change the way you interact in the world—physically, emotionally, and socially. Going back to a job can be harder or nearly impossible. It can really become a vicious cycle—if you have to get up to go to the bathroom several times at night, then you’re not sleeping well. A lack of zzz’s can make your pain worse, which in turn, can affect your mobility and the desire to do any kind of physical activity. It soon turns into a cycle that can wreak havoc with your quality of life. 

There are things that can stop the cycle, and the first step is to be proactive in your care. If you feel your doctors are throwing up their hands on your recovery, try to find new ones. There are also other things you can do to speed the healing process and set yourself up to thrive in your new life, no matter the challenges.

### Physical and Occupational Therapy

Even if you must use a walker or cane, a physical therapist can show you how to align your body to avoid further wear and tear on your joints, which can cause more problems down the line. Or they can teach you exercises that help you regain your balance and strength, so you walk better and more safely. An occupational therapist can give you tips on how to rearrange your home to make it easier for you to get around if you can’t see as well, or if you need help navigating with a cane or other device.

### Exercise

Even if you only get one session ever with a physical therapist who will point you in the right direction, there’s much you can do on your own. What helps is to keep moving in some way, whether you swim, lift light weights, or stretch every morning. (Or all three on different days of the week!) Regular physical activity, especially the kind that gets your heart rate up a bit, can reduce chronic inflammation, help with depression, and even alleviate pain. 

### Healthy Diet

While there’s no concrete evidence that eating more fruits and vegetables can prevent relapses, there is anecdotal evidence that NMOSD patients feel much worse when they eat foods that promote inflammation, like red meat, sugary treats, and simple carbs. And vice versa—patients feel better when they eat fish high in omega-3s, more produce and legumes, and heart-healthy fats. So experiment—add meatless dishes to your rotation, add veggies and salads to every meal, and cook with olive oil. It may make a difference.

### Vitamin D

Most NMOSD practitioners will recommend you take a vitamin D supplement to help lower inflammation. If yours doesn’t, ask for a blood test to check your levels and then have your provider recommend one based on what the labs show.  

### Support

Having a rare disease that no one knows much about can be really isolating. Besides friends and families, finding others who are living with this disease can be a godsend, whether they’re on Facebook or live nearby. Luckily, there are organizations that can offer information as well as connect you to other NMOSD patients as well as specialists. 

Living With

## Where Can I Find My NMOSD Community? 

You can feel really cut off from your old self when you first learn you have NMOSD—you have to learn how adapt to your new normal and restructure your life while explaining it to the many folks who’ve never heard of the condition. So engaging with others who are going through what you are is critical—whether you’re getting help or lending support to someone who’s a few steps behind you on this journey.  For inspiration, we’ve put together this list to help you feel less alone. 

### Top NMOSD Instagrammers: 

[__Taylor Ann Macey, @taylorannmacey__ ](https://www.instagram.com/taylorannmacey/) 

- __Follow because:__ Before she was diagnosed in 2019, she was starting up her own business as a life coach. And while NMOSD has thrown a bit of a wrench in her plans, she didn’t let it stop her—she let it fuel her. Now, she uses her influence and knowledge as a certified life coach to bring you the Brain Diet Podcast, which in turn, helps her NMOSD. 

[__Chelsey Tucker, @chelsey_tucker___](https://www.instagram.com/chelsey_tucker_/)

- __Follow because:__ Her story doesn’t just include her being able to walk after NMOSD knocked her down, but it also follows her weight-loss journey—one she took so her body is better able to fight her autoimmune disease. Like so many, she was misdiagnosed with MS for years (a decade!), and now she’s working hard to find her new normal in NMOSD. 

[__Avery Allmond, @chronically.ave__](https://www.instagram.com/chronically.ave/) 

- __Follow because:__ While she doesn’t sugarcoat the hardships and WTF’s of NMOSD, she also has such an appreciation for the love and support around her that it helps you find the silver linings. More silver linings—or copper, really—is her foundation, Coach Pennies, that’s dedicated to helping those with rare diseases.  

[__Aldelly, @fab_nmo__](https://www.instagram.com/fab_nmo/) 

- __Follow because:__ Mother, wife, entrepreneur, and, oh yeah, mega-NMOSD advocate. She shares a constant behind-the-scenes look at life with NMOSD, including infusions and parenting with a chronic illness. And, she’s an open book with NMOSD Q&As. Bonus: She posts content in English and Spanish, and has tons of videos. 

### Top NMOSD Organizations: 

- [__Guthy-Jackson Charitable Foundation:__](https://guthyjacksonfoundation.org/) The parents of a teenager started this foundation dedicated to researching the disease. You’ll not only get good information on the site via videos, but you can find a list of NMOSD specialists in every state and country with its [__Connect the Dots__](https://guthyjacksonfoundation.org/tools-for-download/) tool as well as support groups, both on Facebook and in various cities across the country.   

- __[Siegel Rare Neuroimmune Association](https://wearesrna.org/) (SRNA):__ This nonprofit is focused on a variety of rare neuroimmune disorders, including NMOSD, plus has information on children and teenagers, as well as adults. SRNA hosts a yearly summer camp for kids with NMOSD and other neuroimmune diseases. (In 2020, due to COVID-19 concerns, it’ll be held online.) You can also find information on clinical trials as well as support groups and a wealth of information, including a newsletter.

- [__The Sumaira Foundation for NMO:__](https://www.sumairafoundation.org/) Sumaira Ahmed, an actress and model, started this nonprofit after she was diagnosed with NMO. The organization has sponsored a “Demystifying NMO” podcast that delves into topics like sex and pregnancy when you have NMOSD, as well as a section highlighting patients’ stories. You can also become a TSF ambassador (or find one in your state or region).


It can be, because the symptoms—blurry vision, impaired mobility—can be similar and even MRI scans can be misleading. Also, the two conditions affect mostly women. But there are differences. NMOSD attacks take place during a shorter period of time, are worse, and leave people more disabled. People tend to be older—on average, in their late 30s and 40s, instead of in their 20s. And 80% of people with NMOSD have specific antibodies not found in MS.

Is NMOSD often mistaken for MS?

It’s another inflammatory attack of the central nervous system (CNS) that can leave you more disabled, especially if it occurs in the same place (like your spinal cord). About half of all NMOSD patients have relapses in the first year, so preventing these attacks is key. That’s why all NMOSD patients are put on immunosuppressant medications.

What is an NMOSD relapse?

The signs of another attack aren’t subtle, and the symptoms usually get worse over the course of a few days. If your first attack left you with recurring blurry vision, the second one might make you go blind in one eye or it may affect another part of your nervous system, like your legs or arms. But these relapses vary from person to person, and there’s some data that suggests preventative meds are associated with milder relapses.

How will I know if I have a relapse?

Primary care doctors can treat NMOSD, but it also helps to have a neurologist who specializes in neuroimmune disorders—like multiple sclerosis (MS) or transverse myelitis (TM)—on your team, too. Even if you only see the neurologist once or twice a year, this specialist can work with your primary care provider to deal with changes in your health and keep your doctor informed about the latest research in the disease.

Will I need to be treated by a neurologist?

Neuromyelitis Optica Spectrum Disorder (NMOSD) FAQ

Neuromyelitis Optica Spectrum Disorder (NMOSD)

NMOSD is an autoimmune condition that affects your central nervous system (CNS), which is comprised of the spine, optic nerves, and brain. Get details on the symptoms, causes, treatments, and more here.

More Info on NMOSD

Benjamin M. Greenberg, M.D.

Director and Professor of Neurology

Michael Levy, M.D.

Director and Associate Professor of Neurology

Maureen Mealy, Ph.D., R.N.

Adjunct Associate Professor of Neurology

We went to some of the nation’s top experts in NMOSD to bring you the most up-to-date information possible.

Neuromyelitis Optica Spectrum Disorder

iStock-470858115

central nervous system-iStock-470858115

Often mistaken for multiple sclerosis, NMOSD can cause serious vision and mobility problems. Help is here.

Let’s Talk About Neuromyelitis Optica Spectrum Disorder (NMOSD)

- __NMOSD Statistics:__ UpToDate. (2019). “Neuromyelitis Optica Spectrum Disorders.” [uptodate.com/contents/neuromyelitis-optica-spectrum-disorders](https://www.uptodate.com/contents/neuromyelitis-optica-spectrum-disorders)

- __NMOSD and Antibodies:__ *Journal of Neurology, Neurosurgery & Psychiatry*. (2020). “Prevalence and incidence of neuromyelitis optica spectrum disorder, aquaporin-4 antibody-positive NMOSD and MOG antibody-positive disease in Oxfordshire, UK.” [jnnp.bmj.com/content/early/2020/06/23/jnnp-2020-323158](https://jnnp.bmj.com/content/early/2020/06/23/jnnp-2020-323158)

- __Children and NMOSD:__ *Children* (*Basel*). (2019). “Differential Diagnosis of Pediatric Multiple Sclerosis.” [ncbi.nlm.nih.gov/pmc/articles/PMC6617098/#B1-children-06-00075](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6617098/#B1-children-06-00075)

- __Race and NMOSD:__ *Archives of Neurology*. (2012). “Epidemiology of Neuromyelitis Optica in the United States: A Multicenter Analysis.” [pubmed.ncbi.nlm.nih.gov/22733096/](https://pubmed.ncbi.nlm.nih.gov/22733096/)

- __Relapse Rates:__ Guthy-Jackson Charitable Foundation (n.d.) “NMO: What You Need to Know.” [guthyjacksonfoundation.org/tools-for-download/](https://guthyjacksonfoundation.org/tools-for-download/)

- __Gut Bacteria and NMOSD:__ *Annals of Neurology*. (2016). “Gut Microbiome Analysis in Neuromyelitis Optica Reveals Overabundance of Clostridium Perfringens.” [pubmed.ncbi.nlm.nih.gov/27398819/](https://pubmed.ncbi.nlm.nih.gov/27398819/)

- __Diet and Exercise in NMOSD:__ *Acta Neurologica Belgica* (2018). “Environmental Risk Factors in Neuromyelitis Optica: A Case-Control Study.” [pubmed.ncbi.nlm.nih.gov/29500680/](https://pubmed.ncbi.nlm.nih.gov/29500680/)

- __Vitamin D and NMOSD:__ *Autoimmune Diseases*. (2020). “Vitamin D and Demyelinating Diseases: Neuromyelitis Optica (NMO) and Multiple Sclerosis (MS).” [ncbi.nlm.nih.gov/pmc/articles/PMC7187724/](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7187724/)

- __Rituximab and NMOSD:__ *JAMA Neurology*. (2014). “Comparison of Relapse and Treatment Failure Rates Among Patients With Neuromyelitis Optica: Multicenter Study of Treatment Efficacy.” [pubmed.ncbi.nlm.nih.gov/24445513/](https://pubmed.ncbi.nlm.nih.gov/24445513/)

- __Eculizumab and NMOSD:__ U.S. National Library of Medicine. (2019). “A Randomized Controlled Trial of Eculizumab in AQP4 Antibody-positive Participants With NMO (PREVENT Study).” [clinicaltrials.gov/ct2/show/study/NCT01892345](https://clinicaltrials.gov/ct2/show/study/NCT01892345)

- __Uplizna and NMOSD:__ USFDA. (n.d.). “Drug Trials Snapshots: UPLIZNA.” [fda.gov/drugs/drug-approvals-and-databases/drug-trials-snapshots-uplizna](https://www.fda.gov/drugs/drug-approvals-and-databases/drug-trials-snapshots-uplizna)

- __Scrambler Therapy in NMOSD Patients:__ *Neurology*. (2020). “Scrambler Therapy Improves Pain in Neuromyelitis Optica: A Randomized Controlled Trial.” [pubmed.ncbi.nlm.nih.gov/32269109/](https://pubmed.ncbi.nlm.nih.gov/32269109/)

- __Depression in NMOSD Patients:__ *Neurology, Neuroimmunology and Neuroinflammation*. (2016). “Insufficient treatment of severe depression in neuromyelitis optica spectrum disorder.” [ncbi.nlm.nih.gov/pmc/articles/PMC5079380/](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5079380/)

__Enspryng and NMOSD Relapses (1.)__: *The Lancet (Neurology).* (2020.) “Safety and efficacy of satralizumab monotherapy in neuromyelitis optica spectrum disorder: a randomised, double-blind, multicentre, placebo.” pubmed.ncbi.nlm.nih.gov/32333898/

__Enspryng and NMOSD Relapses (2.)__*The New England Journal of Medicine.* (2019.) “Trial of Satralizumab in Neuromyelitis Optica Spectrum Disorder.” nejm.org/doi/full/10.1056/NEJMoa1901747



Neuromyelitis Optica Spectrum Disorder: Symptoms, Treatments and More

Occasionally mistaken for multiple sclerosis, NMOSD can cause serious vision and mobility problems. Help is here.

Rare, debilitating, but highly treatable, neuromyelitis optica syndrome disorders (mercifully better known by the acronym NMOSD) are a group of autoimmune conditions that mess with your central nervous system, the pathway by which your brain and the rest of your body send messages to each other. When your brain says, “Touch this!” and your hand sends back the information, “Ow, that’s hot!”, this is your central nervous system at work. But the relaying of such messages starts to falter in people with NMOSD.

With NMOSD, certain cells in your body attack different parts of the central nervous system, most commonly the optic nerve (the cable that connects the eye to the brain), the spinal cord (the long bundle of nerves that run along your backbone), and part of the brainstem (the base of your brain that regulates involuntary actions such as breathing and swallowing). Under attack, these areas swell and become inflamed, which can cause pain, loss of vision, weakness, numbness, paralysis, problems going to the bathroom, and range of other issues. 

Of course, not every symptom crops up all at once during an NMOSD attack, and most symptoms only occur for a confined length of time. But still, an NMOSD attack can do lasting damage. “It comes thick, quick, and fast when it happens, and it can be devastating,” says Saif Huda, M.D., Ph.D., a neurologist at the Walton Centre Foundation Trust in the U.K.

Like multiple sclerosis, a disease NMOSD is often mistaken for, the condition is characterized by periods of normalcy punctuated with recurring relapses, when attacks on the central nervous system occur. These can be years apart. Fortunately, doctors now have effective treatments to help prevent or minimize such events. When an attack does occur, here’s a detailed look at what it can do to your body.

## Blurry Vision

Even though NMOSD can affect your sight, it doesn’t damage the eye itself, says Ilya Kister, M.D., a neurologist and director of the Neuromyelitis Optica Treatment and Research Program at New York University Langone Medical Center. Instead, the disease causes the optic nerve to become inflamed, preventing it from properly transmitting signals from your eye to your brain, a condition called “optic neuritis.”

Inflammation of the optic nerve can distort vision in a few ways. Very often, people feel pain, particularly when moving their eye, accompanied by blurriness or loss of vision in one or (rarely) both eyes. An NMOSD attack could also cause part of your field of vision to disappear when you close one eye. Or color vision might fade, so everything appears tinted with grey.

These vision issues typically last for days or weeks and worsen over time. “It starts at a low burn and then it heats up,” describes Dr. Kister. After an attack, your vision usually improves, though often it doesn’t completely return to normal. 

## Weak Legs, Numb Arms

Your spinal cord is a long tube, as thick as a thumb, responsible for carrying messages between the brain and the body. During an NMOSD attack, a chunk of this pipeline becomes inflamed, an issue known as “transverse myelitis.” Often, this inflammation extends over three or more vertebrae and can lead to a wide range of issues, depending on where the spinal cord is impacted. 

For instance, an attack might cause sudden weakness or loss of sensation in a leg or an arm, causing you to have trouble walking or difficulty using your hands. Often, numbness or weakness will start from your lower limbs and creep upwards. NMOSD can also cause paralysis of the legs and lower body, which may lead to loss of control going to the bathroom. 

Other unexpected issues can crop up too, like intense, painful itching caused by inflammation of certain fibers in the spinal cord. Other times, inflammation leads to recurring spasms, where the muscles of an arm or leg are stuck in one position for 30 seconds or more. 

In most cases, symptoms start off as mild and build up over days as inflammation in the spinal cord progresses. Eventually, the inflammation will lessen, but without treatment, it can result in lasting damage, even leaving patients wheelchair-bound after a particularly severe attack. 

## Uncontrollable Hiccupping

Your brainstem sits at the base of your skull, connecting your brain to the spinal cord. Sometimes during an NMOSD attack, inflammation travels to this area, affecting part of the brainstem called the area postrema. This region controls involuntary bodily actions such as hiccupping and vomiting. When it comes under siege during an NMOSD attack, it can cause incessant bouts of either (or both) behaviors. Fortunately, hiccups and puking are primary symptoms in only about one in 10 cases of NMOSD. But when they do occur, the disease can be very hard to diagnose. “It will look to all the world like someone has a major GI illness,” Dr. Kister says. 

## Why Your Body Attacks the Nervous System
Most people diagnosed with NMOSD have antibodies in their blood that attack a protein called aquaporin 4, which lives on certain cells of the central nervous system and helps move water across cell membranes. These antibodies wreak havoc on the nervous system, igniting a chain of events that results in inflammation.

Other people have a different type of antibody that attacks a protein called myelin oligodendrocyte glycoprotein (MOG) that helps protect the nerves. (A relatively new distinction, it's possible people with the MOG antibody may be classified as having a different, unique disease in the future.)

On the “bright” side, the presence of these antibodies means it’s easy to test for NMOSD. It’s a very rare condition, though: Only a few thousand people in the U.S. have the disease, and about a quarter million worldwide have been diagnosed. If you are one of the unlucky few, the good news is that there are a number of effective treatments which have come out in the last few years that help keep this condition under control. It’s possible to live a healthy life despite an NMOSD diagnosis with the right treatment plan in place. 

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NMOSD causes inflammation in the nervous system that comes in periodic attacks and can damage the optic nerve, spinal cord, and brainstem. 

NMOSD and Your Body

The trouble may start with your nervous system, but this condition can cause serious problems with your vision and movement. 

This Is Your Body on NMOSD

- __An Overview of NMOSD:__ *Clinical Medicine*. (2019). “Neuromyelitis Optica Spectrum Disorders.” [ncbi.nlm.nih.gov/pmc/articles/PMC6454358/](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6454358/)

- __Clinical Presentations:__ *Rheum Dis Clin North Am*. (2017). “Neuromyelitis Optica.” [pubmed.ncbi.nlm.nih.gov/29061244/](https://pubmed.ncbi.nlm.nih.gov/29061244/)

- __How Common is NMOSD:__ National MS Society. (2019). “Neuromyelitis Optica (NMO).” [nationalmssociety.org/What-is-MS/Related-Conditions/Neuromyelitis-Optica-(NMO)#:~:text=Neuromyelitis%20optica%20(NMO)%20and%20NMO,may%20also%20attack%20the%20brain](https://www.nationalmssociety.org/What-is-MS/Related-Conditions/Neuromyelitis-Optica-(NMO)#:~:text=Neuromyelitis%20optica%20(NMO)%20and%20NMO,may%20also%20attack%20the%20brain)


How NMOSD Affects Your Optic Nerve, Spinal Cord, and Brainstem

The trouble may start with your nervous system, but this condition can cause serious problems with your vision and movement.

If you’ve heard of neuromyelitis optica spectrum disorder (NMOSD), it’s probably because you know and love someone who has this super rare condition. Maybe you even have it yourself. It’s often confused with multiple sclerosis (MS), but it’s definitely its own thing. Though both conditions involve a faulty immune response that attacks the central nervous system (CNS), causing similar symptoms—vision, walking, plus bladder and bowel problems—experts say the two disorders are entirely distinct. 

Those distinctions all involve the immune system: *how* it attacks, the severity of those attacks, and the clues an NMOSD attack leave behind.

## A Normal Immune Response

First thing to know? NMOSD, like MS, is an autoimmune condition, which means the immune system goes rogue and attacks healthy tissue by mistake. 
 
“Our immune system should know our own brain, but when it doesn’t or if it gets confused, it can cause autoimmunity, where it goes after our own parts,” explains Mary Rensel, M.D., a neurologist at Cleveland Clinic’s Mellen Center for Multiple Sclerosis Treatment and Research in Cleveland. She’s treated people with NMOSD for more than two decades. “It’s not that the immune system isn’t working at all, but rather that it’s doing too much—and it’s doing it wrong.”

Before diving into what exactly it’s doing wrong, it may help to understand how your immune system is *supposed* to work. The immune system’s main job is to protect your body from invaders, like viruses, bacteria, and toxins. When these invaders harm the body’s issues, your immune response kicks into gear and causes inflammation at the site of the damage. Inflammation causes swelling, which actually helps your body keep any invader away from other healthy tissue. White blood cells also rise to the occasion and do their best to help fight off the invaders. 

In people with NMOSD, though, the immune response is unfortunately not that simple.

## Your Immune System on NMOSD
 “When we test [NMOSD patients’] blood, we find in 75% of patients, their immune system is producing antibodies directed against aquaporin-4,” says Elias Sotirchos, M.D., director of the NMOSD clinic at Johns Hopkins University in Baltimore. “These antibodies are considered to be the cause of the disease.” What are antibodies, again? They're proteins in your body that are supposed to fight off bacteria, viruses, and toxins.

Aquaporin-4, or AQP4 for short, is none of those things. Instead, it's a protein we all have that helps transport water in and out of certain cells, especially those in the CNS. It’s found on what are known as __astrocytes__ in the CNS, according to the Siegel Rare Neuroimmune Association (SRNA). Astrocytes, Dr. Rensel says, are kind of like a safety net for the body’s nerves—they help protect the blood-brain barrier, among other important tasks—so if they get hurt, nerves get hurt, too. 

These AQP4 antibodies, also called __NMO-IgG__ or __anti-AQP4__, circulate in the blood, he says, and then gain access to the CNS, where they bind onto AQP4 water channels. “We think the antibody binding to its target triggers a cascade of inflammation, leading to damage to the nervous tissue in the area.”

Much like a domino effect, “when these antibodies bind there, they can do other things, such as recruit inflammatory cells,” Dr. Sotirchos explains. Another thing they can do is activate the complement system, which is part of our immune system that, among other things, “can essentially poke holes into cells and cause damage.” Normally, that ability comes in handy when you’re trying to destroy actual bacteria and viruses. Not so much, though, when it’s your own healthy tissue.

NMOSD most commonly targets AQP4 in optic nerves and the spinal cord, followed by a part of the brainstem called the area postrema, says Dr. Sotirchos. Depending on which of these areas is under attack, different symptoms will arise.

- __The optic nerves__. These nerves carry information from the part of your eye called the retina to the brain. So for those with damage and inflammation in the optic nerves (called optic neuritis), vision loss can occur, along with pain in the eye, says Dr. Rensel. One or both eyes may be affected.
- __The spinal cord__. “The spinal cord is an extremely important part of the nervous system because nerves relay information from the brain to the body and vice versa, including the nerves that tell our limbs to move and that control urinary and bowel function, for example,” Dr. Sotirchos explains. When NMOSD affects this area, it leads to what’s called transverse myelitis, or inflammation of the spinal cord. This can lead to symptoms like pain, mild to severe paralysis of the lower limbs, and loss of control over bowel and bladder functions, according to the National Organization for Rare Disorders (NORD).
- __The brainstem__. The area postrema, which is part of the brainstem, only occurs in about 10% of people with NMOSD, Dr. Sotirchos says. “Involvement of this area can lead to a specific syndrome where patients have nausea, vomiting, and hiccups, because this area controls these reflexes in the body.”

Compared with MS attacks, Dr. Rensel says, NMOSD attacks come on “fast and furious” and can be much more severe. “NMOSD relapses are more disabling, and the damage to the nerves is more irreversible,” she says. That means that a single attack can cause permanent vision loss or even blindness, or, if the spinal cord is attacked, paralysis. 

## What Causes NMOSD Antibodies to Form in the First Place?

Unfortunately, the frustrating answer is that researchers still don’t know. That said, there does appear to be some level of genetic association, and there are certain groups that appear to get NMOSD more than others, Dr. Sotirchos says. “About 90% of patients are women. And there seems to be higher prevalence among people of African descent.”

A significant number of people with NMOSD have other autoimmune conditions, too.
“A number of them will have features consistent with lupus or other autoimmune conditions that are associated with abnormal antibody production, which tells us that there is something abnormal about their B-cells, which are the kind of cells that evolve to produce antibodies in the blood,” Dr. Sotirchos explains. “So it does seem that these patients with NMOSD are prone potentially to develop other autoimmune conditions that involve autoantibodies, suggesting that there is something with their B-cell development that may be dysfunctional and causing the disease.”

## How Treatments Target the Immune Response

Scientists may not understand what triggers the immune system in NMOSD, but identifying the target of this haywire response means there are now super-effective treatments available.

There are currently two NMOSD-specific medications approved by the U.S. Federal Drug Administration (FDA), with a third widely believed to be approved in the U.S. soon. And, Dr. Rensel adds, there are a few off-label drugs that doctors already use with some success to help prevent a future NMOSD attack. That’s because the main goal of NMOSD treatments is to help prevent another relapse (which occurs in the vast majority of people with NMOSD, usually within one to three years of the initial attack). “These drugs help lessen relapses and … irreversible damage,” she explains.
 
Interestingly, many of these treatments work differently on the immune system. “For example, some are trying to block the immune response, some are trying to block the cell that causes the response,” Dr. Rensel says. “All the medications that are being tried work a little differently, which is actually good for the patients because if one doesn’t work, you could actually try a whole different family of medications.”

Treatment has come a *long* way in the more than 20 years since Dr. Rensel started treating people with NMOSD. “It’s a really exciting day for this disease that used to be a really hard diagnosis with no treatment,” she says. “It’s wonderful that there are treatments now, and trials going on, and treatments work very well. It’s a new day for this disorder, which is wonderful to see.”

When the immune system goes rogue and attacks a normal water protein in the central nervous system, neuromyelitis optica spectrum disorder is the result.

NMOSD Immune Response

We explain the rogue immune process that brings on the rare autoimmune disease neuromyelitis optica spectrum disorder.

What the Immune System Gets So Very Wrong in NMOSD

- __How Your Immune System Works:__ (2020). National Institutes of Health. “Immune Response.” [medlineplus.gov/ency/article/000821.htm](https://medlineplus.gov/ency/article/000821.htm)
- __NMOSD Information From SRNA:__ Siegel Rare Neuroimmune Association. (2020). “Neuromyelitis Optica Spectrum Disorder.” [wearesrna.org/living-with-myelitis/disease-information/neuromyelitis-optica-spectrum-disorder/](https://wearesrna.org/living-with-myelitis/disease-information/neuromyelitis-optica-spectrum-disorder/)
- __NMOSD Information From NORD:__ The National Organization for Rare Disorders. (2018). “Neuromyelitis Optica Spectrum Disorder.” [rarediseases.org/rare-diseases/neuromyelitis-optica/](https://rarediseases.org/rare-diseases/neuromyelitis-optica/)

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NMOSD: Mistaken for MS

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