NMO and MOGAD Relapses are Potentially Life-
Threatening and Can Lead to Permanent Disability.
Reducing Time to Rescue Therapy is a Main Factor in Relapse Outcome.
If You Have Signs or Symptoms of a Possible Relapse Please Contact Your NMO or MOGAD Specialist and Healthcare Team Urgently as Soon as Possible.
What are Relapse Signs & Symptoms?
In medical terms, a sign is a manifestation of disease or an exam finding that is objective, not subject to interpretation. Example signs in NMO or MOGAD relapse include loss in measurable visual acuity, uncontrollable hiccups or vomiting, inflamed spinal cord or optic nerve lesion on MRI, paralysis of a limb or abnormal white blood cell count or distribution.
By comparison, a symptom is a disease manifestation or exam finding that is subjective or based on perception of the issue by a patient. Example symptoms in NMO or MOGAD include perceived change in color vision or contrast, bowel or bladder incontinence, type or severity of pain, as well as relative changes in strength or weakness of a limb or muscle.
Signs and symptoms can be directly attributable or indirectly related to a relapse. For example, loss of visual acuity is often a direct result of inflammation of an optic nerve or retina that directly transmits visual images to the brain. Indirect signs or symptoms of a relapse may include fatigue or feelings of anxiety or depression. These signs or symptoms may be real but usually not caused by the immunology of relapse itself.
Different types of NMO or MOGAD relapse may be defined by corresponding signs and symptoms. A few examples include:
|
Relapse Type |
Eye Pain or Loss in Vision |
Weakness in Arms or Legs |
Bowel or Bladder Dysfunction |
Prolonged Vomiting or Hiccups |
Other Cerebral Issues |
|---|---|---|---|---|---|
|
Optic Neuritis |
✅ |
|
|
|
|
|
Transverse Myelitis |
|
✅ |
✅ |
|
|
|
Area Postrema |
|
|
|
✅ |
✅ |
|
Brain Stem |
|
|
|
✅ |
✅ |
|
Brain Proper |
|
|
|
|
✅ |
Am I Having a Relapse or Not ?
Without a clinical exam by an NMO or MOGAD specialist, there is no certain way for you to tell if new, changing or worsening signs or symptoms are those of an actual relapse or other indicators that mimic a relapse (pseudorelapse).
Here are themes that can be helpful for you and your doctor to consider together quickly by telephone or a telehealth visit:
▢ Have you experienced recent signs of infection, such as fever, chills, cough or painful urination ?
▢ Are you having prolonged episodes of nausea, vomiting, pain or extreme fatigue ?
▢ Have you been under new or intense periods of stress in recent weeks ?
▢ Have you experienced a change in sleep or nutrition patterns in your daily routine ?
▢ Are your signs or symptoms in the same location in your body as your onset episode, or lasted > 24 hours ?
▢ Are your signs or symptoms in a new location in your body, or fully resolved in < 24 hours ?
Signs or symptoms that are consistent with a relapse and occur in a location in your body previously affected by NMO or MOGAD are key to report to your doctor or healthcare team right away. This is especially true if the signs or symptoms do not fully resolve within 24 hours of their appearance. Such signs include eye pain or vision loss, numbness or weakness in a limb, prolonged nausea or hiccups or like relapse hallmarks.
Signs or symptoms that fully resolve in < 24 hours may be less likely to be relapses but are best to inform your doctor.
If You Feel a Relapse May Be Coming, Please Contact Your NMO or MOGAD Doctor or Healthcare Team as Soon as You Notice Signs or Symptoms.
Even if You are Unsure Whether it is a True Relapse or a Pseudorelapse, Talking to Your Doctor Can Give You a Best Chance to Get Rapid Rescue Therapy to Avoid Severe Disability.
You are not being a trouble-maker and it is not a sign of weakness to report signs or symptoms of a possible relapse to your doctor & healthcare team as soon as possible after you begin to experience them. Your healthcare professionals do not want you to experience a relapse, and if you do they want to act quickly to minimize its acute and long term impact.
Can I Reduce My Risk of a Relapse ?
NMO or MOGAD relapses can strike without warning and have no apparent cause. There is exciting research underway to predict relapses well in advance to give time for your doctor to intervene to prevent relapses from occurring. However, until there is a reliable way to predict relapses, NMO and MOGAD specialists recommend best practices to help reduce risks of relapse and optimize general health and wellness:
✅ Stay on Track with Your Therapy
The approved therapies for NMO offer the best chance to reduce risks of relapse. Keeping on track with dosing of the therapy your NMO or MOGAD specialist has prescribed is key to best protection. Clinical trials in progress may lead to approved therapies to reduce risk of MOGAD relapse as well.
✅ Maintain Protective Immunization
While the cause of relapses are unknown, infections may be triggers of relapse in some patients. Maintaining protective immunization reduces risk of infection, and in doing so may also reduce risk of NMO or MOGAD relapse. Your doctor and healthcare team can help you consider routine and special protective vaccinations and when best to receive them.
✅ Record Your Signs and Symptoms
If you experience new or worsening signs or symptoms of a possible relapse, it will help you and your doctor to record them in detail. Also, recording other issues such as travel history, allergies, infection, pregnancy or other factors may help your healthcare team to diagnose. Below is an example Sign & Symptom Log that may help you and your doctor:
Example: My Sign & Symptom Log
Be Prepared – Not Pessimistic
Worrying if or when an NMO or MOGAD relapse may strike can create fear and be disabling if it reduces quality of life. In situations where there are unknowns and lack of full control, mental health professionals suggest being prepared rather than pessimistic. Here are ways patients & caregivers may reduce concerns of relapse by being prepared in advance:
✅ Understand Recent Advances
It may sound counterintuitive, but there has never been a better time to be diagnosed with NMO or MOGAD. Why ? Consider this: for 125 years after it was first recognized, there was no approved therapy for NMO. Thanks to the revolution in NMO science and medicine witnessed since 2004–2008, today there are three FDA-approved therapies for NMO that lower risk of relapse by approximately 80-90%. Further, new emerging data suggest that even if a relapse occurs while a patient is being treated with one of the approved therapies, the relapse may be milder than it would have been otherwise. A similar wave of advances is in progress for MOGAD, and for NMOSD conditions that are seronegative for AQP4 or MOG. In addition, today NMO and MOGAD are diagnosed much more quickly and accurately than ever before. In turn, patients receive highly-effective therapy earlier, reducing disability and perhaps reducing risk of future relapses or their severity. We realize it is taking time for the internet and social media to realize these great advances that now give patients and their families & loved ones much more reason to be optimistic.
✅ Establish a Relapse Contact Log
Even with these great advances, it is best to have important information at hand in the event of an emergency or relapse. Having this ready when you do not need it makes keeping track of actions simpler in the event of an urgent response.
Example: My Contact Log
✅ Develop Your Emergency Plan
Work with your NMO or MOGAD doctor and healthcare team to develop your emergency relapse plan. Such a plan should include a logical sequence of triage decision making and escalating urgency based on monitoring of signs and symptoms. A basic emergency plan is illustrated below:
✅ Prepare Your Personal Rescue Kit
If a relapse is occurring, speeding time to rescue therapy is of the essence to reduce chances of severe or permanent disability. Just as preparing for other risks in life such as fire or earthquake can greatly benefit outcomes, having a personal rescue kit for an NMO or MOGAD relapse can help in case of emergency. The GJCF has assembled a recommended STAT-Pack rescue kit based on invaluable input from patients and caregivers. It is best to contain key information & medication as appropriate:
▢ Your Sign / Symptom Log (click here to download)
▢ Your Current Medication Log (click here to download)
▢ Your Contact Log (click here to download)
▢ Your Emergency Plan (click here to download)
▢ Your General Medical History (click here to download)
▢ NMOSD Experience Log (click here to download)
▢ Your Important Daily Medication(s) (if prescribed)
▢ Your Glasses, Pen / Pencil & Other Essential Items
Contents of your customized STAT-PACK can be secured in a small watertight container with neck strap for convenience.
What is an NMO or MOGAD Relapse ?
NMO or MOGAD relapses occur when autoimmune reactivity to AQP4 (NMO) or MOG (MOGAD) proteins inflames the optic nerves, spinal cord or brain / brainstem tissues. Inflammation due to relapses causes signs or symptoms that typically reflect which tissues in the central nervous system are most affected.
By definition, a relapse occurs subsequent to the first disease presentation or onset of the disease. While relapses are often called “attacks”, the term relapse more precisely distinguishes a recurring event from the onset disease episode that led to the original NMO or MOGAD signs, symptoms or diagnosis.
What Causes a Relapse ?
In a word, causes of NMO or MOGAD relapses are unknown. Relapses most likely occur when the immune system exceeds a baseline threshold of inflammation, loses its ability to control inflammation, or both. Relapses are likely due to changes in multiple cellular and molecular factors that were stable during remission. Limited data suggest relapse risk may increase in relation to some kinds of infection, during or after pregnancy, relative to high stress levels or due to other factors.
To date, results from large clinical trials have not shown any significant link between vaccination and NMO or MOGAD relapse. There are data suggesting that vaccination may reduce risk of relapse by preventing or reducing severity of infections that have been associated with relapse.
In NMO or MOGAD, other autoimmune conditions may also exist in the same person. For example, up to 25% of NMO patients also have laboratory or clinical hallmarks of lupus, Sjogrens’ Syndrome, Hashimoto’s disease, rheumatoid arthritis or like autoimmune disease. It is possible that flares in other conditions may contribute to NMO or MOGAD relapses.
What is a Relapse Prodrome ?
A prodrome is the medical term for “pre-syndrome”. It is not yet known if prodromes exist in NMO or MOGAD relapses. However, considerable research is ongoing to investigate this possible phenomenon. An NMO or MOGAD relapse prodrome may include signs or symptoms that a patient experiences preceding a relapse. While not predictive, characterizing features of a relapse prodrome could speed rescue therapy to minimize negative impact or disability and promote recovery.
Can Relapses Be Predicted ?
Relapses can occur without any warning or may be preceded by signs or symptoms experienced by a patient. Relapses can emerge in just a few hours or emerge slowly over the course of several days or weeks. Relapses can vary in severity from very mild to life-threatening or permanently disabling.
To date, there is no specific test to predict whether a patient is about to have a relapse. However, exciting new science is emerging from GJCF CIRCLES research that we hope will lay the essential groundwork to predict relapse. Our vision is that a simple relapse prediction test will eventually be available to patients or caregivers to perform at home. Ideally, results will provide ample time in advance of a predicted relapse for your doctor to intervene with treatment to prevent the relapse.
Can I Help Advance Relapse Research ?
Yes ! Consider participating in relapse prodrome or prediction research in collaboration with your NMO or MOGAD specialist neurologist and healthcare team. Every person who has been diagnosed with NMO, MOGAD or seronegative NMOSD has the power of rare to help understand these diseases, find ways to prevent and treat relapses, and ultimately find cures.