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There are some other demyelinating diseases that cause similar symptoms to MS, in which the immune system attacks and damages the central nervous system (brain, optic nerve and spinal cord). One such disease or group of diseases is myelin oligodendrocyte glycoprotein antibody disorders (MOGAD).
Find information here on MOGAD, including information about the disease, recent research findings and resources for people living with MOGAD.
Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an inflammatory demyelinating disorder of the central nervous system (CNS). It is different to both MS and NMOSD. Myelin oligodendrocyte glycoprotein (MOG) is a protein which is present on the outermost layer of special cells called oligodendrocytes in the CNS, which produce the protective myelin sheath around nerve cells. In MOGAD, the immune system mistakenly makes antibodies targeting MOG, which results in demyelination. As there is some overlap in signs and symptoms of MOGAD, NMOSD and MS, it has sometimes been difficult to separate the three different diseases, and every so often NMOSD and MOGAD were previously diagnosed as being a form of MS. This happens less frequently now with the availability of specific diagnostic blood tests for the detection of these important MOG antibodies, and recognition of the clinical and radiological features (MRIs of the brain, optic nerves, and spinal cord) which distinguish these three disorders. We now know that MOGAD is a distinct disorder, separate from both NMOSD and MS, with different underlying processes occurring in the brain, optic nerve and spinal tissues, and unique clinical and radiological features, treatment approaches, and prognosis.
Most cases of MOGAD appear with optic neuritis (inflammation of the optic nerve behind the eye) in one (unilateral optic neuritis) or often both eyes (bilateral optic neuritis). Optic neuritis causes visual disturbances as messages passing between the eye and the brain are disrupted due to the damaged myelin. This includes blurred vision, problems seeing colours, and partial loss of vision. Optic neuritis can also be painful, especially with eye movement, as the optic nerve is inflamed and swollen. Some people with MOGAD may present with transverse myelitis (inflammation of the spinal cord), either by itself or in combination with optic neuritis, although this is less common. Transverse myelitis may present with weakness or sensory changes of the limbs, and problems with bladder, bowel, and/or sexual function. One of the most common presentations of MOGAD in children below the age of ten is acute disseminated encephalomyelitis (ADEM), where children present with inflammation of the brain (encephalitis). This is quite different to the symptoms of MOGAD described above and can cause decreased levels of consciousness, headache, seizures, behavioural changes, and may occur in isolation or together with optic neuritis and/or transverse myelitis.
As a relatively new diagnostic category, the exact incidence and prevalence of MOGAD in the Australian community is not yet known, but that is gradually changing as identification of MOGAD improves. Based on Australian research and international studies, MOGAD appears to be in the order of 2-4 times more common than NMOSD, but significantly less common than MS. MOGAD can present in children, and young adults, but does occur in older age groups as well. Unlike both MS and NMOSD which have a greater prevalence in women than men, MOGAD appears to affect both genders almost equally. There is no clear racial association seen in MOGAD, with most people in the Australian MOGAD population being Caucasian.
Both MOGAD and NMOSD may present with involvement of long segments of the optic nerves (longitudinally extensive optic neuritis) and spinal cord (usually three or more vertebral segments, referred to as longitudinally extensive transverse myelitis). Both disorders may present rather quickly with significant disability. However, there are additional clinical and radiological features (MRIs of the brain, optic nerves, and spinal cord), and therapeutic responses which help to differentiate between NMOSD and MOGAD.
MOGAD patients with optic neuritis may present with swollen optic discs more commonly, which can be visible by bedside examination with a special light instrument – this is less common in NMOSD. Additionally, seizures are more commonly seen in MOGAD (mainly with ADEM in children) but are very rare in NMOSD.
Radiological features may also be of assistance in differentiating between the two disorders, with different features on MRI around the optic nerves and the positioning of damage on the visual pathway. In the spinal cord, involvement of the lower part of the spinal cord that controls bladder and bowel function is more prominent in MOGAD than NMOSD. Brain involvement in the two disorders may also differ. Brain lesions in MOGAD are present in only a third of patients, and are often described as being much bigger than the usual lesions seen in MS.
In some people, it can be very challenging for the medical team to distinguish between the two disorders and depending on the clinical presentation, it is likely that both antibodies, for NMOSD aquaporin – antibodies (AQP4 and MOG) will be sent for testing in the blood simultaneously. In fact, about 40% of people negative for AQP4, will have MOGAD.
People with MOGAD generally have a better clinical outcome compared to NMOSD, although a small proportion of people with MOGAD may have residual disability. There may be some people who have clinical and radiological features suggestive of an antibody-mediated demyelination rather than MS, who are negative for both AQP4 and MOG antibodies. These people are referred to as having ‘seronegative’ demyelination and pose an ongoing challenge. However, they are currently the focus of much research attention.
Although MOGAD looks much more like NMOSD than MS, there is still overlap with some features of MS. From a clinical perspective, bilateral optic neuritis (affecting both eyes at the same time) and longitudinally extensive transverse myelitis are rarely seen in MS and are common presentations of MOGAD and NMOSD. However, there remain some overlaps to be aware of. People with MOGAD can also present with unilateral optic neuritis (affecting one eye) and short transverse myelitis, which are common findings in MS.
Radiological changes in the brain that are very characteristic of MS include lesions around the brain ventricles and in the corpus callosum, which is a thick bundle of myelinated nerves joining both sides of the brain. These are uncommonly seen in MOGAD. The larger brain lesions typical of MOGAD are extremely rare in MS. Other important distinctions are that the accumulation of silent brain lesions (brain lesions that don’t result in an obvious attack) is well-known in MS but rare in both MOGAD and NMOSD. Furthermore, brain lesions in MOGAD tend to resolve completely or close to completely after treatment, while in MS there are often residual lesions despite treatment and clinical improvement.
People may respond very differently to therapy. MOGAD is classically very responsive to corticosteroid treatment (methylprednisolone or oral prednisone) with rapid recovery. However, people with MOGAD may relapse quickly if the steroids are weaned too rapidly or on lower doses of steroids, so this needs to be managed carefully with cautious weaning of the dose by the treating clinician (this is called “tapering” the dose down). In MS, corticosteroids are not routinely used unless patients have significant disability with a relapse and tapering of the dose is usually not required after a short course.
Accurate diagnosis of MOGAD is critical as treatment options are different to those used in MS. Using MS disease modifying therapies for MOGAD can be ineffective, resulting in permanent neurological disability and may lead to worsening of disease activity.
The most common MOGAD presentation in children is acute disseminated encephalomyelitis (ADEM), which may often be preceded by a systemic infection such as a respiratory infection or a gastrointestinal illness. While over half the cases of paediatric ADEM may be monophasic, meaning it does not recur after recovery; a proportion of children with MOG antibodies will experience relapsing disease. Therefore, careful monitoring and follow-up is required, particularly early in the disease course. Attending future appointments for continued monitoring is key to the best outcomes.
Clinical examination and radiological assessment (MRIs of the brain, optic nerves, and spinal cord) are likely to be the first assessments for patients presenting with demyelination. In people in whom MOGAD is suspected, blood samples will be sent for the detection of the MOG immunoglobulin G (MOG IgG) antibodies. It should be noted that sometimes MOG IgG presence can fluctuate and may need repeating. It is still unknown whether MOG IgG levels over time can also help doctors predict a relapsing disease course or help with forming a prognosis, however there is much research currently being undertaken to identify biomarkers (signs) which can assist clinicians with decision making. Clinicians may additionally use investigations such as optical coherence tomography (OCT) in MOGAD patients with optic neuritis, to monitor retinal nerve fibre thickness and identify the risk of permanent visual loss, to help guide management decisions.
Initial treatment often consists of high dose corticosteroids to reduce inflammation. This may be in the form of a short course of intravenous methylprednisolone followed by a taper of oral prednisone. As mentioned above, people with MOGAD often have a good response to corticosteroids but may tend to relapse on rapid corticosteroid weaning or cessation. As a result, clinical practice may involve cautious weaning of the corticosteroid dose to try to reduce the risk of relapses. About half of people with MOGAD experience a monophasic course and do not have a further relapse requiring treatment after this. However, the remainder may go on to have further relapses (relapsing MOGAD), at which point the need for maintenance immunotherapy to suppress the immune system will be considered. There is accumulating evidence that intravenous immunoglobulin (IVIG) from healthy donors, may maintain remission. The use of other immunosuppression including monoclonal antibody therapy that depletes B cells (such as rituximab) has been shown to be helpful in some, but not all people with relapsing MOGAD. If people with MOGAD maintain disease remission for a period, a trial off therapy may be considered by the treating clinician. This treatment plan differs to that seen in NMOSD, where immunosuppression is usually lifelong.
Evidence is being gathered by collaborative multicentre and international study groups which should shed light on optimal treatment protocols for the future. All patients need very careful follow-up from their treating neurologist and neuroimmunology team to evaluate the effects of the treatment and to inform a management plan.
Commonly, people with MOGAD are looked after by neurologists who usually also specialise in MS and NMOSD, as MOGAD also falls under the category of demyelination. Additionally, there may also be immunologists and ophthalmologists involved, as well as other specialists such as rehabilitation, continence, and psychology, depending on the functions affected by MOGAD. As with MS and NMOSD, it is important to let your healthcare team know of any struggles or concerns being experienced, no matter how insignificant they may seem, so that any help or referrals can be organised early.
There remains much to learn about MOGAD, which was first identified as being an important cause of paediatric demyelination in 2007, and adult demyelination in 2014. The field is relatively young, but research in MOGAD is progressing rapidly both in Australia and internationally. Most major scientific and clinical meetings focused on MS will have dedicated streams to explore MOGAD and disseminate the latest research and management advice for both clinicians and patients. There are current international expert panels and collaborations with the aim of developing the first consensus diagnostic criteria in MOGAD, understanding the clinical and radiological evolution of this condition over time, and unravelling the underlying disease processes and how it develops, to better refine treatment. The next few years will undoubtedly improve our understanding of MOGAD.
If you or a loved one has been recently diagnosed with MOGAD, you will most likely be wondering what comes next, and what the future holds. As with other demyelinating conditions, MOGAD presents differently for each person, and whilst the prognosis can be good for many people, it takes time to establish how MOGAD will affect each individual in the long-term. However, there are some common factors that unite people with MOGAD, and indeed most of the demyelinating disorders. The suddenness of the onset, the worry about vision loss, whether walking will be affected and if and where the next attack might be are some common worries felt by people with MOGAD. In the beginning, there may be some confusion as the medical team try to unravel the cause of the demyelination and very likely many tests, examinations and blood and spinal fluid samples collected and then often waiting for the results to all come together to confirm the diagnosis.
In the early stages some people want to learn all they can about MOGAD from as many sources as possible, and for others, they simply want to rely on the advice of their specialist or medical team. Most people are somewhere in the middle of these two situations, keen for some information to better understand MOGAD and the impact it is going to have on their lives, but also not wishing to be overwhelmed with too much information. Whichever way you are leaning, do what feels right for you and your loved ones, and be willing to ask for help as, and when, you need it. The resources listed on this page are trusted sources of support and education and can also be helpful for family members and friends to learn about MOGAD. The symptom pages on MS can also be helpful, as many of the symptoms are similar and management strategies for MS can also assist in MOGAD.
The first few months after diagnosis can be difficult as you navigate a disease you had very likely never heard of before the first relapse happened. As such a rare disease, many of your loved ones and work colleagues have probably never heard of MOGAD either, which makes it hard to explain to others when you are just starting to understand things yourself. If optic neuritis has been a feature of the first attack of MOGAD, it can be a worrying time as vision is affected and you might be concerned about making a good recovery and being able to function at work or home. Parents of children presenting with ADEM may be feeling particularly vulnerable at this time, after seeing their children experience seizures or behavioural changes that can occur suddenly with acute encephalitis, and also being subjected to an invasion of tests and scans. It is important at this time is not to overwhelm yourself too much, to just take one step at a time, surround yourself with support, ask questions as they come up for you, and to ask for help if you need it from your medical team.
Keeping a pad and paper (or phone notes) handy is a good idea to jot down things as they come to mind to ask your medical team at your next appointment.
Sometimes medical appointments can seem overwhelming and there can be a lot of new information, so it is good to have questions prepared before you attend to help keep you on track and come away feeling more empowered with the knowledge you need.
It is important to let your medical team know how you are feeling and to ask if you need professional help along the way. This can happen at any time, not just after a first attack. Parents of children with ADEM may also need some help at this time to help them adapt to the suddenness of the situation and what is needed along the road to recovery.
In the early days, mental health can come under threat from many different aspects. This may include the suddenness of the relapse, the severity of the relapse, the many tests to secure the right diagnosis and most likely consultations with several doctors along the way, as well as the various medical treatments and drugs, including high dose corticosteroids. It is important to let your medical team know how you are feeling and to ask if you need professional help at any stage. Connecting with a psychologist can be empowering and helpful in learning tools to help you navigate living with an unpredictable disease. It may be helpful to discuss this with your GP or healthcare team at each visit to stay on top of things and identify early which referrals may be of the greatest help to you. Living with MOGAD requires a partnership with your medical team, and one that benefits greatly from open and honest communication. Additionally, there may also be immunologists, ophthalmologists, rehabilitation specialists, continence specialists and psychologists involved in care, depending on the functions affected by MOGAD.
The resources section below lists important sources of information that may be helpful to you and your loved ones. Practical help can be sought from the state and territory MS organisations, who have expertise in demyelinating diseases and can advise on sources of help and various forms of allied health therapies. Some people benefit greatly from support groups where they can talk to people who have been through similar experiences, which provides understanding and encouragement. The most important thing to remember is that your journey is your unique experience, and to ask for the help that you need.
Most large tertiary hospitals in capital cities Australia have a dedicated Neuro-Immunology or MS healthcare team care for people with MOGAD, usually a neurologist and MS Nurse and sometimes other multidisciplinary staff such as psychologists, Continence Nurses, and physiotherapists, but this does vary from site to site. People living in regional areas may visit their specialist from time to time and be managed in their local community under the care of a local physician or GP. MS Australia provides a list of MS centres around Australia.
MS Australia’s member organisations in each state and territory understand MOGAD and can assist with a range of services and support including referral advice and information, everyday tips and tricks, education, emotional support, wellbeing support, symptom management, opportunities to connect with others, employment support, living arrangement support, individual assessment and NDIS and My Aged Care support.
The MOG project is an American web-based patient support and resource hub and has educational and treatment explanation downloads, including podcasts (called Mogcasts) and a MogBlog. The MOG Project is supported by internationally recognised neuroimmunology specialists and scientists and has valuable information about current and future treatments and many presentations and discussions on current developments in MOG diagnosis and treatment.
