The MG questions patients almost never ask – but clinicians should
How can more targeted questioning improve care for people with myasthenia gravis (MG) ? John Vissing (University of Copenhagen, Denmark) shares perspectives that can support clearer clinical judgment and more purposeful conversations in MG care.
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How should clinicians identify patients with MG who are most likely to benefit from thymectomy? Joshua Sonett (Columbia University Medical Center, USA) outlines key factors influencing patient selection, including antibody status, disease severity, and age at onset, and discusses how these inform surgical decision-making.
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Nils Erik Gilhus (University of Bergen, Norway) highlights key takeaways from the Unlocking Myasthenia Gravis podcast series
Nils Erik Gilhus summarizes key insights from the Unlocking Myasthenia Gravis podcast series, highlighting a proactive, individualized approach to disease management that integrates emerging therapies to optimize long-term outcomes. View transcript .
Meet the experts
John Vissing is Professor of Neurology at the University of Copenhagen, Denmark, and Director of the Copenhagen Neuromuscular Center at Rigshospitalet. His research focuses on hereditary muscle diseases and MG.
Disclosures : Consultant on advisory boards for or speaker honoraria from Alexion Pharmaceuticals, Amgen, argenx BVBA, AstraZeneca Rare Disease, Dianthus Therapeutics, Hansa Biopharma, Johnson & Johnson, Lundbeck A/S, Merck, NMD Pharma, Novartis Pharma AG, Regeneron, Roche, Toleranzia, and UCB Biopharma SPRL. Principal investigator in MG clinical trials for Alexion Pharmaceuticals, Amgen, argenx BVBA, Dianthus Therapeutics, Johnson & Johnson, Novartis Pharma AG, Regeneron, Roche, and UCB Biopharma SPRL.
Joshua Sonett is Professor of Surgery at Columbia University Medical Center, New York, USA, and Chief of General Thoracic Surgery at the NewYork-Presbyterian Hospital. His clinical and research interests include thoracic surgery and the surgical management of MG.
Disclosures : No disclosures were shared at the time of publication.
Nils Erik Gilhus is a Professor of Neurology at the University of Bergen, Norway, and Consultant Neurologist at Haukeland University Hospital. MG represents his major research topic.
Disclosures: Honoraria from Alexion, argenx, Denka, Dianthus, Huma, Immunovent, Janssen, Merck, Roche, and UCB Pharma.
- Hello everyone. My name is Sir John Vissing. I am a neuromuscular specialist based in Copenhagen at the National Hospital. Today we will talk briefly about what MG patients never ask in the consultation and what we as clinicians perhaps should ask the patients. Of course, this is clearly my opinion. So usually what I do when I see the patient the first time is to chat a little bit with the patient. You know, maybe talk about their vacation or such things, just to create a personal alliance with the patients if you don't already have it, if you follow the patient for a long time. And then after this, move to asking the patient, what is really the most important question or problem that you want to talk about today? Open this, if the patient hasn't started this conversation by themselves, if they don't, I mean, you can start asking about what problems that they have. Do they need to take breaks during the day? What the routines are they doing that take longer time? And do they have specific physical symptoms such as we see here on the right side in green chewing, head control, stair climbing and so on. If it is not really clear what the patient tells you here, they maybe have problems expressing what their problem really is, I usually move on to ask them another kind of open question, and this is the patient acceptable symptom state or the past question, which just reach like this, if you had to stay in your current state for the next months, would you say that you are satisfied with your current disease state? And the patient can then answer yes or no. And typically what we see is that 1/3 of patients are not happy. And then this could prompt you to go on to ask a little bit more, also to examine the patient using some of these functional tests as we see here, or fatigue test as we see here on the right side in green. And then apply the myasthenia gravis specific disease severity scales. The MG ADL and the QMG. QMG is usually takes some long time, so you would need extra time for this. Maybe your physio can help out but also probe into depression. So this is the major depression inventory that you could pose to the patient, like 20% of patients do have depression and maybe have a hard time telling you. So you have to examine this, and the same with fatigue. Either the fatigue severity scale or the multidimensional fatigue inventory as we see here, which can then lead on to something you can actually do for the patient. The other thing you need to talk to the patient about is a shared decision making on treatment expectations. There are many things to consider. Obviously we all aim for a complete remission and I think physicians and patients align on this, but sometimes this is not achievable. What should you achieve then? What is the most bothersome symptom you should ask the patient that you have? Is it diplopia or is it that you have swallowing problems or something different? Ask the patient, what is it they want to have relieved by the treatment. Other things in the treatment that you need to accept and agree on is what side effects does the treatment actually give you? One thing is effect, but the other thing is there's sometimes a trade off, also on side effects. So this is really some of the key questions to ask the patients that they may sometimes not ask themselves. Thank you for your attention. - Hello, I'm Joshua Sonett, Professor of surgery at Columbia University Medical Center, Chief of General Thoracic Surgery and Director of the Price Center for Comprehensive Chest Care at New York Presbyterian Hospital. I'll be talking to you about thymectomy, a practical pre- to operative management roadmap for patients with myasthenia gravis. Thank you for your attention. Extended thymectomy for myasthenia gravis is now the only proven surgical treatment for myasthenia gravis. There was an important international trial that was prospective and randomized that definitively proved that for patients with clinically proven myasthenia gravis and acetylcholine positive antibodies, they had significantly better clinical outcomes at three and even late follow up five years and longer for patients with myasthenia gravis, making this a core aspect of treatment for patients with myasthenia gravis. But not all myasthenia gravis are the same. They come in different subtypes and in different pathologic etiologies. So just as we stage and assess for lung cancer, we need to stage myasthenia gravis. And it's generally staged by the antibody type, the clinical presentation, and the age of the patient. Predominantly 75% of patients with myasthenia gravis will have acetylcholine receptor antibodies that are positive. Patients, however, can have what's called MuSK positive myasthenia gravis or lipoprotein receptor 4. And then some patients, we cannot measure any antibodies. Patients with acetylcholine receptor antibodies have the best response to the thymectomy. Patients with muscle-specific kinase receptor and lipoprotein 4 receptor don't respond very well to thymectomy. Patients with seronegative can respond but also may not respond. It's a mixed bag. The Osserman classification score grades patients how they present. So 1 is purely ocular, and 5 is near death on a respirator. 2, 3, and 4, different gradations of increasing muscle weakness and effect of the patient. For the most part, we do not operate on Osserman classification 1 because they're doing too well, or 5, because they have to be significantly improved medically. 2, 3, and 4 are the target patients that do best with myasthenia gravis and of those patients. Patients with Osserman classification 3 and 4 need significant preoperative optimization to improve their outcomes. Patients can be early or late onset depending on when they present. So basically less than 50 or greater than 50, and patients in less than 50 years of age tend to do better. A summary of my assessment from clinical data of how patients respond to myasthenia gravis. They're sort of overall best benefits. So acetylcholine positive, early onset age 18 to 50 have a five-star response to the thymectomy, and the thymectomy results will make themselves evidence about at least one year after the thymectomy. Acetylcholine positive but late onset over than 50 still have a benefit, but not quite as good as the young patients. Seronegative early onset can do very well with thymectomy and are good candidates. Seronegative late onset older patients can have a response but have a much less intense response or good results from thymectomy. So consideration should be made when discussing surgery with these patients. There's two types of juvenile, less than 18-year-old patients with myasthenia gravis. Those that are acetylcholine positive and they can do well, and those that are seronegative that don't do well. Ocular well-controlled, generally can be managed medically. Ocular poorly controlled can be managed with surgery and medicine. And as noted before, MuSk and LRP-4 are not great candidates. Preoperative planning, you need to measure pulmonary function tests, the negative inspiratory force, Osserman classification, and significant bulbar symptoms. If any of these are not optimized, the patient should not undergo surgery at that time, and they should be optimized by your neurologist with maximal dosing of prednisone, mycophenolate, and then if they're really sick and prominently, a significant portion of the patients will need IVIG or plasmapheresis. More recently, new biologic agents are key to their treatment. It's important to know when you're talking to your surgeon that thymic tissue is not one gland. The thymus predominantly is located in these black lobes in the mediastinum, but thymic tissue is seen throughout the mediastinum. So you need to talk to your surgeon, and surgeon needs to understand that any approach to thymectomy, and that's almost certainly minimally invasively now, be it robotic or be it scopic, needs to include all the extra tissue to get maximum benefits of thymectomy. I thank you for your attention. - Hello. Together with Medthority we have recorded a podcast series, Unlocking Myasthenia Gravis, where we have discussed with experts on selected topics. Today I will try to sum up and give some main conclusions of this work. A typical aspect of myasthenia gravis is variability. It is fluctuations during the day, from day to day, and, more important, risk exacerbations that can be severe and even life threatening. Such exacerbations can be triggered, but also come without any obvious triggering factors. Infections are the most common triggers, perhaps also stress, more difficult to define. And in women, hormones, for example, during pregnancy or related to the menstruation cycle can be relevant and important triggers that sometimes can be avoided, or perhaps treated. Medications, new medications can also trigger deterioration and severe exacerbation in myasthenia gravis patients, but this is individual. These responses varies between patients. Most important deterioration is a crisis with respiratory insufficiency and need of intensive care treatment, most commonly with respiratory infections. So the aim of the treatment is to be proactive, to treat efficiently so that exacerbations are avoided, and also trying to avoid known triggers. There are luckily many new treatments that improves the situation, but still from today's studies, there is an annual risk of myasthenia crisis in an individual patient of one to 2%, and a lifetime risk of at least 10%. Treatment goals should be ambitious. One should have ambition and the patient should have an ambition of minimal symptoms only or even pharmacological improvement with no symptoms at all. Also, in addition to symptom control, it is an aim to control the disease itself. There are several treatments, and there is an increasing use of new treatments, such as complement inhibitors and FcRn inhibitors. Also, B-cell therapies and T cell therapies are important. Such therapies will define target in the immune system, although they are not specific for the acetylcholine or MuSK-directed immune response. In addition to this active treatment effect, it is important trying to avoid side effects that are common, for example, with corticosteroids. There is also an important aspect that many patients are elderly. Most, the majority, are above 65 years, also those with long-standing myasthenia and those with debut of their myasthenia. This is relevant for comorbidities and polypharmacy. In this treatment of myasthenia gravis, the active cooperation with the patient is most important. This communication can be digital but also personal. The follow-up of patients is based on what patient tell you, what you can find with your examination, but most important are patient-reported symptoms. And there are as well assessment scales for these examinations, MG-ADL, QMG, and also quality of life are important parameters well known in the follow-up of myasthenia gravis patients. And by such follow-up, it is possible to recognize early warning signs for the patient, and also to give a broader support and follow-up of the patients. So I think the conclusion on this podcast series, the main conclusion is that you should treat actively and ambitiously, that you should use all treatments, all drugs that are available in your center, in your hospital, in your country. You might often combine them and you should follow-up the patient and listen to the patients during the whole time period of their myasthenia gravis. Thank you for watching this video.
John Vissing, MD, DMSci
Joshua Sonett, MD
Nils Erik Gilhus, MD, PhD