Myasthenia Gravis Learning Zone
Transcript: A case of myasthenic crisis
Tobias Ruck, MD, MHBA
Interview recorded July 2025. All transcripts are created from interview footage and directly reflect the content of the interview at the time. The content is that of the speaker and is not adjusted by Medthority.
Hello, everybody. I'm really happy to share a very interesting case of you, a case of myasthenic crisis. My name is Tobias Ruck. I'm Director of the Department of Neurology at the University Hospital Bergmannsheil Bochum. And so for the first part, of course, maybe we have a quick look, what is a myasthenic crisis? How is it defined? We know that there are different definitions out there, but the most common is that there's an acute worsening of myasthenia gravis requiring a ventilation. It might be invasive or non, not invasive. And in some cases also it's implemented that there's a need of parenteral nutrition. So a very severe state, which might affect approximately 10 to 20% of individuals in MG, and still is associated with high mortality. So this is something you should be aware of, and know of course, also the red flags to, yeah, start therapy already in incipient crisis or exacerbation to prevent mortality. So most common cases are infections. And another common cause is medication errors. So most of the time maybe antibiotics that are not allowed for MG, so always check medications in MG, as you might be familiar of.
So if you see the patient, and here we have a patient, which you can see on the right lower side, who presents with a dropped head. So severe weakness of the neck flexors. And this is always, or in many cases, associated also with swallowing and ventilation difficulties. Also, the chin might have dropped, and there might be ineffective swallowing, coughing, throat clearing, also nasal speech. And also in many cases, as I said, it's related to infections. There might be an infection that needed antibiotic treatment in the past, yeah, two weeks. So be familiar with those red flags. This is the worst case scenario of myasthenia. And this is something where you should be really aware of and keep the patient in your clinic, or admitted her or him to your ward. So of course, this is not all myasthenia. Myasthenia is very heterogeneous. It might affect all voluntary skeletal muscles in many cases and sometimes also isolated.
There's ocular symptoms like ptosis, diplopia. We have bulbar symptoms like dysphagia, dysarthrophonia, or facial weakness, more severe, and also always leading to more vigilance, or should lead to more vigilance as respiratory insufficiency, and of course also the extremities or the voluntary muscle of the extremities and also the whole body can be affected. Now let's have a look at the case. So the patient was 69-year-old female patient. She was first diagnosed in the year of manifestation in 2015, so pretty early on. Not in our hospital, but in another hospital, and showed up firstly with the first crisis in 2021. You see what is common in all patients that are above 60 or 70. We have a lot of common or concomitant diseases like arterial hypertension, hypocholesterolemia, chronic heart disease, and COPD. So quite some risk factors that a myasthenia crisis could also be life threatening, but also those factors are themselves an increased risk for a myasthenic crisis. Serostatus was typically with AChR antibodies, reducing mean test was positive, so no doubt on the diagnosis, thymectomy was not performed, since she was pretty old and also very fragile. And thymectomy is more or less refrained, or should be restricted more to the youngest patients early on after diagnosis, so we did not perform this in this case. She was of course treated with pyridostigmine, so symptomatic treatment prednisolone in the first year. She had a lot of weight gain, so as always, azathioprine was induced to spare the steroids. You see one of the common side effects, there was an increase of liver enzymes. We switched to mycophenolate mofetil, two grammes a day. But this was not sufficient to control the disease, so she also received an add-on of IVIG every four weeks.
But you already see with the repeated crisis in 2022 and '23 where plasmapheresis or in one case, also immunosuppression was needed. The disease was not very well controlled. With the last crisis, she showed up once again in our emergency ward. She needed first non-invasive ventilation, then also invasive ventilation. We did seven cycles of plasmapheresis, but there was really no effect, and no sign that we can get rid of the ventilation. So we thought one of the good parts, and the very good parts of the new add-on therapies like efgartigimod as an FcRn inhibitor. Also later I will show the complementary, which just can also be used is that their effects is very fast, and that's what you can also see. We applied here efgartigimod in this case, and only after one and a half week the patient stabilised, and was able to be extubated to get dismissed of intensive care. Of course was also still being at the normal ward for one or two weeks, but then could or was able to go home. Then we continued efgartigimod, like the first time, the common interval of four weeks, which we now increased to six weeks. You see it's already a bit time since the myasthenia crisis was incipient and now she's on six weeks intervals, no myasthenic crisis. She still has some myasthenic symptoms, but is back to normal, so to say. So activities of daily living are more or less possible for her, but there's still some fatigability, but she's very happy with the therapy. But yeah, treatment is good, but prevention is always better. So we should be aware of that there are already some hints that we can prevent crisis. We did this study with some the German centres, together in a, by large cohort. We had to look at exacerbation and crisis risks and what were the most common factors that were associated with crisis or risks. And as you can see, it's really related how good the patients are. So QMG below six, or minimal manifestation status with no functional impairment in the physical exam.
These are factors that yeah, might decide if there's a crisis or an exacerbation. If patients reach those aims, they are at much lower risk for exacerbation of crisis. And very interesting is the right side, as you can see, so the ox ratio from a myasthenia crisis, it's not only important to reach those aims, but we should reach those aims pretty fast. So we had to look at QMG cut off at six months after diagnosis, and you see, if you already are able to really control the disease, the risk for a myasthenic crisis is as low as it can be in a myasthenic patient. But after that, after QMG cutoff of six, it's linearly increasing the risk. So we have treatment, clear treatment aims, but this should also be achieved in a very fast fashion. So something like a hit hard, an early strategy with certain aims. New treatment strategies, you might be familiar with all those new studies. We know that those add-on therapies to the standard treatment can really help to control disease, to control the disease also very fast. But as I already said, different FcRn inhibitors, or complement inhibitors have already been tested also in the situation of a myasthenia crisis, which is an off-label situation, but might be a good step, a good option to bring the patients, yeah, from the intensive care back to a normal life. And currently we just have certain case reports as you can see outlined here on the left, the complement inhibitor side with the case of ravulizumab. And you also can see that there was much done and, but after ravulizumab, as you can see it also for FcRn inhibition, it took just one or two weeks, and patient were able to be dismissed from the intensive care. Of course due to the high prices, this might be expensive, since it's an off-label situation, but this might be in some cases preventing mortality. And so be a good strategy also in your practise. I hope this case report is kind of instructive for you, brings up some new ideas in myasthenia treatment. For us, it was a very educative case, where we learned a lot also on the new add-on therapies and this new therapeutic area.
This content has been developed independently by Medthority who previously received educational funding from argenx in order to help provide its healthcare professional members with access to the highest quality medical and scientific information, education and associated relevant content.