Some patients with heart or other problems, who cannot be treated with IVIg
anymore, are treated with plasmapheresis. But after some plasmapheresis sessions If immunosuppressant drugs and steroids prove not sufficient, rituximab they developed allergy. What is the choice of therapy available?
Allergy to these treatments are extremely rare in my experience (to say the truth, I have no experience of it). Unmanageable side-effects are more
What are the therapeutic options for patients allergic to cortisone and mestinon?
common. If corticosteroids cannot be used, immunosuppressant drugs are recommended. In France, Mytelase can be used instead of Mestinon
Is there an alternative therapy for those patients who receive IVIg but are allergic to
Plasmaphereses can be tried, beside other usual immunosuppressive drugs
Yes. Association to another auto-immune disease is usual, but the type of
Are MG patients more susceptible to other autoimmune diseases ? Like autoimmune abnormality varies a lot. Thyroiditis is probably one of the most common
abnormalities. I have no experience of auto-immune deafness, which sounds very rare to me.
Another disease can render it difficult to appreciate the symptoms; for example, arthralgia can blur the appreciation of muscle strengh. When there
Could associated autoimmune diseases modify the outcome of MG? in which way?
is pain, it is difficult to maintain strength. The treatment can also be complicated. Fortunately, most auto-immune disorders require the same type of treatments as MG.
In some cases only. ANA are not very specific and one should consider the
Could the presence of ANA antibodies and other autoimmune antibodies in a patient level of the antibodies (a 1/80 level is very common in patients who get an
with MG only (no signs of other associated disease) predict the development of a
immunological workup for any reason). Some antibodies are more specific.
For example, a high level of anti-CCP antibodies is probably linked with a risk of developing rheumatoid arthritis.
There are several possible reasons: 1) Because of the specificity and affinity of these antibodies. For instance, some patients have a majority of their antibodies directed against the fetal form of the AChR. 2) because of other factors, such as the level of complement that participates to the severity of
Why some people are in remission although they still have antibodies ?
the symptoms; 3) another possibility is linked to the ability of the muscle to compensate after the autoimmune attack. Even if the RACh is degraded by the antibodies, it is re-synthesized, that compensates the negative effect of the antibodies.
EMG can be repeated (enough nerve-muscle couples must have been tested in the first place), as well as antibodies if performed at a very early stage of
Is there any medical examination that can confirmed the MG diagnosis when you are the disease. Obviously, the development of routine tests for unusual
seronegative (apart from the scanner of the thymus, and EMG and the antibody titer antibodies (low-affinity AChR ab, anti-LRP4,.) will help to solve some cases.
of AChR and MuSK) ? Effort test can not be proposed ?
In other cases, still possible, clinical follow-up by expert teams will only allow to confirm the diagnosis.
Many CMS do not resemble classical auto-immune MG, and patients with pseudo-myopathic CMS sometimes wait for a long time to be diagnosed as
Why it is so difficult sometimes to diagnose congenital myasthenic syndrome (CMS)?
having a potentially curable disease. In cases looking like auto-immune MG,
Is it because it is less widespread and looks like autoimmune MG ?
seronegativity, young age at onset and resistance to treatments should draw attention on a possible genetic case.
Is it difficult to distingush between an autoimmune MG seronegative and a
The clinical pattern is often distinct, with LEMS presenting as proximal weakness associated with dry mouth or other signs of autonomic nervous
How is myasthenia reconciles with Lambert-Eaton syndrome?
system dysfunction. EMG is crucial do distinguish both entities of to confirm the presence of both diseases, which occurs from time to time.
No specific criteria exist, it is true, but scores tend to give an idea of the severity of the disease. Other non specific criteria designed for muscle
Why there is no evaluation criteria for disability?
disorders (i.e. Walton of Boston) can be used, although the variability of the disease introduces a difficulty.
Myasthenia gravis can induce disability, but the severity of the disease is
Are patients with Myasthenia Gravis considered to have a disability?If yes, why there
highly variable according to the patients and in a same patient at a time and
is no evaluation criteria for disability?
Is there food recommendations for MG patients? Are Ach precursors recommended
Nothing has been yet demonstrated to the best of my knowledge.
The treaments available for MG are anticholinesterasic , corticosteroids,
Rituximab has already become a further treatment in resistant forms of the
Netherlands immunosuppressive therapy, IvIG and plasmapheresis, What can patient can expect disease.
Rituximab is one the new therapy that can be used in refractory MG
What are the latest developments or discoveries in the cure of Myasthenia Gravis?
patients. Several other therapies are under investigation and need to show a
What are the Recent advances in Research on Congenital Myasthenia Gravis?
No, but a genetic background probably induces more susceptibility to
Is Increased gut permeability may be related to myasthenia gravis? Is there a is
Yes, very likely, but there are no studies in the MG field
relation between intestinal issues and autoimmune disease(s) ?
Do you think it would be interesting to do the intestinal permeability assessment
(lactulose–mannitol) in all MG patients and consider the GI health as part of the MG Yestherapy?
Could also chronic corticotherapy be a cause to intestinal permeability
Are specialists aware from such studies of “leaky gut” in Myasthenia Gravis
For this question a proper response was not reached
(especially MG related with other autoimmune disorders)?
At this time we are performing experiments to determine if it is the case. An
Is a cytomegalovirus infection can cause the disease ?
Is urinary infections can be the cause of the onset of MG ?
An urinary infection can not be "the cause" of MG, but can trigger
How long do we have to wait until it is possible to say that MG will remain at ocular
Ptosis alone should be treated if it is responsible for some impairment.
Netherlands Is ptosis has to be treated and if yes, is surgery the best way to do it ?
Surgery is indicated only in case of fixed ptosis.
Is MG considered as a degenerative disease ?
How it is possible to have the information about the reference centers in all the
All countries do not have reference centres and do not work as national
networks. In France, the RC are easily found on the internet.
It is a difficult question, as the boundaries of "seronegative" myasthenia are
Are there differences in the evolution and development of the disease and in its
blurred by the fact that patients will prove eventually not to suffer from
treatment according to the patient is seropositive or seronegative?
myasthenia. In "real" myasthenia, my impression is that patients may respond to therapies in both groups.
Not really. In France, medical students are taught about MG and reanimators
Are there alerts in hospital emergency departments for patients with myasthenia ?
are aware of the disease. It is adviseable to always have the card delivered by the reference center which contains the basic facts about the disease.
No more, no less than in any person suffering from the problem. Adapting
Is there a solution to avoid the well know relation between stress and MG (stress
the working place should be mandatory, but if that proves difficult, anti-
worsen the symptoms) in order to help patients to feel better ?
stress approaches like sophrology, yoga or others are adviseable.
Does MG patients can present sclerites ? if yes what is their profile ?
What myasthenia association and in which country has more impact on the
For this question a proper response was not reached
relationship with professionals and institutions?
It is not well known, but there are two recent articles addressing these questions. A paper by a japanese group showed that among 371 MG
Is that the CNS affected in MG ? What about the taste ? Olfaction?
patients, 4.3% had taste disorders (higher than in the general population). Concerning olfactory dysfunction, a study by an American team in 27 MG patients revealed a smell score significantly altered in the group of MG patients
Does Myasthenia and IgA lambda monoclonal gammopathy of undetermined
Not to the best of my knowledge. There is one case described by a Japanese
significance, can be related to each other ?
Nothing demonstrated. However, having a healthy way of life is always
Is there things that can improve our quality of life on a day to day (food, sport .) ? adviseable. Concerning sports, activity should be adapted to muscle fatigue.
Do estinon 60 mg and kalymin 60mg have the same therapeutic potency? Patients I have no experience with Kaylimin, which is basically the same active
have reported that estinon 60 mg was beneficial, but now in Cyprus only kalymin
pharmacological principle as Mestinon. The substitute in France would be
ambemonium chloride (Mytelase) but it should probably not be available in
Is there a drug to substitute for estinon and kalymin?
Has Imuran an effect at short term like steriods ?
No, it takes longer (between a few weeks and several months). If you take
Is Imuran safe after 20 years of treatment?
the drug for a very long time, you should carefully monitor your skin with a dermatologist and still have a blood cell count every 6 months.
There should not be. Side-effects may though be different and changes are
Are there differences in the efficency of the different IVIG available
Mestinon 180 mg is "Mestinon retard" in France. It allows a longer delivery
We have found that in the USA and Canada Mestinon is administered in 60mg and
in the blood, and is usually prescribed here at night for patients with
180mg doses. Is the 180mg dosage available in Europe?
If you mean for myasthenia, the lighter treatment, the better. All immunosuppressive drugs and steroids will be a problem for diverticulitis. In
What is the best medication in an old MG patient presenting diverticulitis or sinusitis
that case, anticholinesterasic drugs should be used alone, and if that proves
insufficient, IVIg or plasmapheresis can be discussed. All depends of course of individual cases.
Could early immunosuppressive therapy prevent the development of the second (or
third and more) immune disease in a MG person ?
Some patients will see the disease disappear after a few months or a few
years, so yes, but it is unpredictable.
What is the success rate (a possible remission) of the surgery considering the young I have no experience of thymectomy in such a young patient. In Japanese
age (5 years old) and the ocular form of the disease ? IS there repercussions on the series, many patients are very young. Most of them have an ocular form and immune system after surgery?
Yes. However there is currently a clinical trial to bring irrefutable evidence.
Is there evidence that ensure improvement after thymectomy?
Is the new technique to perform the operation (laparoscopically with 3 incisions,
Just one of my patients got through the Da Vinci robotized thymectomy.
much less aggressive intervention to open the sternum) recommended?
Why thymectomy is not proposed for young teenager ?
It is not really known. Total thymectomy as a very young age (first months) induces significant alterations of the peripheral immune system, but this has
What are the repercussions on the immune system of performing the surgery at
not been studied in patients older than 5 years. However, on can presume
that most of the T cell repertoire has been created in the first years, and that total thymectomy will affect only slighly the immune system, when performd after 5 years.
At this time we are performing experiments to determine if it is the case. An
Can an infection be the cause of the disease?
infection can trigger symptoms, and many experimental arguments suggest that viral infections are associated with the disease.
Chapter 4 Carbon and the Molecular Diversity of Life The Importance of Carbon 1. Explain how carbon’s electron configuration accounts for its ability to form large, complex, and diverse organic 2. Make an electron distribution diagram of carbon . It is essential that you know the answers to these questions: a. How many valence electrons does carbon have? b. How many bonds can
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