we don't. this is where the problem lies. the understanding of most disease has significantly evolved over the last 20-30 years, as did better treatment approaches, stemming from this understanding. the understanding of MG has stayed exactly where it was. even worse, it seems that neurologists are even more confident then before that they know it all. therefore anything that is out of that box, just doesn't exist.

That is why a patient that had a very typical clinical picture of MG from day one (there are very few diseases that can cause such changes in muscle strength from one day to the other, in the way she describes) required respiratory support due to her illness, responded to achetyl-choline esterase inhibitors and plasmapheresis, but probably did not have a diagnostic SFEMG or antibodies to support this diagnosis was not treated as MG by any neurologist.

a normal SFEMG during such severe weakness "rules" out MG. is there anything in the medical literature to support this notion? no, there isn't. in fact there are very few studies done on SFEMG in seronegative or MUSK patients and those show that doing a SFMEG is about as good as tossing a coin.

can myasthenic weakness be without abnormal transmission of the electrical signal at the NMJ. of course it can. there are so many things that can go wrong after that on the way to proper muscle contraction.

myasthenia is not one disease, but many, each is very different in its cause, clinical course and response to treatment. I believe that there are many patients like chloe, but only a few that can articulate their experience in such a way. many are treated in this manner, but are not able to fight it, and just succumb to their illness and the way they are treated. I believe some probably die without us even knowing about it. wasn't she very near to that? possibly internet support groups like this one (that did not exist when she was struggling with her illness) may change some of this, by giving such people hope and support and feeling less isolated with their ordeal. but, without knowing what your illness can be, how do you find one? she is not a physician and probably never heard of MG during all those years.

when I hear those horrible stories, I can't avoid thinking how fortunate I am to be a physician and be able to take care of myself, and although I have my share of humiliating experiences (which in a way are harder, because those are my colleagues), I have taken care of my crises at home, where I don't have to face those idiots, and can manage my illness in the way that I think is best.

I remember how relieved I was when after one of my more traumatic hospitalizations (the last one in a neurology ward, as I promised myself that what ever happens I am never going to be hospitalized in a neurology ward again after that), when having severe shortness of breath I was surrounded by my loving family who quickly put my respirator mask on, and I thought how great it was that no one was trying to measure my VC (only to document it on the chart and do nothing about it) or tell me that I am "doing" this because I do not want to go home (as I was home).

A while ago I asked the head of our ICU (who has been of tremendous help, like almost every physician who is not a neurologist or has not been influenced by them) under what circumstances it would be dangerous for me to be at home and I would require intubation and not NIV. he said that he thinks this is not likely to happen, as I have learned to master my illness so well. recognize early signs of respiratory compromise, use my respirator before further deterioration occurs, avoid aspirations when I have difficulty swallowing, and mange to overall keep a reasonable caloric intake.

every now and then I make the mistake of trusting a neurologist again and thinking that he is going to be different, he is going to be able to think out of the box, he is going to understand that atypical MG is still MG.

but, until now I have always been proven wrong. none of them was capable of that, or of understanding the respiratory manifestations of this illness, in a patient that has learned to live with it, using intermittent respiratory support.

alice