Negative SFEMG but still have MG?
 

I have a friend in Europe who is negative on BOTH antibody tests -- negative on the EMG and SFEMG -- but, POSITIVE on the Tensilon test AND has great results with Mestinon -- she currently takes 10 tablets per day. She was a competitive tennis player and now runs a day care. She is seeing a highly respected Professor of Medicine -- he ran the SFEMG yesterday -- it was negative for the 2nd time -- and he told her that she did NOT have MG based on that result.

I think that he is wrong -- and she does, too. But, we need reports, journals, EVIDENCE for her to SHOW him. Can you tell me where to find some "hard" written proof?

Sfemg
 

Jana,

I wish it was so simple, there is an excellent review by Michael Ben-Atar, which goes over all the tests that are used for the diagnosis of MG.

there is also another study that shows that in patients with seronegative MG it is not that rare to have a normal SFEMG.

the problem is that many neuromuscular specialists do not accept those studies, or have all kinds of explanations as to why they have such results, and think that it is impossible to have a normal SFMEG in a muscle that is weak by myasthenia.

this is one of the "windmills" I am trying to fight. and it is not going to happen in one day, if at all.

my suggestion to your friend it to find a more open minded neurologist, that does not think that this is an "infalable" test.

alice

Thanks, Alice. My heart breaks for her -- and for all who are fighting "windmills". *sigh*:(

Hi Alice,
 

nice to meet you

Hi Moshe!

So glad you made it over here!! You and Alice (not her real name for obvious reasons) will have lots to talk about!!!

(((HUGS)))
jana

Jana, This is the toughest subject for me. I had a few SFEMG's and no one had told me that the Advair I was taking could make everything look normal. Any steroid a person takes can make EMG's and antibody tests look completely normal. That's their job - to suppress the immune system. Is your friend on any other drugs that would interfere with a SFEMG?

Plus, they do need to test clinically weak muscles. AND the person doing the test needs to be VERY good at it. Often it's done by those doctors who are training to do it.

The congenital myasthenic syndromes, the ones where a person has less acetylcholine (there are some where they have too much), may not show up on SFEMG. It's confusing, I know. The CMS ones are genetic, not autoimmune. They can be milder and may not show up on anything but an exercise EMG or a nasty repetitive nerve stimulation where they keep stimulating the muscle for 5 - 10 minutes. It's horrid, I've had it.

In MG, a person can go along for awhile and be "ok." The threshold where their muscles go "downhill fast" is different for everyone. If they aren't tested at the "downhill" phase of MG, they can look clinically normal and normal on EMG. That's also why some offices actually warm up someone's muscles before they test. And some do the exercise EMG's. Some of the CM syndromes tend to be okay for a long time and then the muscles simply crash. Think of all this as varying degrees of a bell curve. Remember that from school? ;)

None of this is simple. I had a borderline SFEMG but I do have antibodies. My clinical picture is MG. My Tensilon test was highly positive. My neuro-ophthalmologist diagnosed ocular MG (fatigable muscles). I respond to Mestinon and have for almost nine years (with absolutely no side effects or overdose effects). I didn't know until almost a year ago that I had positive antibodies (it was kept from me). So your friend may want to get all her records in case a doctor is doing the old "pooh-poohing" of test results (i.e., only slightly positive results).

CMS is common in Nothern Europeans. Also in some Arab populations. Heck, all our genes are intermixed across the world. They only do testing of it here in the U.S. in 2 places. In Europe, they can do it in England, Germany and France. Italy too really. Not too sure about Sweden.

A negative test, as my diagnosing neuro has said, does him no good! Only positive results and/or positive clinical exam. I am beginning to believe that unless you have MG really bad, a doctor may miss it. There are so many variables that come into play (i.e., time of day you're tested)

I hope this helps. It stinks to not have someone take your bad health seriously. It can wear you down.

Annie

Good to see you again, Moshe.

Annie, this DOES help. I think that I am going to have to see if I can get her "over here". It will be so much better for her to ask these questions -- and to be able to answer them. I'm not sure about the time difference -- I was talking to her about noon today.

CMS -- that really makes a lot of sense.

Alice,

You are so right!

I am a seroneg and 'just' passed the SFEMG. My neuro's exact words were "I hate it when this happens. (deep sigh) But it doesn't really matter, you have MG based on clinical symptoms and your clinical response to Mestinon."

Later I was told that I had jitter(s) - but they were just shy of the accepted level for MG dx.

After meeting all the folks here who are (or have had) so much trouble getting a dx - - I feel really, really fortunate I found this wonderful neuro on my second try!! And even more grateful that my symptoms are extremely mild at this point and well controlled with Mestinon.

Sue

Sue, You bring up another good point. MG is VARIABLE. Fatigable = variable.

The presentation varies. It varies as to what time of day. What you're doing. If you have other complicating factors like hypothyroidism or infection. It runs the spectrum from "able to work" to "being intubated."

And, as I've said before, MG is not inert, like some kind of gas. You add movement or heat and it "explodes." It is not a "static" disease!!! :eek:

I guess MG is a shape-shifter! ;)

So how can neurologists approach MG as if it is this finite disease with these "gold standard" algorithms, as if this disease or diagnosis is a one size fits all?

A lot of doctors are only comfortable with clear cut answers. Not that easy with MG. Lucky us.

Annie

I wanted to add one more thing. The SFEMG "results" are compared against the "mean consecutive difference" or MCD of "average" MCD for different age groups and muscles. For example, let's say the average MCD for age 40 of the frontalis (forehead) muscle is 35. What if YOUR average is lower or higher than that? Higher wouldn't be a problem but if your "normal" is 20, for example, then they will be seeing an increased jitter for you as okay.

No, I'm not saying I know more than doctors!!! Or the SFEMG experts. I'm simply looking at how they analyze the SFEMG by "averages" for specific ages and asking the question. What if you are normally "below average?" This is a simple statistics question, and one of educational standards, that questions what "normal" really means.

There is a pediatric neurologist in England who has seen firsthand that the SFEMG's of many children with MG needed to have the "average" MCD lowered. They obviously had MG but were showing normal on SFEMG.

I compare it to B12 results. My Mom was in the "normal" range of B12, or what the lab calls normal. But she was on the lower end and had parasthesias in her hands and feet. After taking methylcobalamin for a few months, they went away.

I just don't think this is an easy disease to diagnose for LOTS of reasons. And if the test you're using has problems, because not everyone is the same, then how can they expect the results to be accurate? Again, they aren't asking enough questions and have "guidelines" that are simplified to make it easy for them. Well, it's not easy for us, the patients.

Doctors cling to certain standards because THEY need to be right, to know they've made the right decision. But diseases often don't stick to the same standards. They have a mind of their own. And we aren't all the same, thank goodness. And our disease "looks" different on each of us. Cookie cutter thinking is easier but it isn't exactly effective. Or nice.

Annie

http://emedicine.medscape.com/article/1141438-overview


I can't stop thinking about this issue!

Men and women aren't created equally. It takes longer for women to be diagnosed than men (7 to 1 difference!). It has to be more than sexism causing this.

I know that men and women lose weight differently. Women also store estrogen in their fat cells. There's skin on top, then fat, then muscle. I found the article below on the "fatigability" differences between men and women. Very interesting. So, if men fatigue more quickly then maybe their SFEMG's will look more dramatically abnormal. And maybe obesity or being overweight is one issue but maybe the "type" of fat above the muscle is an issue too.

http://ep.physoc.org/content/93/7/843.full

So, maybe women who have "mild" MG, can look normal because their "jitter" doesn't go up as much and may be determined as a negative test when, in fact, it is positive. And maybe because it takes longer to show "fatigue" on an EMG.

If I had more energy, I'd dig around more. This has been nagging at me for awhile now. Please let me know what you think of all this. Thanks for getting me thinking, Jana.

[QUOTEI just don't think this is an easy disease to diagnose for LOTS of reasons. And if the test you're using has problems, because not everyone is the same, then how can they expect the results to be accurate? Again, they aren't asking enough questions and have "guidelines" that are simplified to make it easy for them. Well, it's not easy for us, the patients.

Doctors cling to certain standards because THEY need to be right, to know they've made the right decision. But diseases often don't stick to the same standards. They have a mind of their own. And we aren't all the same, thank goodness. And our disease "looks" different on each of us. Cookie cutter thinking is easier but it isn't exactly effective. Or nice.

Annie


I can't stop thinking about this issue!

Men and women aren't created equally. It takes longer for women to be diagnosed than men (7 to 1 difference!). It has to be more than sexism causing this.

.[/QUOTE]



Annie,

there are many biological differences among men and women, and this is being gradually recongnized over the last decade. and diseases do manifest differently, normal values of tests are different many times, and even tests such as a stress test are of different accuracy. but beyond that there are numerous problems with the SFEMG.

the bottom line is that it is not as accurate as those that use it would like to think.

all a normal SFEMG can tell you (assuming that it was done properly and there are no technical problems, or missinterpertation such as you have reffered to among others) is that there is a normal transimission of the signal from the nerve ending to the muscle fiber.

but what if the problem is after the receptor? what if this electrical signal is not properly translated into a muscle contraction?

it is like your computer is not working properly, and a technician comes, sees that it is plugged properly into the electical socket and says to you that there should be no reason for the computer not to work properly.

there are multipe steps from the activation of the acetyl-choline-receptor to the generation of muscle contraction. and all those steps should work properly, in order for muscle contraction to occur.

very schematically-calcium goes into the cell, it then leads to the release of calcium from intracellular reservoires (this is governed by other receptors, which are activated by calcium that enters the cell, but also caffeine and other pharmacological agents), this in turn leads eventually to the "sliding" of myosin and actin, which leads to shortening of the muscle fiber. each of those steps involves multiple proteins, many of which function is poorly understood at this point. MuSK, DOK-7, Rapsyn- to name a few.

I was quite surprised to find out that the assumption that any process that leads to fatiguable weakness should also involve the acetyl-choline receptor directly or indirectly, is not based on any evidence.

to me it seems that certain "axioms" have been created over the years, which are enforced by everyone agreeing to them. or as in the "hunting of the snark" by Lewis Caroll- what ever is said thrice can't be wrong.

and most patients don't "dig" and try to understand, they just accept what they are told. I probably would have done the same, if it wasn't becoming totally ridiculous, and if I didn't have an enormous amount of "thinking time" (thanks to one of the world leading experts, who gave me treatment that totally "knocked me down", and gave a few months in which I could not do much more then think, most of the time) .

alice

nice to meet you too.

Annie, you are thanking ME?? Thank YOU!!!!!!!!:hug:

Hello,

I am Sonja from Belgium. I'm the person who had the single fibre that was negative.
Thank you all for the answers. I'm gonna give the doctor a call tomorrow!

Sonja

Hallo,

I am Sonja from Belgium. I'm the person who what the negative sfemg. I don't have antibodies but I have had a positive tensilontest.

:)

YAY, Sonja!! So GLAD that you joined us!!!

(((HUGS)))
jana

Thanks, Alice, that explanation is exactly the one a neuro gave me once. He's the kind of neurologist who wouldn't dare go against the "status quo" openly but would talk straight to a patient.

I imagine that when someone says "I'm right" for decades, it might make that person - and their colleagues - look unprofessional or unethical if they said now that they were "wrong." And it might make others not trust them again. Which, in my opinion, is super silly. None of us are perfect. And science and the human body is never easy.

One problem I have with this is that these "professionals" aren't thinking. Or learning. Or they know all this and are hiding it. Like you said before, Alice, "hoarding" the information until they can make the next "great discovery" or drug.

What makes me literally crazy about all this is that so many patients are suffering. Patients who so obviously have fatigable muscle weakness.

Hi, Sonja. Welcome. I hope you will start a post and tell us about yourself! Sonja, I would add to that . . . get the copies of ALL your tests. I had a positive antibody back in 2002 and my doctor didn't tell me about it nor did he give me a copy. It wasn't in my records either! I had to go to the clinic who ran the test to run it down - seven years later.

And, yes, Jana, it was indeed you who stirred all this up (via Sonja). So thank you both.

Annie

"He's the kind of neurologist who wouldn't dare go against the "status quo" openly but would talk straight to a patient."


that's a shame. because it seems to me that unfortunatley most of the more open-minded, humble neurologists are intimidated by the more arrogant ones. I have to admit that I have not seen it to that extent in any field of medicine. although, medicine does tend to be hirerchacal and there are those who are considered leading experts in every field.


"I imagine that when someone says "I'm right" for decades, it might make that person - and their colleagues - look unprofessional or unethical if they said now that they were "wrong."

this in fact is not true in science or medicine. many approaches that were considered "state of the art" 20 years ago, are now abandoned. the advances in medicine are dependent on constant constructive criticism, questioing of what you have known, in view of new evidence, some degree of skepticism (obviously you can't make major changes in your managment approach based on anecdotal evidence).

this is true for the specific patient as it is for the entire field.
I have used this quotation from the "hunting of the Snark" when as a resident I presented a patient in which we all went wrong (including me), during morning conference. I talked about the dangers of being given false reassurance about your (in this case completely wrong) diagnosis by the fact that all the rest of the team thinks like you. I said that there was a hidden clue that we all missed, because we didn't have to think too much about the diagnosis as we already "knew" what it was.

As physicians we have to be ready to admit our mistakes (as hard as it is) and be ready to learn from them and from the mistakes or others as well.
I think that this was one of the best parts of my training that we had those conferences, where we discussed medical errors, without pointing fingers, but in order to learn as much as we can from them.


"What makes me literally crazy about all this is that so many patients are suffering. Patients who so obviously have fatigable muscle weakness. "


I fully agree with that, and feel the same way. and that is why I am trying to do everything within my ability to change that.
the amazing fact is that the neurologists know it as well, but just see it as some "natural disaster" they have no control over, and can do nothing about. instead of understanding that some of it, at least, is due to their own missconceptions. (at least this is the conclussion I have reached after almost a year of learning as much as I can about this illness, and the way it is managed-from the patients, the medical literature and the neurologists, and I know that many physicians and patients will not be ready to accept what I say).

I know that at least in my case they have made serious managment errors, based on such missconceptions. and I am quite sure that I am not the only one. although I am really not sure what the true extent of the problem is. and how many more patients are missmanaged, but sort of accept it, and think that it is OK.

according to the leading neurolomuscular experts, it is very rare for a patient with MG, to be missmanaged in that way, once they have reached a good MG center, and had all the proper tests, but I am really not sure how true this is.
my own experience is very different.

alice

Hi Alice,

I got an email-adres from Prof KUKS in the Netherlands. I wrote him bij question about the single fibre negative, antibodies negative and that the doctor concluded that if he couldn't see it in the mussle I don't have mg.
I've got a mail back, he says that there are exceptions with seronegative mg, different variants.

I called my doctor about it but offcourse he didn't liked that, he says he has a lot of experience with single fibre and he has never had a patient like that.
Then i thought offcourse not because he sends them back home without nog diagnose.

I believe that you are unfortunately quite correct in what you are saying.

I also do not think that there is any way for you to convince this doctor that he is wrong, even if there are others that think differently.

my practical advice to you, is to find some one who is ready to accept this, even if it requires some efforts. why not go to the neurologist who wrote you this e-mail?

alice.

Alice, maybe I'll do that but it is 4 hours away and 4 hours back.
So to go back for other examinations is difficult. I'm not sure that he's gone give me the diagnose. I hope so. I don't know if it is possible with the insurance to. If it is only one visit, no problem but otherwise it will be difficult.

I would be ready to go any distance to see a neurologist that is my real partner in this. (and actually did).

the funny thing it that my current neuro is about half an hour away, and I have no problems with the medical insurance because we belong to the same medical system.

but, I have seen and consulted neurologists all over the world.

and gradually learned more about this illness, and could judge better who is just "nice" and who can really be of potential help.

I was not ready to accept the answers- I don't know what to do, there is nothing more that can be done, or all you need is a good psychiatrist, without persuing it.

there was a point where I asked myself-is this life worth living? and my answer was-no!, so the next answer I gave myself was-then do something to change it!

alice

Alice,

If I was sure that he would give me the confirmation of the diagnose I go immediately.

Sonja,

Unfortuanately, there are never any guarentees in medicine (or life for that matter) - and esp. with this little gem called MG!! If it were me, and I was unhappy with my condition and of the belief that there was a probable diagnosis with another doc, I would make the effort.

Perhaps the other alternative would be to contact this doctor's office to see if he has a collegue (of same mindset) that was closer to you and covered by your insurance. Worth a try anyway.

It is sad, but while the meek may inherit the earth, they rarely get a DX for MG!!

Good luck,
Sue

I felt I needed to contribute to this thread just a little - While I was having my EMG tests done a couple of weeks ago, the technician was constantly remarking (mostly to the student observing the testing, but to me as well) about how responsive my nerves were and how that's a significant problem in general - they don't have any honest individual baselines. He was saying that in 20 years when and if my neuro response levels drop to "merely normal" they'd say that everything is fine, except that *my* normal response is much higher than the statistical average. So I could be feeling significant neuromuscular deficiency but if I'm within "normal" response would not be diagnosed with any loss. That could very well be what is happening to Sonja... her "normal" could be quite beyond statistical normal and so she's feeling the effects but cannot convince her doctor of what the problem is.

Best wishes for you and hope you get well treated...

Brian

OH BRIAN!! This makes SOOOOOO much sense!!! I think that I have mentioned that Sonja played competitive tennis (I think I have this right -- language barrier and all). This might have made a difference?!?!

I mean athletes DO have lower resting heart rates, right?? So, it would make sense that other physical aspects could be different.

I don't think anyone knows the answer to that.

but, almost every human trait tends to have a bell shaped curve of normal distribution, and there is almost always some overlap between the low normal to the abnormal.

for instance some people have a Hgb of 13, some of 15, and some even 11.5, and they are all normal, but the 11.5 would be more then 2 standard deviations from the average, so it would be considered abnormal, and unless you have known that person's Hgb from birth you would think they have anemia. (which technically they do, but it has no clinical significance). on the other hand a Hgb of 13. can be a sign of GI bleeding, if that person always has 15.

a person is a very complex biological system, and that is why you can't just look at one test, and base all you decissions on that.

alice

Hi all seronegatives!
I just found a reference from the Oxford group which has developed a more sensitive testfor antibodies. This test allowed to find antibodies in 80 % of the seronegatives tested...
http://www.ncbi.nlm.nih.gov/pubmed/1...ubmed_RVDocSum
Maurice.

hi Maurice
 

How are you?

Thanks for that, Ive just had a look at it!

Rach

Hi Rachel,
Not so well…I'm still fighting against that pneumonia shot which sent me back about two years ago: arm, jaw, neck muscle weakness reappeared, somewhat alleviated by an upped intake of Mestinon, 8 x 60 mg per, one every two hours.
I really feel when I need it, that's how I come up with that scheme. This means that my production of antibodies has been drastically increased, about double that it was 1.5 month ago.
I'm so sorry for you that you haven't received any answer from France (yet…). If you want to send them a reminder and if I could help, you have my e-mail…
And from the bristish side, what is the outlook?
Take care,
Maurice.

Hi Maurice
 

So sorry to hear that you aren't doing too well. I hope that things start to turn around for you soon.

I heard from France but they suggested I see someone in the UK. I have persued their suggestion as its someone the professor has worked with and I have an appointment in late December. So Im crossing my fingers for that one.

Thank you so much for all your help

Love
Rach

Hi Rach,

I will be crossing my fingers also for a good outcome!!!!
Hope you are doing ok
Kate

Rach, This might just be the one this time. At least I'll be keeping good thoughts that it is. You have been through so much and have been so brave. Hang in there just a little longer...you will get the help you need.;)


Maurice, I'm sorry to hear that the pneumonia shot caused you to have so much trouble. I hope you get back to feeling better soon. When I was in the hospital they offered to give me both the regular flu shot and the pneumonia shot, which I declined. Had I felt better I would have accepted the pneumonia shot, but perhaps it's just as well I didn't. I had a violent reaction to the regular flu shot (my first one) about 18 years or so ago, so I haven't had another one. Now with having the MG and such sensitivities to so many meds, I'm even more leary about getting the regular or the N1H1. I'm just going to be very careful and hope for the best. Feel better;)


Hugs,
Pat

Dear Alice,
I know this is a really old post, but I'm desperate for help. You write:

"there is also another study that shows that in patients with seronegative MG it is not that rare to have a normal SFEMG"

Do you know where I can find this? I have been treated for (seronegative) MG the past year, but now, after my third negative SF-EMG, my dr has changed my diagnose from MG to unclear muscular weakness. He now believes it is functional, something I can physically train to become better. I have a feeling he is wrong.

I have been on Mestinon, Prednosolone, and immunosuppression (Prograf) the past year. It has helped me gain so much strength. This makes be believe it could be MG even if my dr doesn't think so. So I need to read and find out as much as I can about if it is possible to have seronegative MG AND negative SF-EMG. The SF-EMG was made when I was really, really weak, from not taking mestinon for 22 hours. And the dr who performed it is a specialist who has worked with those tests for many years.

I am so thankful for any response!
Kind regards from Europe

Hi, Olle. Welcome to the forum!

Alice hasn't been her for a while. But I believe I can help.

When someone is immunosuppressed, the signs of MG will be reduced or disappear. There are studies, one done by the inventor of the SFEMG (Erik Stalberg), that clearly show that the SFEMG becomes normal while on Mestinon and immunosuppressants.

SFEMG.Info

The studies I found are in book form. I can't find the articles online. You could try to search PubMed. Or you could contact Erik Stalberg and ask him! He might reply!

Any doctor should understand this basic principle. The whole point of suppressing the immune system is for the disease state to be suppressed! Therefore, the signs of that disease (clinical and tests) are equally effected.

I'm sorry for your struggles and that they are now acting as though you're fine. When someone has cancer, has chemo and/or surgery, and the signs of cancer go away, do doctors then say that the patient never had cancer?!!! If someone has pneumonia and take an antibiotic and there are no signs in 2 weeks, does that mean they weren't sick? If someone has seasonal allergies and takes Singulair or Benadryl and the symptoms go away, does that mean they don't have an allergic response any longer?

This is a DANGEROUS way to think. If they take you off of meds and put you on physical therapy, for example, you could go into a MG crisis.

Did you have positive antibodies? How did they diagnose you? And if you didn't have MG, how do they explain suppressing your immune system for months (longer?) and subjecting you to the possibility of cancer and subjecting themselves to the possibility of a lawsuit! :cool: They are being rather hypocritical.

Are you symptom-free? If so, your doctors should consider you drug-induced remission. Please, don't worry! Sometimes you just need to talk their language in order for them to stop being, well, just plain ignorant.

:grouphug:
Annie

Dear Annie,
6 months later, thank you so much for your reply. Im very happy to tell you that I got my diagnosis back by my new neurologist. I saw the top MG specialist in our country and she told me right away that I had been extremely mistreated at my previous neuro and that I do have MG... So, Im back on my medicines, happier than ever waking up to take the pills in the morning. Im just so extremely glad to simply be alive, dont know how I would have survived if I hadnt got the right help.
Just wanted to share the good news. Changing doctors, doing resrearch and being persistant has saved my life and I encourage everyone in my situation to do the same.
Wish you all the best! :)

Oh I'm so happy for you. I have given the same advice to my sisters. A lot of neuros are not as smart as they think.
Mike