I'm going to try to upload pics to demonstrate the damage done to the neuromuscular junction for those who have not seen it: I hope this works, lol.....the 1st shows the difference in normal & affected junctions; then the cross section of small affected nmj in MG; lastly, the normal nmj......


Dottie.

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Dottie, Thanks for the info.

I don't think MG is as "easy" to understand as some people say it is! So many things can affect MG, make it worse (or better), etc.

Dr. Angela Vincent works at Oxford University in England. She has a great article on MG and the AChR antibodies (below in PDF form). They are mainly IgG subclass 1 and subclass 3. There are four total subclasses of IgG. If you have a deficiency of one of these subclasses - like I do - your antibodies may not show up when the tests are done. Just like in Celiac Disease if you have an IgA deficiency, antibodies may not show up in the blood tests.

Also, if you are on another drug like Advair before you go through diagnostic testing for MG, it may make the antibodies disappear or SFEMG appear negative.

I think equating disease process with either the antibodies or the SFEMG is deceptive. There are articles that say that even in those who supposedly have only ocular MG, they can look at the neuromuscular junction in other muscle groups and "see" damage. It may not be enough for frank weakness but there's damage just the same.

I don't know how anyone can say that an autoimmune process is not or cannot be progressive. We can slow it down or stop it with drugs. We can lessen it with treating infections, reducing stress, etc. (like Dottie was talking about). I kind of see antibodies like locusts, eating as much of our NMJ as they possibly can!

Bottom line is to do whatever we can to calm the disease down and make our immune systems happy. We may never have a definitive answer to this question.

Annie

Here's the explanation for the thumbnail.

Figure 2
Effector mechanisms of anti-AChR Abs. (A) Ab binding to the AChR activates the complement cascade, resulting in the formation of membrane attack complex (MAC) and localized destruction of the postsynaptic NMJ membrane. This ultimately leads to a simplified, altered morphology of the postsynaptic membrane of the NMJ of MG patients, which lacks the normal deep folds and has a relatively flat surface. (B) Abs cross-link AChR molecules on the NMJ postsynaptic membrane, causing endocytosis of the cross-linked AChR molecules and their degradation (antigenic modulation). This ultimately leads to a reduced number of AChR molecules on the postsynaptic membrane. (C) Ab binding the ACh-binding sites of the AChR causes functional block of the AChR by interfering with binding of ACh released at the NMJ. This results in failure of neuromuscular transmission.

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vincent1.pdf (21.2 KB, 523 views)

Dottie - Those are great pictures! Thanks!!

Annie - How did you find out that you were deficient in one of the subclasses? Does anyone ever get a muscle biopsy to show mg?

It's great to have you two sharing your knowledge. And Angela Vincent rocks!!

Thanks Annie, the "antibody" is a good one! & the excerpt by Dr vincent also!....keep'm coming!!

Dottie

Annie, one more question? How does Advair affect the outcome of the antibody tests and the SFEMG?

Thanks!!!

Angela Vincent does indeed rock! She's so smart - and nice. Back when they were the only ones doing the Musk, she ran mine.

Bluesky, I'll make a long story short. An immunologist back in 2002 ran the IgG subclasses, and other Ig's, because I had infections a lot. Another not-so-lovely doctor in 2002 ran the AChr Antibodies . . . and kept the results from me. I didn't get those results until late last year: They were positive. They had been almost positive two years earlier. But my antibodies since then have been negative or just below positive. It wasn't until I got that result and did some digging that I put those two things together. I have Celiac Disease and knew that IgA could affect those antibody tests. So I looked into what "Ig" the MG tests were.

Advair has Flovent. Flovent is a steroid. Flovent goes systemic in the body. They now have lots of warnings about Flovent suppressing the immune system. And for people who are sensitive to drugs or have more exaggerated responses to them (like people with immune problems), it can suppress the immune system enough to make antibodies disappear. And it can make whatever autoimmune disease you have "better" and disappear, like during a SFEMG.

I have another one by Dr. Vincent but it's too large. Just Google "MG IgG subclass" and you should find it.

It's amazing how much there is to know about MG and what can affect it.

Annie

Yes, I had read earlier about your doc not passing on the positive results to you. I was disgusted, but not surprised. So many neurologists don't realize the gravity of those positive results.

I'm hoping that the internet will usher in a new era where the docs can't just get by on what they think they learned 20 years ago. Their patients (like you and I) will be coming in more and more with better and better information. Right now, it just makes them mad. But they're going to have to get used to it!

Thank you for the answers! I've learned so much from you.

I found this on the MDA website. It has a very easy to understand explanation with pictures .

http://www.mda.org/publications/fa-mg-qa.html

Becky, That's a good, easy to read article. Unlike my more complicated ones!

Bluesky, I'm afraid it wasn't that the neuro didn't realize the importance of the antibody test; it's that he DID realize the importance of the test. He realized that the neuro friend of his that I saw two years earlier who did not do sufficient testing for MG and said I was "fine" would be open to a medical malpractice suit if I got that test result. It was perhaps the worst thing I've ever witnessed a medical "professional" do.

Annie, I had a similar thing happen to me at the Mayo. I had asked for a week to be tested for mg. The morning of my last day there, the doctor who had been managing my case acted very strangely. He had a hard time looking me in the face, he became very cold and couldn't get rid of me fast enough. He told me that I needed to see the neuro that afternoon before I left which really surprised me because the neuro had totally blown me off earlier. I asked the managing doc if any of the tests had come back abnormal and he said, "no not at all"." He was so odd (and he would stare horrified at my drooping eye - the first time it showed to him - and then quickly look away) that I knew something was up. The neuro told me about the positive antibody tests at the end of the meeting in a very round about way, completely minimizing it. In fact, I feel like it slipped out - "oh, your managing doctor didn't mention that?" Call me completely paranoid, but I don't think I would have been told at all if Mayo didn't have a policy of sending out all the records to the patients. I think the way it played out colored their entire view of what was wrong with me. The neuro kept staring, horrified, at my eye too.

I wanted to ask you what you knew about low IgA. I've been having my son tested for a lot of things because he has many symptoms similar to mine (antibodies for mg came back neg though). Since he had a low ferritin level and restless legs I asked the doctor to test for celiac. I just got the results today and it came back negative but with a low total IgA, which I understand (thanks to google) means a much bigger possibility of false negatives on the celiac test. But could the low IgA mean anything else? Could it give me any clue as to why he is so sick (horrible, zombie like fatigue, difficulty breathing (low PEF), double vision)?

Thank you!!!!