This is my first post. I have been extremely weak and resting for three months, like falling off a cliff after a very active life (46 athlete seven children). I tested positive (0.48) for AChR binding antibodies, but negative in the single fiber EMG test. The neurologists are definitive that I do not have mysasthenia gravis, but I am at a loss to explain my profound weakness. In two weeks, I have an appointment with rheumatology/immunology. This week I am also afflicted with inexplicable systemic hives (which might be autoimmune because I woke up with them before eating or drinking anything).

If it's not myasthenia gravis, what is it?
Becca

Diagnosis: Algorithmatosis.

Hi, Becca. I am, of course, being silly. But there's nothing silly about doctors being stupid.

There is one thing it could be, which is a tumor of your thymus gland. Did they do a chest CT? I'm guessing they didn't. Some people who have a tumor removed have antibodies go away. NO, I'm not saying that you have a tumor!!! Some people with MG have a thymoma, however.

I'm saying that you probably have MG, and the idiot doctors are following an algorithm that doesn't hold water. Quite a few people with MG have a negative SFEMG. The test is fraught with problems.

MG is a clinical diagnosis backed up with tests. A negative test doesn't prove anything. A positive clinical diagnosis, which I assume that you had given your symptoms, and a positive AChR antibody test would mean that you DO have MG.

Might I ask, where do you live? We might be able to refer you to an MG expert who won't be so stupid.

I'm concerned about your weakness, because you said it's profound. If you are at a point where you can't move well, can't breathe, can't swallow, or are simply unable to function due to the weakness, it's time to dial 911. MG can be dangerous, and you can have a myasthenic crisis. Weakness can become much worse and rather quickly.

Do not get overheated, because it can tank MG very fast.

To ignore the probable diagnosis of MG is not only dangerous, but unethical. Heck, it's downright malpractice (but don't repeat that or doctors get all "testy" and take it out on you).

If you are short of breath, you can see a pulmonologist for breathing tests to see if you have fatigability of your breathing muscles.

You can see a neuro-ophthalmologist to assess if you have ptosis and if the cause is MG. Do you have droopy eyelids (ptosis)?

If you're strong enough, you can gather this evidence and/or seek out a second opinion from an MG expert. But don't do all of that in lieu of going to an ER if you need to!

In the meantime, don't overdo things. And realize that weakness can come on so slowly that you don't know how severe it is.

What are you doing for the hives? You should have gone to the ER immediately. You don't know how badly a reaction will get or how quickly. Did you take Benadryl? You need to get some help. Be aware that some of the antihistamine drugs can make MG relatively worse. You really should be at the hospital!!!

And don't assume that you could only have one thing going on. You might have more than one disease state, unfortunately.

I'm really sorry you're going through all of this. You are not getting the help that you need. A positive antibody test is HIGHLY indicative of MG. Period. Let us know what else we can do for you!

Annie

Thank you for answering me, Annie.
I live in Durham, NC and my SFEMG test was performed by Dr. Vern Juel and ordered by Dr. Justin Mhoon. Dr. Juel took a long time with the SFEMG and tested my arm and my eye. Dr. Mhoon says that I do not have a clinical diagnosis and that I definitely don't have MG.

I do not have a droopy eye.

I am able to breathe, and my swallowing difficulties make me choke several times a day, but I can still eat and speak fine. I say profound weakness, but maybe, compared to some, it's not that profound. I can still squeeze the doctor's hands and lift my knees with power. This morning I made my toddlers breakfast, and now my arms feel like lead and I'm stuck in bed trying to type. I am still able to go to church, but by the time the sermon starts I have trouble keeping my head upright on my neck. I have trouble keeping the brake pedal pushed down when I'm waiting at a stop light, so I'm avoiding driving. I guess I'd revise profound to marked weakness.

This past week I got some blood test results which show subclass IgG immunodeficiency in classes 2 and 4. I have had repeated infections (strep, UTI) in the last several weeks. I read that thymoma can cause this as well (Good's syndrome - not good).

When I get the infections, the antibiotics seems to crush my strength. I am allergic to amoxicillin and sulpha, and the only ones left are supposed to be very bad with MG, involving the neuromuscular junction. Unfortunately, there seem to be no options, and considering my immunity issues, there could be need for more.

I did have a chest CT though, and they noted nothing in the mediastinal space. That was before any AChR antibodies when they were trying to clear my lungs. I looked at the CT myself and think I see a thymoma (history major here!). I am asking them to review it.

My hives were treated with a six day pack of methylprednisolone. The hives broke through for the first few days, but now I am a couple of days out and no hives. Thank God! Also, my muscle weakness really improved while on the steroids. I suppose steroids make everyone feel better, but I can't help but think that they shut down my immune system for a bit which I feel like is poisoning me.

I feel lonely, and it's hard to find people who track with even what the neuromuscular junction is. Thank you for answering me!

I'm a little worn out right now, but I need to say a few things.

Some MGers don't do well on Zithromax or Biaxin. I do just fine on both. Biaxin is an amazing antibiotic for sinus or lung infections. It can leave a bit of a strange taste in the mouth, but totally tolerable. I usually use the liquid form of Biaxin because it absorbs better.

You might also check with an allergist for any undiagnosed allergies. I don't have sinus infections anymore due to taking certain foods out of my diet (and knowing that I don't have stomach acid and taking Betaine HCL with food). I also have a IgG sublclass 3 deficiency (variable). Or find a good immunologist, which might be equally as helpful. The hives could be from the immunodeficiency and IVIG might help you.

Did they do IgA or any of the other immunoglobulins? Have you been tested for celiac disease?

Did you know that deficiencies in the IgG subclasses can affect the antibody level in MG? Meaning that it can decrease it? It doesn't decrease the amount of antibodies in the body, only makes it LOOK as though it's less than it is on a blood test.

Do your "brilliant" doctors know that?!!

How do you know that you don't have ptosis? My ptosis was missed by me and everyone else for 41 years! You really have to see a neuro-ophthalmologist in order to know for sure. You might think your eyelids are normal or, rather, your normal. Unless you take a photo when you're rested and one when you're worse, how can you know for sure? It's the relative change that's important.

A clinical exam where you do a few resistance movements against a doctor's hands is not a true test of MG. It's only when an MGer is really bad that a clinical exam is telling. MG is all about weakness after repetitive (meaning more than a few movements) or sustained activity. Some neurologists still don't understand this simple fact about MG! As I've said before, they should put us in a sauna and then watch us try to walk up a flight of stairs, and then examine us!

Yeah, when you take Pred and your immune system is already "deficient," you will feel worse in general. That's a positive sign that it made your muscles feel stronger. Pred would probably not be a good drug for you, given your deficiencies, infection history, etc.

These guys at Duke tend to be super arrogant, especially with women. I'm surprised that you didn't try to go to UNC at Chapel Hill. Dr. Howard can be a bit hesitant with a diagnosis, too, but he has a LOT of experience and is an MG expert.

I'm truly disgusted with the lack of attention paid to detail here. And leaving you with "You don't have this, therefore you're fine" BS is simply bad doctoring.

Time to check in with your primary doctor and discuss all of this!


Annie

Annie,
Thank you for answering me. And thank you for suggesting Dr. Howard at UNC. I will look into that. Radiologist took another look at the CT and say it's not a thymoma. Waiting for an appointment with an immunologist.
It means so much to me that you are out here on the internet! I can see that you are tired and your life is demanding, but I just want to thank you for taking this time.
Becca

I waited for my immunologist appointment at Duke and found that I had accidentally been scheduled with a rheumatologist, who told me that I needed an immunologist. They were not sure when I would be able to get an appointment. They did find that I have elevated IgA in combination with the IgG subclass 2 and 4 deficiencies.

Anyway, in the face of more waiting, my husband put me in the car and took me to the Mayo Clinic in Minnesota, where I have been waiting every day in the waiting room for a cancellation. There is still a line of people ahead of me.

I have been unable to find research correlations between immune deficiencies and AChR antibodies, except in the case of Good Syndrome (which would be very bad).

I have also been unable to find any cases of people who had positive AChR binding antibodies but were negative on the EMG.

If anyone could direct me to any such studies, I would be grateful.

Becca

I find this so sad, Becca. Why the heck do patients have to search for studies on "positive antibodies/negative EMG" when that's a doctor's job?!!!

There are plenty of articles in PubMed about it.

Did they give you a reason for the elevated IgA? That most definitely needs further investigation! Did they do serum or urine protein electrophoreses?

I hope Mayo can help you. You do really need all of this investigated.

Be aware, if you aren't already, that we may have severe storms today and more flooding in MN. In the Rochester area, roads are often blocked for miles when that happens!


Annie

Annie,
Every time you ask me a question, I look it up and get an education. Yes, they did serum electrophoresis in April and found no monoclonals. Are monoclonals the same thing as paraneoplastic antibodies or onconeural antibodies?

I read an article which indicated that frequently such markers are detectable in the cerebral spinal fluid when they are not present in the serum.

I've been waiting just over a week for a walk-in appointment here at Mayo, and I'm in the front of the line now. It's a long wait in the chairs. I do hope that the doctors will help me.

Thank you for persisting in responding to my questions. I couldn't find any articles on pubmed about this, except paraneoplastic syndromes. And those never correlate with immunodeficiency issues, as far as I have found.
Becca