abnormal emg

[rumpled]

I read some threads but I cound not find my answers... I hope someone can help me. I asked my neuro to run an EMG as my arms and legs are really weak. I get double vision at night.. but that is kind of an old symptom. However, I had Cushing's so my body was producing its own cortisone (you may take it as prednisone) in abnormally high levels so that may have masked this for a long time. Last year, I had my adrenals removed so no more cortisone and consequently, my weakness has become so acute. My shoulders have become frozen. Physical therapy is not helping much and the pain is terrible.
Ironically, I directed a friend of mine here as she is being diagnosed with MG and has the eye issues and leg issues much more severely and she has positive bloodwork. She needs an EMG to confrm her diagnosis. My EMG is postive but my SED and other things were negative. Weird. I "met" my friend as we had similar endocrine symptoms and ended up with the same two pit tumors, which is mighty rare. Now to have this the same... well...
So, can I have a positive EMG and negative bloodwork and still have MG? I have not been able to work for over a year. What other tests should I get? I am going for an anti-actelcholine blocking antibodies test today and a chest MRI later. I take steriods to live as I have no adrenals... so not really sure about pred - as if i have an adrenal crisis, I would not be able to react well. Plus it may give me Cushing's again. Which is why my muscles are so cruddy to begin with. Is anyone else an endocrine nightmare?

[gecko.hale]

Hi, Jennnifer

I haven't been around BT for over a year now and it has sure changed! What happened to everyone? It used to be hot! Really sorry no one has responded to your posting; all the old regulars must have left.

I don't know anything about adrenals & pit tumors. Nor Prednisone, other than I won't take it unless I am at death's door. I have Hashimoto's, IC and MG for my autoimmune slate... so far.

What kind of EMG did you have, on which muscle, and what was the result? Did you have RNS (repetetive nerve stimulation) or SFEMG (Single Fiber EMG)? I think that if you had a positive RNS on a muscle, then they probably want another confirming test or clinical diagnosis (really weak, double vision, sounds like you pass the clinical exam!).

If you have a positive (abnormal) SF-EMG on an affected muscle, then that is the gold standard. It is not "specific", meaning it could be some other neuromuscular disease -- but with symptoms that are indicative of MG and an SFEMG, that pretty well isolates it to MG. A muscle biopsy would rule out the other possibilities, but with clinical symptoms should not be necessary.

If it wasn't an SFEMG, then a blood test would be helpful. Trust me, I am seronegative and MuSK negative and having either of those antibodies just makes your life much easier, diagnostic wise.

But your neuro should have the full range of AChR antibody tests done - binding (most common), blocking, and modulating. The other two pick up something like another 5% and 2% if I remember correctly. Not big, but if you are looking for a diagnosis, they can help.

If you have been looking for a diagnosis for a long time, I would also push for the MuSk antibody test. If you don't ahve AChR antibodies, then there is a good chance you will have MuSK antibodies. There aren't that many of us unfortunate souls who don't have any of the so-far-discovered antibodies.

WIth a positive EMG plus a positive antibody blood test (any of the above), you should have a positive diagnosis. With a positive SFEMG, you should have a positive diagnosis.

A chest CT is usually recommended to see if there are any abnormalities with your thymus, but the MRI probably gives them pretty close to the same thing (sometimes MRIs are not necessarily better). If it is an obvious thymoma, they will be able to tell, but from what I have read on the medical sites, both CTs and MRIs are not all that accurate on judging the other thymus conditions, such as hyperplasia.

Used to be that neurologists were competent and savy enough to diagnose clinically, but now they all want those labs to back themselves up. Whether they don't have confidence in themselves (haven't seen any of that) or they just don't have the knowledge and training (have seen a LOT of that), have seen almost none who can do a real clinical exam for FATIGABLE weakness as opposed to INITIAL weakness and do a real diagnosis of MG clnically.

Anyway, hope that is some help. I don't know how this all plays in with your other problems, though. :(

Good luck, Sherron

[rumpled]

Thanks for replying! What is IC?
I am not sure about the emg... I had the one with the taped electrodes and then they stuck needles in me. They did my right arm which is my weakest and my finger. The needle was stuck all over my arm (owie). So I think it was an RNS and the single fiber but not sure. It all happened so fast.
My neuro just called me last night and said all my blood work was normal but he still is going to call me MG. I think they are all AChr. They guy that did the test "wrestled" with me a bit to see how strong I was and called me pathetic. Mostly they just have you squeeze the fingers and I am like, duh, I can do that but in 3 minutes I am dead so that is not a good test.
The MRI showed that I had thymus tissue that was pretty good size but he did not order contrast (*sigh*) so no idea if that was a good idea or not. In any case, I had hyperplasia on my pituitary and I know it did not show up on films... and it wreaked havoc with my body.
I don't really have double vision per se - I just have weak eye muscles. I have had to put prisms in my glasses in my eyes for years and the problem - hello! - was really there when I was 6 years old... But when I get tired, I do see double. But since last December, I have progressively lost the use of my arms which is irritating. My right shoulder froze without me injuring it. The left one is becomine immobile. But it does not seem to fit what I have read of MG in others. I cannot stand for long periods or if I do push it - I am literally in bed for days after, in pain and/or sleeping.
I don't know if I really have MG - but something is going on. I think I have to go see another doc but not sure how to find one and man, it is so hard to drive.
Yeah, your post made me laugh! It is soooo true.

[Smedy]

Hi!

I only have a minute so this will be short.... I have heard of patients with MG who had negative labs.... many neuros do not want to diagnose based on EMG only though. I think there are 3 types of MG labs... try to get all of them.... many people have tested positive to MUSK and negative to the antibodies.

[rumpled]

He does not want to do the MUSK for some reason. I would like all of them just for peace of mind. He is giving me a trial of mestinon to see if it has an effect and sending me off to some motor neuron specialists in New York City. My eyes seem ok but my arms and legs are the worst.
I am also seeing another local doc tomorrow.

[lilviking]

I have been away for a while, but I thought I would add a little... Unfortunately, there are no foolproof test for MG. The most accurate is the SFEMG around the eye. This is said to be about 95%. As you have probably found out by know, the answer to your original question is "yes". A positive EMG can be enough to diagnose MG even if other labs are negative... if there is a positive reaction to the mestinon. However, most neuros will not want to make a firm diagnosis unless at least two tests/labs are positive, medical history is indicative of MG, and other possibilities have been eliminated.

[rumpled]

I wanted to give an update. I had another EMG by the NY specialist which they said was normal. I just had an SFEMG of the arm (my eyes are normal) and it was abnormal showing mild to moderate MG. He did run another set of tests including the MUSK tests, however, I do not have the results. He is sending me for a muscle biopsy next. He is waiting for all results to be in before a diagnosis is final but it may be mild to moderate MG or a primary muscle disease.

[rumpled]

I had the muscle biopsy and it was abnormal. Provisisional results are "skeletal muscle with rare atrophic myofibers".
Under the findings they said:
"Rare atrophic myofibers found among normal sized fibers"
"Most of the atrophic fibers are type 2. There are no significant fiber type grouping or fiber type dominance"
"Four myofibers shoe increased staining of succinate dehydogenase. At least two of the fibers suggests hyper contraction, a common artifact"
"The epimysial adipose tissue shows mild reactive changes including early focal fibrosis and a few histiocytes or macrophages"

Any ideas? Thanks.

[Raechel]

Jennifer, You have certainly been through a lot. It sounds like they may be able to figure out what is going on with your muscles now.

You got your results but a doctor didn't tell you what they meant yet? That's not very nice! I can't tell you what they mean. I can "guess" that it might be some kind of mitochondrial disorder but I don't have a clue what the results mean. Here is a good site on muscle biopsies:

http://www.neuro.wustl.edu/NEUROMUSCULAR/lab/mbiopsy.htm

I hope that they can now give you treatment that you need to feel better.

Raechel

[rumpled]

Ah the doc said he won't tell me until ALL the results are in for all tests and that path report is just provisional. More stains are coming. Thank you for the link.

[rumpled]

"Final Diagnosis: Skeletal tissue with rare atrophic myofibers. Reactive Chages in epimysial tissue, focal, chronic.
Comments: The muscle biopsy specimen shows three COX-deficient fibers. This abnormality raises the possibility of a mitochondrial disorder although the COX-deficient fibers are rare and may not be significant clinically. In addition, the epimysial fat show focal reactive changes. The cause of this abnormality is not clear based on the pathological examination, but may be related to a monor injury to the area in the past."
So it kinda does look mitochondrial. I always get weird stuff. First Cushing's, now this. I don't recall injuring my thigh ever. I have to wait until late Feb. to get an answer but I guess it is probably not MG.

[Raechel]

Jennifer,

A mitochondrial disease would make a lot of sense in your case. They often affect multiple systems within the body. I think what you need is a mitochondrial disease expert. I have no idea if your docs are experts but please do not leave the analyzing up to doctors who don't thoroughly understand this area.

Have you ever had a cardiac evaluation? I'm asking because it is often affected in mito diseases.

Here are a few articles for you. I know this is overwhelming but it sounds like you are finally get the answers you need.

While you may not have MG, it has been found that some people with MG end up having damage to their mitochondria. There are lots of cross over elements to diseases. I guess what I'm saying is that if you need support here, then you should keep posting. Not a lot of people post in the mito forum.

I hope you can get a good treatment plan and feel better.

Raechel

http://www.neuro.wustl.edu/NEUROMUSCULAR/mitosyn.html#biopsy

http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=1377234

http://www3.interscience.wiley.com/cgi-bin/abstract/109709346/ABSTRACT?CRETRY=1&SRETRY=0

http://www.eurekah.com/chapter/1726

[rumpled]

Thanks! I saved the links to look at...
I have been seeing a cardiologist for a couple of years even though I am in my 40's. I see her again for my regular check up and I am going to bring this to her attention. I seem to have something funky going on which is why I have been going but nothing with me as ever been straight (medically that is). Perhaps this is the reason.
I am seeing an excellent neuro but I am not sure what he knows about mito... but he is the head of neuro for a large hospital and has been ever so helpful so far which is more than I can say for most of my docs. He did say that I may have more than one thing...

[rumpled]

The neuro said that even though I am sero-negative, and only my ANA was slightly elevated, he thinks that since I flunked the SFEMG and today did not pass an eye test he did, that he will call me with 70-80% certainty, myasthenia gravis. He is not so sure due to the lack of antibodies (which 20% of MGers are like that so it is not unheard of) and just that my presentation has not been typical.
I am starting another trial course of mestinon. I tried it before and it did not work (hence why I saw him) but I am going to take it a week solid and call him. If it works, great, I continue and MG is certain. If it does not work, I will be hospitalized for 4-5 days for and IVIG treatment (immunoglobulin). If that works, I will need that monthly (it can be done at home by a nurse) and it supports the MG diagnosis. If that does not work, then I have to re-evaluated. My muscle biopsy results were consistent with MG.

[rumpled]

I went to bed last night with a lower led that was twitching quite badly. I woke up this morning, really late, with a lower eye that has been twitching for at least an hour. I had twitching off and on as well as the other side effects (gastric -fun) but I am not liking this. As I did not see any benefit and my eye is driving me nuts - the medication should have worn off during the night? So, have decided to quit the mestinon.
Anyone else have a lot of twitches?

[rumpled]

The doctor agrees that the mestinon did not work. I am going in for the IVIG treatment. For some reason, they like to hospitalize you for the first treatment so I will be in for 4-5 days. My lower eye continues to twitch for hours at a time even though I am off the mestinon. At first it was slight twitches to my lids and stuff - but then it got to be big ones to my calves and the pouch under my eye - anyone else get that?

[rumpled]

Well, I did the IVIG. Sort of. After two of the expected 5 doses, I developed aseptic meningitis, a rare side effect, and due to my adrenal issues, well, I got a nice 103.5 fever instead of the usual 101 and with the inexperience of the staff to endocrine issues (and their lack of listening to me!) I came a lot closer to death than I really wanted to... so they have said that I can no longer ever have IVIG.
I had to stay in the hospital as I was sick from all that... and I still have the darn twitch.

[rumpled]

Still mostly talking to myself here... the neuro said that since most of the mild treatments did not work, that I am just to wait without treatment and see what happens. Any of the other treatments would have so many side effects and the risks would outweigh the benefits now. I am still choking on food all the time, all the weakness is there, but I just have to live with it unless or until it gets worse or resolves all on its own. Is this normal?

[Raechel]

Jennifer, You might want to start a new post - maybe more people might respond. It's a very slow site.

I'm not a doctor but I still believe you may have a mito disorder and not MG. This MDA article is really good and talks about the Cox deficiency.

http://www.mda.org/Publications/mitochondrial_myopathies.html

Have you considered getting a 2nd opinion from an expert who deals with MM/CM?

Cell metabolism is diminished with mito disorders. I'm wondering if that's why you had such a bad reaction to IVIG. Have you had reactions to other drugs in the past? Here's an article on that:

http://www.clevelandclinic.org/health/health-info/docs/1600/1678.asp?index=6957

I just hope you can find answers and get some real help.

Raechel

[rumpled]

They told me the mito abnormality they found was less than 3% and found in a lot of patients with MG and it was considered normal. I guess even if it is mito, then doing nothing is the right treatment. Yes, I often have terrible reactions or no reactions. Thanks for the articles!
I am going to get another opinion as my doctor is leaving, so I have no choice but to find another doctor.
I am very discouraged and on top of all the other things I have... I just don't really want to keep running around to doctors.