muscle biopsy
 

I have been testing for MG and they were not sure if that or a muscle disease. I had a postive EMG, then negative EMG, all negative antibodies. I had a positive SFEMG.

I had the muscle biopsy right after Christmas and it was abnormal. Provisional 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.

I received more on the path report... I think the final.
"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."

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.

Hi rumpled

sounds like you're going through a stressful time.
How many mestinon are you taking?
I hope you get some results somehow.
Let us know how it all goes, and take care!!
redtail

I am taking 3 a day of 60mg... but already it is ripping up my stomach. :( I don't see a change which is like last time - just stomach and head problems but I am going to stick it out and see if something different happens.

Yep I to had bad tummy trouble, got up and vomited most mornings, until I started taking my mestinon with food, even though advised not to, I also started taking probanthine, and now very rarely have problems. Its so hard mg being such a rare disease, Im the only one in my area, and not having people close by to talk to makes it hard, which is why these forums are so great!!
Hope thing start improving soon!!
redtail

I am taking mine with food - the label on mine says to take it with food or a "small snack" if there is stomach upset. Now I am getting eye and er, bum twitches... and choking even more. So I am already thinking this is not working out so great but this is only day 2. I did not have so much hope since it did not work last time.

Hi Rumpled

just thought I'd check in and see how you are doing???
Hows the mestinon going, I have been having muscle twitches lately, my right eye and in my upper arms, all a bit wierd.

I hope all is going ok, and if the mestinon is not working your drs can find something that will.
take care
redtail

I am getting lower left leg twitch and I woke up this morning, even before mestinon, with my left eye, below the eye, twitching like crazy. Argued with my husband since it is not working and all this twitching and the other effects do not feel good but he want me to keep taking it until I go to accupuncture. So I took another pill but not happy about it.
My eye is still going and it is driving me nuts. The mestinon makes me choke more, not less.
Not really wanting the ivig either... just not really sure. Prednisone is not an option with me due to my cushing's history. I just don't feel well at all.

Sounds like you are having a terrible time at the moment!!
Its so hard coping with just MG and all its side effects tablets etc, it must be worse still to have other things to cope with as well.
I know how scary it is choking, and if the mestinons not working it really must be awful!
So when do you see your doctor again? Is he/she good helpful? I hope so.
Have they said anything about any other medication? Im now cellcept, as the imuran was not working to its full potential.
I hope things dont get you too down, and feel free to talk as much as you want, Im good at listening!!
take care
redtail

He said we were going to talk tomorrow. I already sent him an email telling him all my troubles with the mestinon. I am just exhausted with it. Feel like I have run a long time.
He was very helpful but now he is leaving his post and seems to be more in a hurry now. He said the next step would be IVIG. Ugh. I cannot do steroids.

I just read your posts about the Mestinon. Sometimes you have to start at a lower dose and build up to a higher dose as your body tolerates it. Maybe you could ask your doctor if you can lower the dose.There are times when I only take 30mg (half a tablet) and others when I take 120mg. Sometimes I need it every 2-3 hours and sometimes I only take it 3 times a day. If you stay on it and it works for you, you will learn to tell what you need. It is good that you are trying to talk with the doctor about it all.

Do you have a Muscular Dystrophy Clinic anywhere in your state? If you do, it might be a good idea to contact them. Myasthenia Gravis falls under there services. That way someone who knows a little more about Myasthenia Gravis could coordinate your treatment and you wouldn't have to reinvent the wheel every time a new doctor cycles through your town.

Here's a link to the MDA website and you can search for clinics in your area.
http://www.mdausa.org/clinics/

I hope things are getting better.
Gabrielle

I had tried it - building up - the first time I tried it - and there was no effect.
This time, this doctor told me just to take it 60 60 60 and see how it went. It was miserable as my symptoms has progressed this time. I was not having problems swallowing last time and now I am - so it made it all worse with the increased saliva. The test was would it make it better.
What I find very strange is that since I stopped the mestinon is that I got a twitch under my eye. It happened Sunday and lasted 5 hours. It happened again Wednesday and lasted 6 hours. Then it happened Friday and well, so far into Saturday, it has not stopped. 30 hours so far. Irritating and aching my face. On the mestion I got eyelid twitches and leg ones. Crazy.

Ok, so I went for the IVIG. After two doses, they said my white count plummeted "like a really old person" and they would hold off and see what would happen before they gave me more. By the end of that day, I was almost dead. I had a 103.5 fever, an adrenal crisis and aspetic meningitis. I kept telling them how to treat the adrenal crisis but they kept lowballing me on the hydrocortisone which is a very very dangerous thing for me with a fever that high and no freaking adrenal glands. I asked for 100mg, they gave me 5mg kind of thing (not joking!). Finally 5 hours later I got 80mg and after a really bad night, I pulled through with a lot of pain meds and anti-emetics.
They told me no more IVIG... gee...
So now I have to find yet another option. They did a lot of testing while I was in though so my swallowing difficulty is now documented and I got a lot of occupational therapy. I escaped the walker as it seems my weakness seems more upper than lower but my legs still are not great.

Well, I guess my option now is to... do nothing. So, since any other medication would have too many side effects in the doc's opinion - I am stuck living like this. I may progress to get worse, or simply resolve and magically get better (wooee!) but I have to say, I am not thrilled at life like this. Back in limbo-land with arms of crud, choking and ... but oh well, they do not have to live my life.
He explained that it was just like before, when they did not have treatments for MG. I feel like such a throwback. Is anyone else out there completely untreated?

Is it possible they ran the infusion too fast? What type of I.V.I.g did they use?

Hi rumpled,
sounds like things are pretty bad at the moment!!
Im sure you've said it before, but humor me(I've got the flu and my MG is playing up!!which usually means my brain goes on holiday!!!!!) why can't you take immune supressants?
It must be very frustrating being in this position, wish I had something good to tell you, but keep comming here and talking, getting it all of your chest helps(well I know it helps me),
take care
redtail

It was Carimune 6%. They run IVIG all the time (two other people were getting it the same time as me, inpatient) so they ran it nice and slow and tested me. The day I had the reaction, I actually did not even have any - my tests had showed that my WBC had dropped already so they were giving me a day off but my body did not recover, instead tanked later in the day. It was my lack of adrenal glands, they think, that caused the reaction to be more severe than usual.

I cannot take steroids due to my history of cushing's - it will just give me cushing's back right away and I have to be able to take various doses of steroids to control my life (literally) and so to take high doses would take that away. Plus my body is already severely damaged by the steroids my body made and they do not want to add to that. The other drugs, they were worried about the side effects being more serious than the benefits.

Checking In
 

Hi-I found your post online and even though it's from 2007, I was wondering how the discovery process is going. Have you been properly diagnoses yet?

Based on the few things you mentioned it doesn't exactly sound like you have Myasthenia Gravis (MG). I have MG and though we have very similar symptoms the fact that the Mestinon was making you sick may be an indicator that your body does not need what it is supplementing. I currently take 180mg of Mestinon 4 x's per day and don't have any side effects.

When I read your biopsy results it sounds more like you have a Mitochondrial disease. Do a goodle search on it and see if it sounds like what you've been experiencing...

I hope you've found what you need by now, but your post touched me so I wanted to see how you're doing.

Take Care,
m