I am going to give your smart noggins something to think about. I have seen multiple Neuro's and 3 out of 5 have confirmed dx of MG--Seronegative with 2 positive EMG's, 1 negative (1/2013)--Today Johns Hopkins Neuro stated, he does not think I have it, but doesn't know what I have.
I started 2009 with eye twitching, loss of peripheral vision, and needed glasses to help. (astigmatism on eye dr exam) then 3 months later, I had severe neck weakness and strain to causes headaches, and a COMPLEX Migraine where speech was effected. CT was fine...then 2 months later, shortness of breath, chest pressure when exercise or steps, Cardiologist sent me to get a ct of my chest and my Thymus was enlarged to 6cm...so they removed it.
All this and EMG gave me the MG dx. After this, I stayed on Mestinon and Imuran. I went in for a knee replacement, and the day after surgery, I stopped swallowing and was intubated. I have excessive saliva production while having MG symptoms/crisis. Then 4 months later, after small exercise again, experienced facial drooping, severe body twitching, loss of bowels, vomiting, and become paralyzed for days, and get IVIG and steroids in the ICU. I can't move my muscles to get my hands legs or body off the bed for 4 days, gradually coming back. When home, very weak for 2-3 weeks, still having double vision, muscle twitching, speech problems. After another surgical procedure, the same symptoms, and was intubated due to respiratory muscles being weak, under 1 liter of volume. This time, after intubation, I had to be on a NG tube b/c not being able to swallow for 4 days afterward. There was now 5 crisis', 3 intubations--the neuro doc states "crisis' are usual, but I don't believe that she has MG."
What do you believe could be the culprit? I need to get life back...I have symptoms of shaking muscles daily...

wow, you have been through the ringer!!! So sorry to hear about all of that!!! I don't have a firm diagnosis, but take IVIG every 2-3 weeks, hopefully someone else can give you more insight... I do however have the muscle twitching/ shaking thing... I feel like I have a cell phone vibrating at times, it's really strange. Also after surgeries i have had difficulties and almost had to get intubated with my c section epidural.. couldnt' move my tongue or face or anything after it for a long time..

Have they checked for some of the periodic paralysis? (Hypokalemic/hyperkalmeic periodic paralysis)? Not sure if they can get so severe that it would result in intubation... Lambert eaton can also be affected by anesthesia.. and my last thought would be charcot marie tooth - it is an anesthesia affected disorder - but it may be that it can have a relationship with hyperthermia, not sure if it causes paralysis.. just a few thoughts..

Good luck! keep us updated!

fasciculations are uncontrolled muscle twitching in muscles I have used that day--I can move the arms if the twitching starts, but the muscles won't stop twitching until I rest them for a while. The paralysis is not chemical based, more inability to move with NO MUSCLE STRENGTH. The ability slowly comes, and with a great deal of strength needed that 5 days ago, I didn't have to have. I have 3 children, husband, and a home to care for so weakness to not get out of bed is not allowed. The muscles slowly get stronger, and I do loose some muscle, and small PT helps. The ANESTHESTIC in my last procedure, followed MG guidelines for no paralytics, wasn't intubated, just a device to keep the airway open in my throat. ONE NEURO stated that my immune system is noticing the incision and there is a problem when it has to go into action...I also have had a immune IgA count to show an Autoimmune disease exists...because of this marker, now another piece to the puzzle.

Hey there...I get the cell phone thing too. In my stomach, back, feet, all over.

What exactly doesn't fit?

To me you sound like MuSK MG. Were you tested for that?

The response to mestinon is unpredictable in MuSK.

Some patients respond to it like AchR MG.

Others have increased weakness+fasciculations.

Others have both, but in a very hectic manner. The same dose at the same time of the day can lead to significant improvement, severe worsening or no response.
You can have fasiculations with improved muscle strength or with severe weakness.

The reason for that is (probably) that MuSK is a very complex protein. The antibodies cause: presynaptic abnormalities (less release of achetyl-choline like LEMS), post-synaptic abnormalities and also to complicate things even more- decreased activity of the acetyl-choline esterase (which is a mestinon like effect).

The balance between those effects can change, with activity and probably other factors. and thus the response to mestinon will change accordingly.
(those are my explanations).

Severe worsening with steroids may also fit. ( MuSK MG tend to have more myopathic response to steroids).

Were you ever treated with PLEX? IVIG is less effective in MuSK.

Also, did anyone suggest that you receive non-invasive respiratory support after having so many crises.

Karen, Have you had the MuSK antibody test, as Alice suggested?

The one thing that doesn't fit MG is loss of peripheral vision. Did they give a reason as to why that is happening? Was it a temporary thing or is it still there?

Ptosis, especially long-standing ptosis, can CAUSE astigmatism. That's what caused mine. I've had MG since birth or shortly thereafter.

You don't have fatigable weakness so badly that you have to be intubated from nothing going on!

What do you mean by "immune IgA count" and that it revealed something. Are you talking about the serum IgA blood test? Was yours normal. I'm not certain what you're saying - can you clarify that? Did they run the IgE, IgM, IgG too?

"Your immune system is noticing the incision." Wow. Really? That's not very scientific. So your immune system, "sensing" that there had been surgery, is causing your muscles to weaken to the point of paralysis and intubation. Do they think patients really buy that BS?

MG, either ACh, MuSK or any other of the unidentified antibody MG's, is quite simple. 1. Do an activity 2. Muscles get weaker to varying degrees. 3. Rest (and drugs) make muscles stronger (relatively).

No other disease is quite like MG, except LEMS (Lambert-Eaton Myasthenic Syndrome) and CMS (Congenital Myasthenic Syndromes, which are genetic and not autoimmune). They all involve variable muscle strength either worsened or improved with activity. LEMS can initially improve with activity but then get dramatically worse. Some CMS are like MG in that you don't have enough ACh (acetylcholine). Other CMS's are where there is too much.

Did the start of your symptoms in 2009 coincide with pregnancy at all? Just curious.

Did they do a pathology of your thymus? The fact that it's growing back so soon is interesting.

Even if you follow the protocols for MG during surgery, the fact is that the drugs given during any surgery have the potential to make it worse. You can take all of the precautions in the world and still get worse. Were you given morphine or any other pain killers? Morphine can tank an MGers breathing.

Sometimes with Mestinon, it's not only the space in between taking it but the dose amount. Do you have a good neuro to talk all of this over with? Some people do better on a smaller dose but with more frequency. It all depends! Don't make any changes without talking to your prescribing neurologist first!! You are obviously not doing well and any changes could make you worse, like Abby mentioned about cholinergic crisis being an issue too.

Myasthenic crisis: Not enough acetylcholine getting to the muscles.
Cholinergic crisis: Too much acetylcholine getting to the muscles due to too much Mestinon.

Keep talking! For that Johns Hopkins doctor to so arrogantly say this isn't MG is nonsense. And, BTW, an EMG or SFEMG in MG can be positive and then negative and then positive . . . Oy. A good MG expert knows that!

Take it easy. And even though you're a Mom, there are ways to reduce any impact on your MG. Or "incision disease."


Annie

Did you totally lose peripheral vision or your peripheral vision is just not clear. I know that if I do not look directly head on at something that my vision is blurry or double due to weak eye muscles, which of course progresses as the day goes on.

I just took my first mestinon pill and the first improvement I noticed is how much better I can see and focus.


kathie

Annie, THE EYE SYMPTOMS --PERIPRIAL VISION WHILE DRIVING WAS GREY, AND COULD NOT SEE, BOTH RIGHT OR LEFT, YES STARTED WHEN I WAS 6 MONTHS PREGNANT WITH 3RD CHILD. THE OB STATED IT WAS HORMONES AND not to get glasses, because it would get better...but my vision was blurry. That has come back now. Also with this pregnancy, I was having severe uterine contractions like preterm labor, but no cervix dilation, was that part of myasthenia? I didn't tell the dr's about the contractions...
In my answer to Alice MD, there was a increased IgG and decrease in IgA. what does that confirm?.. an Autoimmune disease?
The pathology to the Thymus was not thymoma, thymic hyperplasia and the hospital stated myasthenic based thymic tissue
In regards to the SFEMG, and EMG's being positive and negative, at different times makes me feel a little better. I love medicine and know that tests can vary very much...I also took some of the EMG print outs, and from what I have researched, the decreases in Amp and Area % are shown just like an Abnormal MG EMG results.?.?.?
the Neuro's at Hopkins aren't MG specialist

THANK YOU ALICE MD-FIRST OF ALL, I HAVE BEEN HOPING TO HEAR FROM YOU WITH SEEING THE GREAT UNDERSTANDING YOU HAVE IN OTHER THREADS IN THIS FORUM:
OK so MuSk testing has been done, and was neg. The IFE tests show increased IgG and decreased IgA; normal CMP, copper, SPEP, thyroid panel, ANA, and Mayo panel.
I have been offered PLEX 4 times, but IVIG was running. I have very bad vascular access and need a port placement, but with my hx of respiratory and throat failure, I can't risk another surgery.
I have been weaned down off Mestinon to 1/2 tablet 3 times a day, and I could hardly move without assistance, and was in bed for days. It was hard to see, breathe, eat, and walk.
I also wanted to add that I have not been able to regulate my temperature when it is an indoor warm environment. I will sweat profusely. I think it is from my muscles working harder, like running a marathon, but not able to cool down. Afterwards, I have to go sleep, so I can stop my muscles shaking so badly, to function. Sleep will help the symptoms tremendously.
I have not needed respiratory help at home non-invasively, but they are stopping my IVIG now, so I might be needing something sooner.
I am on a steroid taper now, and the meds give me energy, which helps me to function. I really hope this added information will help with figuring out what I need to do or say to have a clear DX and be able to stop symptoms everyday, from ruining my life, and families lives.

Go to another neuro. This is outrageous. You need a neuromuscular specialist. I'd also get tested for Lyme disease and all the coinfections as fast as you can.