Quote:
Originally Posted by alice md
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.
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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.