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Jul 24, 2017
Loved one DX
Dear PALS, I've had an earlier thread regarding my mother, who's diagnosis is still uncertain. Both Laurie and Karen were very helpful. A short intro:

My mother is a very complex case and has shortness of breath, orthopnea, loss of strength in upper arms and some atrophy of the deltoid muscles. The symptoms with breathing started in April 2016 and have progressed since. EMG'S ( 2, about 6-7 months apart) showed only the cervical region match the El Escorial criteria. Both ALS, MG or MG and Cervical radiculopathy (without sensory symptoms or pain) are the different options the neurologists are considering.

At the moment she is being treated for MG, (since August 2017) but they think she could have both ALS AND MG.

A few weeks ago, while tapering the prednisone, 24 hours after the lowered dosage, she felt the dyspnea getting worse and the strength in upper arms became even worse.

She was admitted into the hospital 17 days ago for severe shortness of breath and raised PCO2 levels in her arterial blood gas. She was barely able to complete sentences and ended up staying 2 weeks.
The doctors concluded that it could be either the progression of the ALS, or a myasthenic crisis. Because it was a bit sudden after the lowering of prednisone it was considered a myasthenic crisis ( Myasthenic crisis is a complication of myasthenia gravis characterized by worsening of muscle weakness, resulting in respiratory failure that requires intubation and mechanical ventilation.)

She was treated with Immunoglobulin (IVIG) for 5 days, and she has been placed on NIV (Philips trilogy) for during the night, as they noticed she had carbon dioxide retention.

The breathing is going better now, she is not short of breath anymore, but it's not like she can run a marathon and walking up and down stairs still makes her a bit tired. Before IVIG she could not finish 1 sentence and was short of breath almost non stop, even after a toilet break. Her forced vital capacity varied the whole time, from 15% when she was very short of breath, to 22% to 33% and 27%. Not great but definitely improved I would say. It's just weird how it fluctuates. Her cough strength increased from 90 to 270 after 13 days. They also started with Air stacking.

But the strength of the upper arms has not been restored. In a few weeks a new EMG will be made. I wonder maybe it this could be due to the atrophy. Loss of muscle means of course loss of strength.

I am wondering if there are any PALS that had IVIG and if so, if it helped them with anything? I would expect it not to help in case of ALS as I always understood ''you only go downhill' aka it's not reversible. Also, do you fluctuate in strength? And shortness of breath?

Laurie btw, thank you for recommending the BiPap, she is sleeping much better now and we are happy she has it. They decided to start her on BiPAp just in case it is ALS and progression.
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I only did one round of IVIG because I started throwing clots that wound up with me having pulmonary embolisms. The reason I was approved for IVIG was to see if I had multifocal motor neuropathy, which responds to IVIG. It is not prescribed for ALS. It is usually the last thing to try before being diagnosed with ALS. If you respond to IVIG this is multifocal motor neuropathy. You are however looking at having infusions for the rest of your life at $12 000 per month. With ALS symptoms do not come and go, nor do they improve, ever. Once the muscle weakens it never gets stronger. Sorry to be such a downer, but that's ALS.
Thank you for your reply Vincent, sorry to hear IVIG didn’t work for you. You were very unlucky with the side effects! I mostly hear people complain about headaches and fever.

I have often wondered if multifocal motor neuropathy could be an option. I re-read the EMG results from Mayo clinic from July 2017 and saw that phrenic nerve conduction studies showed absent response on the right.

I am wondering if this could be the same as conduction block what you have in multifocal motor neuropathy?

I would love to believe it could be that, but the weakness of the upperarms is proximal and the atrophy of the deltoids is symmetrical. From what I read mmn is usually distal?

The cost is ridiculous! We are lucky in Europe as insurance pays everything and the government pays for the BiPap machine and supplies.
IVIg response certainly points to something besides ALS, with MMN one contender. Phrenic nerve/respiratory involvement is rare but possible in MMN, as is primarily proximal involvement. Was she tested for anti-GM1 antibodies if her EMG was equivocal?

As you say, respiratory insufficiency is a hallmark of a myasthenic crisis, and her rapid/dramatic response to IVIg is one factor that makes MG seem more likely than MMN. I would make sure her non-MG drugs are carefully evaluated as many drugs can contribute to a myasthenic crisis.

Unless/until she returns to a new or the same baseline, guessing about concomitant ALS seems less actionable than trying to continue to improve her condition, which ultimately will yield that answer, since, as Vincent points out, ALS does not backtrack.

Laura you are right, thank you. We have another EMG scheduled for the 5th of April, but I am trying to think along what else it could be, as the EMG will be dirty life before. In the meantime MG medications are continued. If she doesn't take Mestinon in the morning she cannot even wash her face or comb her hair. After Mestinon it goes better, but definitely not a 100%. Seeing as the deltoids have atrophy, I think it's logical that this won't happen in a short while, even though the IVIg definitely helped with the breathing.
I know from research that untreated MG on the long run can cause atrophy. In this case symmetrical.

Regarding improvement, you seem to know a lot about NIV. Can/do ALS patients improve after starting NIV during the night? I understand from you, Vincent and the rest that ALS does not backtrack, but I am wondering what the effect of NIV is when ALS patients with respiratory failure start NIV during the night. Would they feel less shortness of breath during the day and have a better forced vital capacity or would you probably just see les carbon dioxide retention ? I am trying to figure out if it's the IVIg that helped or maybe if it's the NIV, though she started improving before the NIV already I think.

Sorry for all the questions, I don't want to be biased or too optimistic. It's been a long journey. I know there was a forum member here (schatzie?) that had her diagnosis changed from ALS to MG, but I am wondering if there are any PALS here with both MG and ALS.
Sorry btw, I know it's Laurie but it was autocorrected !
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NIV at night can definitely improve daytime breathing, as the PALS uses less energy for and gets a better result from breathing at night. However, what you describe is more dramatic than I would expect NIV at night alone to achieve, which points back to Mestinon and/or IVIg having an effect, as seems fairly clear from the timing you describe. So I would think the NIV is more additive in your mom's case, but definitely worth continuing.

I don't recall anyone here who reported both MG and ALS, but a review of 27 reported cases across multiple publications was recently summarized here.
Laurie thank you for the reference to publications concerning MG and ALS. Our son was diagnosed and treated with MG at the very young age of 9. Following treatment and including a thymectomy he has been in remission since the age of 11. He is now 38. 18 months ago his dad (our PALS) was diagnosed with ALS. One of the first things we questioned was the family connection between the 2 diseases. We were told that there was no connection between the two diseases at all. Suffice it to say that we heard what was said however I continue to still question the "coincidence" of being somehow related. Your info is helpful.
Laurie thanks so much for the link, I have read this article before indeed and concluded that it is possible but quite rare. I guess you can always be so unlucky.

At this point everything seems possible sometimes!
My mother started with shortness of breath in April 2016 when they thought she had a lung embolism after a transatlantic flight, continued with a 'stiff' neck slow loss of upper arm strength, yet we are still looking for the diagnosis after visiting 3 renowned Academic Hospitals, including Mayo. I guess the longer it takes, the better the prognosis maybe as it is so slow. Last year August when she started treatment for Myasthenia, the neurologist here said he was 95% sure it wasn't ALS. Now that it has progressed, he is not sure and again ALS is a possibility. Every EMG is nerve wrecking and makes me wonder, is this the day that they will diagnose ALS?

Of course it is all very complicated, and for example, the use of Prednisone in the dosage that she has been getting (60 mg a day) for the Myasthenia could also cause atrophy of the deltoid muscles, but that could also be caused by the stenosis. Her knee reflexes are symmetrical but a bit brisk. Mayo said it was an UMN sign, here they say no it's normal and some people just have that. I think it's weird that everything is symmetrical and the atrophy and weakness is also symmetrical. Strength testing is also very symmetrical.
Also, there are no fasciculations visibile

The NIV like you said is great, but the improvement with breathing starting before they even started with the NIV in the Pulmonology department, so I think you're right and it is indeed coming from the IVIg.

Sorry for the long post, I just had to ventilate a little.

@initforever, I am sorry to hear that your husband was diagnosed with ALS. I don't remember the article, but there are theories that untreated MG can eventually lead to ALS. I will try to look it up.
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Nothing would surprise me about this disease. My brother was dx with Lupus at age 35. He is now 81 and has severe RA. He can't go out in the sun because of the Lupus. He wasn't taking any drugs except 2 Advil a day, then he developed high blood pressure (not real high.) His GP took him off the Advil and put him on blood pressure meds (a beta blocker.) He now suffers from severe pain but never complains. He shuffles around. He refuses to go to my pain management doctor or a rheumatologist here in town. He goes back to his tiny village in upstate NY in early May. There are no doctors in town and the nearest hospital is an hour away.

My mother and father both had RA. My niece has Type 1 diabetes since age 2. Another uncle had MS. I have Hashimoto's. I don't know if there is some connection between autoimmune diseases or ALS or not. ALS isn't an autoimmune disease but there is so much nobody knows about it, I don't think it's a stretch to think they could be connected.
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