Not open for further replies.


Jul 24, 2017
Loved one DX
Dear PALS, I've been reading so many threads and posts since June 2017 and today I decided to post for the first time. First of all, thank you for all those informative posts and everyone that takes there time to answer.
I will try to keep the post short, but it it's very complicated so please bare with me! Basicly we went from possible ALS to Myasthenia Gravis to possible ALS.

In January 2016 my 62 year old mother noticed she was out of breath quickly. After a flight in April it was so severe, that she was rushed to the ER as they thought she had a lung embolism. They did not find anything and suggested it might be stress.
Other symptoms were loss of strength of her upper arms. Lifting things is difficult, and combing her hair is also difficult. But she also had double vision, especially after watching tv or reading. There has been no weight loss and she gets worse during the day. First EMG only showed Carpal Tunnel Syndrome on both sides and 10.9% decrement. She was sent to the first neurologist, who tested her for Myasthenia gravis, but she did not have antibodies. His final conclusion was that she probably had early stage ALS, as EMG showed denervation in cervical region. The hospital was horrible and that's why we flew from Europe to the US to the Mayo Clinic where she was checked out from top to bottom. All reflexes were normal, but the quadriceps reflex were increased. Which was a LMN sign according to the neurologist. No atrophy. No visible fasciculations or fibrillations. Final EMG conclusion of Mayo was ''EMG findings consistent with early stage of ALS, because of chronic active neurogenic changes in cranial nerve-innervated muscles. Normal needle EMG of lumbosacral and thoracic segments. According to the EMG report the EMG of right bulbar and cervical segments muscled showed fibrillation potentials and long duration, complex and varying motor unit potentials with reduced recruitment in most muscles tested. The EMG was abnormal and showed evidence of chronic active disorder of lower motor neurons or less likely their axons affecting the right bulbar and cervical segments with involvement of both diaphragms. There was also 28% decrement of the accessory nerve. The thing that makes it very complicated is that the neck MRI also showed severe multivel neural foraminal narrowing, moderate on c2-c3, advanced on c3-c4 left, advanced on c4-c5 bilateral, and moderate on c5-c6 and c6-c7. The neurologist said however he could not exclude a possibility of superimposed Myasthenia Gravis and that the overal findings did not fulfil the revised EL Escorial criteria. And also that he cannot be sure whether part of the EMG changes in the right arm could be from cervical spine diseases or not. He also could not explain diplopia and said maybe ALS AND MG, but that MG would not explain neurogenic changes. She has no MG antibodies. Next to that she als has Osteoarthritis and carpal tunnel syndrom on both sides. Vital Capacity was 60%. Like all of you, we were shocked and broken by the news.

She started Riluzole in August and Lunasin shakes and the Mayo neurologist asked us to try Mestinon 3 times a day too. I joined every ALS forum I could find and started looking for trials. At this point my mother was wearing a neckbrace, mostly at the end of the day. After she tried the mestinon, things got a bit better. We went to the ALS Clinic in Europe, where they did not believe she had ALS. Lung VC improved by 9% after Mestinon, and the neck was much better, so she stopped wearing the brace. The Neurologist changed the diagnosis to Myasthenia Gravis, as double vision fully went away after the mestinon, and the neck felt much better. He also said that Respiraroty onset ALS has the worst prognosis, and the fact that she has had the breathing problems for almost 2 years already clinically doesn't match respiratory onset ALS. Also because of the shortness of breath but normal EMG of the thoracic segment. According to him the quadriceps reflex are slightly increased, but not abnormal, and a lot of people have slightly increased reflexes. He concluded that she had double negative myasthenia gravis (no antibodies) so the diagnosis was changed as he was 95% sure it was not ALS. New EMG showed decrement of the accessory nerve was now 20%. In order to fully improve, she was started on 60mg of prednison, gradually building it up for 8 weeks. She started tapering the prednison and is now on 20mg a day. Unfortunately it seems the prednison has only made everything worse. The neck is painful and weak, and she started the brace again. Both sides of the arms upper arms (proximal arms) are affected with the loss of strength. The Carpal Tunnel syndrome has worsened, and gives her a lot of pain, probably because of fluid retention with prednison. No weight loss, finger function intact, legs are fully functional. VC of lungs was 60% and no nightmares of tiredness during the day. Capillary bloodgas is normal, normal carbon dioxide. But both the neck and strength of upper arms has gotten worse. The breathing is stable, but it seems to vary per hour or day. Some days it's pretty good, other days she has a lot of dyspnea. She also cannot lay flat on her back in bed. Last week we had a check up and he said that this point it is not clear if the MG is being treated correctly, or if it's Cervical Radiculopathy, Carpal Tunnel syndrome or maybe ALS after all that is affecting her this way. And that we have to continue tapering the prednison which is until march this year to see. He was hoping for more result with the prednison but everyone reacts differently and it may be that she needs other medicaton. But he is not excluding ALS, as he really doen't know at this point.

Both proximal arms are almost equally affected I would say. There are no pathological or asymmetrical reflexes , arm reflexes are low to not available. Also no visibile fasciculations.

Do any of you have experience with cervical narrowing AND ALS? OR recognize yourself in part of the long and boring story? Do you fluctuate too with good days and days? I know with ALS that once the strength is gone it's really gone but did you fluctuate before that? It is so complicated and going from ALS to ALS to Myasthenia Gravis and then again possible ALS has been a hell of a stressful ride. There are things that point to Myasthenia but there are a lot of things that point to ALS too and also things that not point to ALS. I know pain etc is not common, and the diplopia definitely isn't, but my conclusion after reading so many threads here is that there seem to be a lot of exceptions in ALS. The combination of different diseases isn't exactly helping either lol. It's very frustrating that even the neurologists at this point don't really know and that all we can have is patience. I did read many many posts of forummembers, including the ones from shatzie I think, who's diagnosis was changed to MG. Any help, tips, experience would be greatly appreciated!
Last edited:
This is a complicated case, and unfortunately if the neurologists who saw your mom can’t be certain of the diagnosis, then neither can we.

She does have cervical sine osteoarthritis and likely does have cervical radiculopathy which could explain the weakness in her arms. It sounds like she has had approximately 3 years of symptoms (onset January ‘15), so if it were ALS, one would expect to see more progression typical of ALS. Since she started out with respiratory symptoms, the normal EMG findings in the thoracic segment argue against ALS, as one of your neurologists noted.

So I would have to say it sounds like she has cervical radiculopathy and probably doesn’t have ALS, but I’m not a neurologist. As far as the double vision —- there’s probably some other explanation for that. ALS doesn’t generally afftect eye muscles until very late in the game at which point the diagnosis would be obvious.

If others here recognize this constellation of symptoms, let them weigh in. Best of luck to you and your mom.
Hi Karen, thank you very much for taking your time to answer and your well wishes.

I agree about the complexity, my new hobby in the past couple of months has been reading articles on pubmed and ALS forums. :)
I realize I made a typo. She started with respiratory problems in January 2016 and not 2015 So it would be 2 years now, which according to what I read would also be long for respiratory onset ALS? Unless she has a slow progression? Mayo Clinic is of course highly respected and therefore it makes me doubt even more. The rollercoaster of emotions from hearing the diagnosis and then getting it changed and then getting it changed again is horrible, but at the seem time it gives hope I guess.

If anyone with respiratory onset here (which is unfortunately not common) has had a slow progression, your input would be greatly appreciated!
Hi Pals, a few different questions. Any help would be greatly appreciated please!

In my mothers case, the neurologist that says probably early stage ALS (in july 2017)!considered it respiratory onset ALS. The weakness of the Arms in my mothers case is proximal and symmetrical , no problems with finger function like buttoning shirts or writing (just pain due to carpal tunnel syndrome)z
I read in one article ( that flail arm syndrome does not involve respiratory muscles and the weakness is proximal and symmetrical . And in upper limb onset ALS the weakness is distal and asymmetrical.

Are there any Pals here that have a type of ALS that started with respiratory problems and then progressed to bilateral proximal arm weakness? And can anyone tell me if they have variety per day in muscle strength and shortness of breath? I know once the muscle is atrophied it’s gone and you lose the function, but I am wondering if before or during that process the strength varies per day maybe? Do you feel better after resting or is it always the same? Many thanks in advance
The flail arm subtype and respiratory onset are not compatible. By definition, the flail arm subtype is proximal arm onset, and may or may not entail respiratory symptoms later on.

The neuro is incorrect if s/he implied that respiratory onset has the shortest survival; especially with BiPAP, it has been reported as comparable with bulbar, or better.

At any rate, I don't think the subtype is as important as whether your mom has ALS, of any type, and staying ahead of her needs regardless. She should be on a BiPAP when asleep whether the dyspnea is intermittent or not, since it appears to be worse when supine. But if it is intermittent as you describe, that argues strongly against ALS.

Hi Laura, thanks for your answer.
I have also read 1 article on pubmed which stated that respiratory onset has a comparable survival rate as other forms. I guess it’s difficult to do proper research with such small cohorts of patients. Regarding the dyspnea, there are good days and bad days, but stable. It gets worse with stress and anxiousness , for example when meeting a new doctor etc. When going to bed she will feel a bit short of breath the first 10 min but she can lie on her side after that and breath normally. During the night when she wakes up to go to the toilet , she doesn’t have that problem and sleeps back almost immediately. Stress and anxiousness also seems to trigger her voice to sound a bit nasal I think. But after calming down it gets back to normal. I know it’s common for MG, but does this happen to ALS patients too? And any food triggers? Hot milk seems to make her very short of breath so we stopped that. Which probably points to MG. It is very stressful and I am driving myself crazy overthinking and reading articles. I will discuss bipap again, thank you
Hi Pals,
today my mother got another EMG, it was the first one since June 2016.

The conclusion says: Loss of peripheral nervous system motor axons to muscles in (bulbair)Cervical/Thoracal regions, the findings in the (bulbair) cervical region match the El Escorial criteria. The decremention could match a MND/Neurogenic disease. (repetitive nerve stimulation of right m. trapezius: CMAP decremention ->10%

This is almost the same conclusion as back in June I think, where the final conclusion was also that only the cervical region matches the el Escorial criteria.
Like I wrote above, she does have narrowing on various cervical levels and was/is being treated as a MG patient with mestinon and prednisone.

We haven't discussed the results with the neurologist yet, but I am interpreting this as no change since June as the cervical region back then was also the only region that matched the el Escorial criteria.

What do you guys think? Could this be maybe a slow progression or still something else? Is my conclusion correct? The only thing that has changed since then is a bit less strength of the proximal arms, but the weakness is probably due to steroid myopathy (prednisone). How fast did you progress?
Last edited:
The situation is complicated enough that you really need to hear from the neurologist.
Hi guys,
just a quick update for the those interested.
So I finally spoke to the Neurologist after almost 2 weeks.
The EMG showed no progression or change since July 2017.
The only part of the body that matched the el escorial criteria was the cervical area. T
The other parts did not have any abnormality. There was fibrillation in only 1 lumbar muscle, but according to the neurologist this was due to my mother not being able to fully relax. And it would be odd to have it in just 1 muscle.

The neurologist thinks, based on this and the clinical picture, that it's not ALS but he cannot be certain.
It could be that the myasthenia gravis is not responding to the prednisone and therefore we would need to try different medication.
But maybe it could be slow ALS as well, though that seems very odd according to the neurologist as respiratory onset usually has a bad prognosis as it tends to move fast. And also because she is responding to Mestinon.

Any thoughts? Anyone with a very slow progression of ALS ?
Meanwhile the mayo neurologist who diagnosed my mother says he still thinks it's ALS, though he admits finds it weird that the EMG did not change, as he would expect changes after 4-6 month. There where a few bulbar changes in July, and they are still there on the EMG. Unchanged. He says that has to do with ALS, the neurologist here says those changes are from the Myasthenia.
I believe that an ALS diagnosis requires consistent abnormalities in at least 3 spinal segments, either by physical exam, EMG, or both. Evidence of progression, as well as both upper and lower motor neuron findings are also key in making the diagnosis. However, most patients don’t read the textbooks.

So in your mom’s case, unfortunately nothing is textbook. It’s probably going to be a “time will tell” situation. How much time, nobody knows. But the longer she goes without progression in a direction consistent with ALS, the less likely ALS becomes.

You said Mestinon helps. Is she still on that? I think it’s important to continue what seems to be helping and keep monitoring the situation with an open mind. Best of luck.
I agree with continuing to treat as MG, if it helps, but also would continue to advocate for BiPAP if her breathing is significantly compromised, even intermittently. Any doc can write it so the disagreement should not matter. And MG is a reimbursable diagnosis for it, as is ALS, of course.
Not open for further replies.