Speech and Communication Issues

[BethU]

Beth,
How did the 3rd neuro diagnosis ALS? Did he do another EMG that showed lower motor neuron damage? How long have you had your speech symptoms that would make the 2nd neuro say it cannot be ALS because you've had it for too long?

Hi, Sral. The 3rd neuro was a consultant at my PCP's clinic, leading a group of medical residents around to look at patients with unusual neurological problems. He did no tests, and I don't know if he even reviewed the blood tests, MRI, or EMG that had already been done. (He must have.)

He had one of the residents take my history and do the usual bonking here and there with the little hammer. Neuro #3 reviewed my history & the exam with his group outside the exam room, then came in and said, "First of all, I want you to know you don't have anything progressive, and I can tell you right now that your symptoms will probably get better." Well, I almost proposed marriage to him on the spot! (I'm 72, so it was a little late to promise him my first-born child.)

I think what put the cherry on top of his diagnosed was the fact that I had just gotten out of the hospital after 3 weeks treatment for respiratory failure, with entubation and all that fun stuff. I think he figured that was a "Myasthenia Crisis" ... which if I had MG it would have been. I also had double vision and the "myasthenia snarl" (your face stiffens up, and you can't smile ... it comes out a grimace). He diagnosed me in January, 2008. I had had the speech & vision problems since March, 2006, and swallowing problems since January, 2007.

Another thing he told me was that my tongue fasciculations were not the kind you get with ALS: they didn't roll like waves. (I have since been told that that's irrelevant. ALS patients can twitch any way they want to.)

He prescribed Mestinon (without checking my other meds, which he had a list of ... he didn't realize that Mestinon was a no-no with beta blockers and I had a really bad time with low b/p, dizziness, unable to walk, etc. until people on an MG forum told me I shouldn't be taking both). But when I started the Mestinon in January, it improved my speech remarkably ... speeded it up and made it much, much crisper ... for 2 days. Also gave me a HUGE burst of physical strength and energy.

The speech improvement went away, but Mestinon continued to give me remarkable energy and physical strength as long as I took it. (I quit in May when I got the diagnosed of ALS, as I thought I should go back on my heart meds.)

One thing that has bothered me about the ALS diagnosed. I told Neuro #5 and #6 (at a free MG clinic at UC Irvine) that Mestinon had continued to improve my strength and energy, and Neuro #5 said, "that doesn't mean anything. It improves EVERYBODY'S strength and energy." Neuro #6 just shrugged.

I wondered, if it improves *everybody's* strength and energy, why the heck aren't they giving it to all their ALS patients? But I didn't say anything. I'm going to try Mestinon again after I see my cardiologist and get a substitute for my beta blockers. I have read somewhere that a small percentage of ALS patients do get some benefit from Mestinon.

What it does is make the signals from the brain linger longer at the junction with the muscles, thus giving them a little extra time to batter their way through.

Sorry this is so long. Can't seem to write short.
Hope your mom is hanging in there. She is so lucky to have your support.
BethU

 

[sral]

Dear Beth,

First of all I'm glad to hear that your progression is so slow - who knows they may be wrong and you may only have PLS!

Secondly, please never apologize for long posts as they are always so full of insight.

Thirdly, thank you for describing the MG snarl. Although my mom has absolutely no problems laughing or smiling. I find that many times her face seems like she is grimacing. I ask her what is wrong and she says nothing. It's just that when she's trying to make certain expressions they turn into a stiffened snarl look. I have this underlying feeling that my mom has something other than her diagnosis and MG has also stood out for me. The neuro said she did not have it and would not even do the blood work. I had her GP do some thyroid tests and she said everything came back in normal range. However, she has difficulty with stairs - going up. She does not have atrophy. She does not have fasics. She can walk slowly. She has pain in her leg. When she wakes up her eye is almost shut closed and takes abit to open up. It just does not add up for me. She can still speak but it's slow and at times slurred. There was a period when it was nasally too but that comes and goes. (It's been almost 3 years since symptom onset Jan 06)

Fourthly, all the best to you Beth and thank you again for your response!

 

[BethU]

Dear Beth,
I have this underlying feeling that my mom has something other than her diagnosis and MG has also stood out for me. The neuro said she did not have it and would not even do the blood work. I had her GP do some thyroid tests and she said everything came back in normal range. When she wakes up her eye is almost shut closed and takes abit to open up. It just does not add up for me. She can still speak but it's slow and at times slurred. There was a period when it was nasally too but that comes and goes. (It's been almost 3 years since symptom onset Jan 06)

Boy, it sure sounds like it might be MG. That should absolutely be ruled out! (There is another condition that mimics MG, too ... it's called LEMS. Lambert Eaton Myasthenia Syndrome. It is *sometimes* a precursor of cancer ... sometimes the presence of even a few cancer cells anywhere in the body throws the immune system into overdrive to fight them, and MG-like symptoms are the result. However, LEMS is REALLY rare and very hard to diagnosed and treat. But ... it is treatable, and if it's connected to cancer, you find that very early.)

I would sure hope that your mom gets tested for MG antibodies. Can't believe your mom's neuro didn't try to rule out anything and everything else it might be! I thought that was Rule #1 in ALS diagnosis.

But that kind of happened with me, too. When I went to the MG clinic, they said they needed to test again for MG antibodies, but after the EMG, they said, "That's it. You've got ALS," and refused to do more blood tests for MG. (One of the three neuros I saw at the clinic said he DIDN't think it was ALS, but did think there was "something else going on." He was the big cheese in the clinic ... the clinical professor in charge and a member of the board of the Calif. MG foundation ... but I never saw him again. The ALS diagnosed was made by an associate prof. and a "fellow" of the college.)

There are two antibody tests for MG ... the ACHr, which detects the most common antibody, and the MuSK test, which tests for a second MG antibody. This second antibody has only recently been discovered ... in the last couple of years. The MuSK was difficult to get done. I had to have blood drawn 3 times before they figured out they needed to send the blood to a specialty clinic to get the results. Took about a month and a half.

I was negative on both tests (although, it was interesting to see the actual numerical results, rather than just a postcard saying, "negative" or "positive." I was at the top number of the scale for normal in the ACHr. One more point on the scale, and it would have been a diagnosed for MG. Since all my baseline vitals are low ... normal temp is 95F, low b/p, hypoglycemia, hypothyroid ... I thought perhaps my baseline ACHr level might be lower than average, too, and the high number on the test might be an abnormal reading for me. Just as, when my temp is 98.6F it doesn't mean I'm suddenly "normal," it means I have a fever!

Also, they never identify ANY antibodies in about 7% of MG patients. Many MG patients are diagnosed by clinical symptoms alone.

Your mom's droopy eyelid is a red flag for MG, just as tongue fasics and atrophy are for ALS. Thank heavens your mom's progression of whatever-it-is is so slow. Is her breathing still OK? Respiratory failure is the big danger with MG ...

I asked my PCP to order the ACHr and MuSK tests, just for my own peace of mind, since the neuros wouldn't do it. I hope your doctors will keep investigating your mom's condition and rule out every other possibility! Considering that MG is treatable, it is outrageous that neuros would not do everything possible to test for it.
Good luck!
BethU

 

[sral]

Beth,

Thanks for your reply. I didn't even know there were MG blood tests. I thought MG was detected through a series of thyroid tests. My mom had these done (per my request) and they were all within normal range. However, I too read that some MG cases do not show abnormal results in blood work.

My mom breathing is totally fine. Her speech which was her first symptom almost 32 months ago is slow and sometimes slurred. I can understand her because I am use to her but others need to pay close attention. As for the droopy eyelid it's only in the morning and does not last long. I wonder if this is normal because she is tired in the morning.

As for you, fortunately your progression is really slow too. Hopefully it is something else.

Thanks for all your support

 

[Judith]

Hi, Sral. The 3rd neuro was a consultant at my PCP's clinic, leading a group of medical residents around to look at patients with unusual neurological problems. He did no tests, and I don't know if he even reviewed the blood tests, MRI, or EMG that had already been done. (He must have.)

He had one of the residents take my history and do the usual bonking here and there with the little hammer. Neuro #3 reviewed my history & the exam with his group outside the exam room, then came in and said, "First of all, I want you to know you don't have anything progressive, and I can tell you right now that your symptoms will probably get better." Well, I almost proposed marriage to him on the spot! (I'm 72, so it was a little late to promise him my first-born child.)

I think what put the cherry on top of his diagnosed was the fact that I had just gotten out of the hospital after 3 weeks treatment for respiratory failure, with entubation and all that fun stuff. I think he figured that was a "Myasthenia Crisis" ... which if I had MG it would have been. I also had double vision and the "myasthenia snarl" (your face stiffens up, and you can't smile ... it comes out a grimace). He diagnosed me in January, 2008. I had had the speech & vision problems since March, 2006, and swallowing problems since January, 2007.

Another thing he told me was that my tongue fasciculations were not the kind you get with ALS: they didn't roll like waves. (I have since been told that that's irrelevant. ALS patients can twitch any way they want to.)

He prescribed Mestinon (without checking my other meds, which he had a list of ... he didn't realize that Mestinon was a no-no with beta blockers and I had a really bad time with low b/p, dizziness, unable to walk, etc. until people on an MG forum told me I shouldn't be taking both). But when I started the Mestinon in January, it improved my speech remarkably ... speeded it up and made it much, much crisper ... for 2 days. Also gave me a HUGE burst of physical strength and energy.

The speech improvement went away, but Mestinon continued to give me remarkable energy and physical strength as long as I took it. (I quit in May when I got the diagnosed of ALS, as I thought I should go back on my heart meds.)

One thing that has bothered me about the ALS diagnosed. I told Neuro #5 and #6 (at a free MG clinic at UC Irvine) that Mestinon had continued to improve my strength and energy, and Neuro #5 said, "that doesn't mean anything. It improves EVERYBODY'S strength and energy." Neuro #6 just shrugged.

I wondered, if it improves *everybody's* strength and energy, why the heck aren't they giving it to all their ALS patients? But I didn't say anything. I'm going to try Mestinon again after I see my cardiologist and get a substitute for my beta blockers. I have read somewhere that a small percentage of ALS patients do get some benefit from Mestinon.

What it does is make the signals from the brain linger longer at the junction with the muscles, thus giving them a little extra time to batter their way through.

Sorry this is so long. Can't seem to write short.
Hope your mom is hanging in there. She is so lucky to have your support.
BethU

Hi BethU,
thought I'd jump in about your neuro #5's remark about Mestinon. My neuro prescribed that to me when I was being tested for MG. It didn't improve my strength and energy . It messed with my vision and also madee my stomach quizzy. I took it for 3 weeks then stopped.

judith

 

[sral]

Beth,
Based on Judith's experience with the MG drug and how well it worked for you, maybe there is more to the neuro's shrug when you told him. Is it possible you have been wrongly diagnosed?

 

[BethU]

Beth,
Based on Judith's experience with the MG drug and how well it worked for you, maybe there is more to the neuro's shrug when you told him. Is it possible you have been wrongly diagnosed?

I spent the last year and a half in complete denial that I could possibly have ALS ... it was the worst thing I could think of ... but the last 2 neuros were so convinced, they convinced me. I think the tongue atrophy is pretty conclusive, it's something I can see for myself, and atrophy doesn't happen in MG. I've also developed twitching and weakness in my left arm and weakness in both hands and I think there's some atrophy in my left hand.

MG patients do get limb weakness and great fatigue, but no atrophy. Also, the emotional lability points to ALS.

It seems logical to me that the muscles controlling the eyes could be weakened with ALS, resulting in blurred and double vision, even though neuros say it doesn't happen that way. But if I've got ALS, and also have double vision, then it obviously DOES happen that way (sometimes).

Once I get squared away with the cardiologist and different heart meds in September, I am going to resume Mestinon, and see what happens. If I again get that burst of energy and strength, then I'm going to have a serious talk with my current neuro and ask her to review my history more closely and NOT shrug off any symptoms. Possibly redo some of the tests. Al said something a while back on this forum that sometimes MG doesn't show up in tests till later in the disease process, which I didn't know.

My own hunch is that I either have some weird variant of ALS, or there may actually be two disease processes going on. Because of my age, it seems to me logical that two "systems" could fail at the same time. Right now I'm just so grateful that this is progressing slowly, and that I am able to function OK. If I pace myself, I'm actually living a pretty normal life, except for my wretched speech and some swallowing issues.

Thank you so much for your feedback and your concern!
BethU

 

[kelly]

Thanks so much for the insight. Although the intelligent side of me knows that my husband is not yelling at me...sometimes it is difficult not to internalize. My husband often speeks loud..in frustration..or just to project his thoughts. Your comments are much appreciated. Fondly, KR