Progressive Dysphagia - Is it bulbar MG?
 

I've had progressive swallowing issues for 7 months. For the last 4 months I've been on thin liquids.

I had a modified barium swallow month or so ago, but refused the solids. The liquid swallow apparently looked normal, even though I found it difficult.

Since then I've experienced increasing aching and fatigue of the tongue and swallowing muscles around the Pharynx (slightly more on one side than the other). Talking is hard because the tongue and throat gets fatigued, achey, and 'stiff'.

I've been examined by an ENT, and a neurologist who specialises in ALS/MND and also had a nerve conduction study and EMG done which were apparently normal.

I often feel if I did some of the tests again, particularly the modified barium swallow, I'd fail it.

Its got to a point where even liquidised soup is difficult - the swallowing is slow and weak and gets worse the more I try.

Each medic has looked at me and said "you don't have MG" when I've asked if it may be that. My eyelids droop a little, but continually and not any more when fatigued.

I'm at a loss because the medics think its in my head but the feelings and worsening is very real.

My questions are:
1. Does this sound like MG?
2. I understand most have problems with liquids first - it is solids initially for me - does it still sounds like MG?
3. How did you get diagnosed (particularly bulbar MG folk? What test convinced a neuro?
4. Is it worth trying to get Mestinon to try (don't know how though)?

Many thanks,
Flash

Ok, this is "my" area -- LOL!! Well, not funny -- but, I'm "Bulbar".

Can you still whistle? Smile -- does it look "normal" or sort of like a "snarl"? Look at yourself in the mirror WHEN you swallow. Can you SEE your throat REALLY making an effort to move when the food/drink is going down? Have you ever had liquids "float" back up into your nose after taking a drink -- sort of like water gets into your nose sometimes when you swim? Does your voice ever get "slurry" -- like a drunk or stroke victim? Do your lips get sort of "numb" feeling -- but actually, they just aren't moving very well -- and you can't "purse" them -- like you can't form a KISS or pucker? Use your tongue to feel the insides of your cheeks (I learned this from an oral surgeon). Can you feel ridges/lines -- especially close to your mouth? This indicates shortened muscle fibers.

Personally, I don't give a "hoot" if the doctor you are seeing is a neuro-muscular specialist or not. SOOOOOO sorry to say this. I think that something like 85% of MG symptoms start out as OCULAR -- and that is what some of the "neuro-gods" are looking for. I'm NOT saying that you HAVE MG -- I'm NOT a doc. BUT, if my paragraph "rings a bell", then, if I were you..............I'd get a SECOND OPINON -- SOON!! Cause Bulbar MGers are the MOST likely to go into respiratory distress -- i.e. the ones that end up in the hospital on a vent.

BTW, I was initially told that I had EITHER had a stroke OR had a brain tumor. I had done some research and was pretty sure that I had MG -- I think that they ran the blood tests just to shut me up. :D Actually, I LIKE the doc that diagnosed me -- I don't blame him. He was just doing what he had been taught. I like to think that we BOTH have learned from my "weirdness".

Oh, and I'm pretty sure my RNS and EMG were normal (run about 5 years after diagnosis for a different reason) -- but, my bloodwork was positive -- all THREE times -- three DIFFERENT labs!!

Should ALSO note -- you don't have to have positive bloodwork to have MG, either. You can have MG with negative bloodwork, negative EMGs, negative SFEMGs, etc. LOTS of smarter people in here than me -- they'll let you know more!!

Hi, Flash, and welcome. Reading your description of symptoms, I don't understand why the neurologist hasn't given you a blood test for myasthenia gravis. They can test for the antibodies. If they find them, that's a clear diagnosis. If they don't (about 20% of the time the antibodies don't show up on the blood test), they can test you further. You said you had an EMG, but there is a special kind of EMG that tests much more specifically for myasthenia gravis, called a Single Fiber EMG (SFEMG).

Many of us here took a long time to get a diagnosis, especially if we didn't test positive for the antibodies (we're "seronegative"). Of the seronegative people here, many of us, including me, were diagnosed on the basis of the SFEMG.

It took me 15 months to get a diagnosis because I'm seronegative and because my initial symptoms were atypical. The way I finally got one was by asking my neurologist to send me to a neurologist who specializes in MG. I think that most doctors are more than happy to give you a referral if they can't figure out what's going on. What got me the referral was a frank talk with my current neuro. I told him, Look, this is getting worse. This is really interfering with my life. I need to do something about this. I can't go on like this.

I don't know if you have MG, or what else you might consider, but I know enough about MG to know that the symptoms you describe certainly merit a blood test and a SFEMG!!! Many neuros would give you a trial of Mestinon based on those symptoms, too.

Abby

Hi Abby,

How would I know if it was a single firbre EMG?

He stuck one thin needle into me at various places. He changed needle before getting to my head for a thinner one. Not sure if that says anything?

Flash, with a single fiber EMG, the doctor puts the needle in, and then it stays in for about five minutes. During that time, he asks you to slightly clench a muscle and hold it clenched, while the machine collects the data. There are no shocks. Then he might move the needle to a different spot, and leave it there for five minutes, etc.

What makes this a bit confusing is that you may have had a regular EMG (with needles and shocks) in the same session. That's how they did mine--they gave me a regular EMG first and then moved on to the SFEMG.

Abby

I guess it was single fibre then - although he only left the needle in for about 30 seconds then moved to another area. No shocks, just asked me to clench then rest.

I had the nerve conduction study in the same session - no needles, just pads and 3-4 shocks on each bit he was testing.

I've read that even single fibre EMG can miss MG - is this correct?

[He said he wouldn't be able to stick them in my throat for obvious reasons!]

Yes, if he asked you to clench, that was a SFEMG.

We have a few people on this board who are diagnosed with MG even though their SFEMG's came back normal.

I had three SFEMG's. The first was borderline, and the second was mildly abnormal, but not (according to the neuro) enough to diagnose me. Then the neuro sent me to another neuro (I had to travel a bit) who is a special expert at diagnosing MG. He did a SFEMG and diagnosed me with MG on the basis of it.

It's so hard not to know what's going on. I hope you get some clear answers soon.

Abby

Update
 

A quick update:

I went to see another neurologist today. One that was recommended by a person who has MG.

Within about 5 minutes of me rambling on about my symptoms, the neuro interrupted me and asked "have you been tested for something called Myesthenia Gravis?"

I explained this has been dismissed by my doctor and a previous neurologist, but he insisted I needed to be tested for it next.

He is recommending a blood test and the appropriate EMG. He said I'd not had the right EMG done, I needed to repetitive stimulation single fibre EMG. He also mentioned another test which is more precise (a need in the forearm or something?)

Feel both happy (someone has seen something I suspected, so I'm not going mad), and sad (hell, I might have MG). I guess on the whole its good as whatever I've got I've got and at least it looks like I'm going to be tested finally and not dismissed.

There's still a good chance its not MG but he seemed say it was definately worth eliminatinig with proper tests given my symptoms

Good, Flash!! So, at least now, sounds like you are in good hands.

Don't fret too much about maybe having MG. There are many FAR worse things. MG usually doesn't hurt -- and we are often better in the mornings, declining as the day goes by. IF you turn out to have MG, just remind yourself that you do NOT have something as serious as ALS, cancer, progressive MS, etc -- and you should be able to keep your "chin up" :hug:. With time, you'll find ways to adapt. Life with MG is "different" -- but, different isn't always "bad".

Thanks for the update. I was so glad to read that you're going to be given the proper tests, but a doctor who evidently knows what he's doing. This is wonderful news.

I don't mean to minimize the suffering of people who have MG, but it's not as devastating a diagnosis as you might think. Remember that the people who post here are mostly in the thick of it--I think that the people who go into remission, or whose symptoms are so under control that they're not interfering with daily life too much, tend not to post here. So you could get a false impression by reading this forum.

One of the (relatively!) good things about MG is that it doesn't usually keep getting worse and worse, like MS, for example. Another good thing is that a lot or all of the symptoms are reversible, especially if it's caught early. It's also usually not painful, and doesn't usually mess with your mind or your bladder (two capacities I, personally, hope to keep under control). It sometimes goes into remission spontaneously, and it often goes into remission under treatment.

I hope you get a diagnosis, and that having a diagnosis lifts some of your mental burden. I felt so much better when my disease got a name. It was so hard not knowing what was wrong, or what it was going to develop into. I'm also not strong-minded enough not to doubt myself (I wondered at times if it was "all in my head").

Thanks for the update.

Abby

The other "good" thing about getting an MG diagnosis is that you can "forgive" yourself for feeling tired (if that makes any sense).

I couldn't understand why exercising always made me SO tired - especially since all you ever hear from people is how great it makes them feel, and how if you are tired all the time, "You just need to exercise".

Well, YEARS before I got an MG diagnosis, I couldn't stand to exercise because it made me feel worse, not better (it didn't help that I kept gaining weight, either - "You just need to exercise!"). And the longer I tried exercising, the worse it got.

Well, now I know why.

And now I can tell people that I CAN'T do strenuous exercise, because it quite literally could kill me. Shuts 'em right up..... :p

I sometimes explain to my herbalist friends (just take this supplement! It builds up your immune system!) that I can't take anything that strengthens my immune system, because it's too strong already. I tell them that I have to eat a lot of junk food and make sure my diet is really poor, because everyone knows that weakens the immune system.

This is utter nonsense, but it does make them get very quiet and back away slowly.

But seriously, folks, I am still learning the lesson about forgiving myself for being tired. I have been diagnosed for almost a year, and I know the effect this disease has on me. But it can be subtle! Before my latest IVIG, I kept thinking, "What's wrong with me? I don't feel like doing anything! I've become so lazy!" The thing is, I notice when a muscle is weak, like if I can't hold up my mug of coffee, or I collapse, or if it's a strain to hold up my head. But if I have mild, general weakness, I don't notice it explicitly--I just feel like I don't want to do anything.

After my recent IVIG, I caught myself thinking, "Why did I think this [you know, normal life] was so hard? I can do this."

I just wonder how many times I'm going to have to learn this lesson before it sticks.

Abby

Another member of this forum, someone who is very knowledgeable and for whom I have great respect, has written to me privately to question the accuracy of some things I said in this post. I thought that by using the word "usually" to qualify them, I was being careful, but now I see that my friend is right: what I wrote doesn't give an accurate picture.

The statements I'm referring to are: that MG doesn't usually cause pain; that it doesn't usually affect the bladder; that it's not usually progressive; and that symptoms can be reversible (I see that I especially overstated that last one).

I have read that these things are sometimes true, but I was saying more than I know when I used the word "usually."

Rather than just remove my post, I'm adding this comment so that anyone I misled is more likely to see my retraction. I won't name my correspondent without permission, but I invite that person to elaborate here.

I especially didn't mean to suggest that the people posting here were here only because they're sick, and not to help others. I certainly know that that's not true! I have received so much from the generosity of people here.

I'm sorry for my carelessness.

Abby

Hey that's OK we are all just trying to help!

Well, Abby, I'm clueless -- AS USUAL -- LOL!

It is hard -- at least for me -- posting in a forum. I rely SOOOO much on facial "clues" when I'm talking to people. And, also, knowing a LOT more about personal situations helps me.

Regardless, I end up stepping on toes and putting my foot in my mouth ALL the time!!! And, my brain fog, I'm sure, doesn't help one bit.

I guess.....I think.....well, I'm trying to see what you and the person who contacted you were talking about -- still not sure. Maybe it is the Topamax messing with my brain this morning.

When I read your original post, I took it "personally". I've been sort of having a "pity-party" of late. The first five years of MG were "bearable". But, this past year.........................not so much. And, I've been whining and complaining about it to my family......a LOT!! Then, I read the "newbie" saying (hell, I might have MG). and it just sort of "slapped" me in the FACE!! I mean, EVERY TIME I go to my MG doc, there are such pitiful people in the waiting room. People who can't walk or talk or hold their heads up from OTHER conditions -- some are dying (from ALS) -- and I'm sitting here having a big ole pity party about MG!! So, when you posted, it just made me sit up and say, "Hey, get on your big girl panties and count your blessings!"

I know that your original post wasn't INTENDED for me -- but, it HELPED me!!

Anyway, I hope you and the person who PMed you are ok. I think you are mighty special -- and I hold other members of this group in very high regard. I learn something almost everyday -- medical or about how to live.

Hope what I've said made sense -- and that I haven't "stepped on toes". :o

:grouphug:

I tell them that I have to eat a lot of junk food and make sure my diet is really poor, because everyone knows that weakens the immune system.

You owe me a new keyboard..... :p

Oh, thanks, Jana, you are so sweet to be concerned! The person who wrote to me did the right thing. I'm not upset--just glad to have the opportunity to correct myself.

It's so hard to find a balance when it comes to having the right attitude towards this disease, isn't it? Today I am especially discouraged, because I had my hopes up that the IVIG, especially with this new plan to give me boosters, would give me back my energy. I don't think it's healthy to pretend everything's fine when it's not...but on the other hand, I don't want to waste my energy on unproductive self-pity, either. I'm still figuring all of this out.

Thanks for your post.

Abby

Stellatum said:

" But if I have mild, general weakness, I don't notice it explicitly--I just feel like I don't want to do anything."

SO THAT EXPLAINS IT!!!!

I struggle with this about 5 days a week. I ponder over and over, am I lazy, am I depressed, what is wrong with me, I used to have to fight myself to stay in bed, why is it that now I don't want to get up? Then I say I'll just get up and bake something, or this or that, then I get up and I just don't FEEL like it, and I get back in bed.

I keep telling myself I've become too accustomed to being in bed, but meanwhile I"m sleeping 12-14 hours a night and feel exhausted! I also have a chronic bacterial infection or 4, so it's hard. I sometimes have a few decent days where I can pop an Adderall and accomplish stuff for the day, maybe one day a week on average. I remember when I had to wrangle myself down to stay in bed one day a week ( but that was pre MG and just when I had Lyme.)

Your line here made me feel SO MUCH BETTER about my laziness!!!

Back to my original subject ad a little update:

I saw my state funded (in the UK) neurologist and he has ordered a load of blood tests (myasthenia tests amongst them) and I await anxously, whilst my progressive dysphagia does not.

He also ordered a MRI of the soft tissue and the repetitive singe fibre EMG.

I actually hope its MG - weird as it sounds. Thats seems to be the only possibly treatable on on the list!

Flash

Re: just a thought flash
 

Did your Upper GI do an endoscopy? I just had one with biopsy and had my throat stretched. I had problems for years with this and it only got worse. This proceedure worked for me. I have barretts esophagus which accounted for my trouble in swallowing. Just a thought.....ginnie

My problem appears to be in the throat (pharyngeal). Could barretts be that high up?

Re: Yes flashster
 

Barretts can indeed feel like it is just in the back of your thoat. Things stop going down, you choke even taking a pill. It didn't feel like much lower to me at all. My stomach also had erosion, that I knew about and was being treated for acid reflus. If things seem to sit at the back of your throat, I sure would have that endoscopy done. Also the test and even the dialiation to allow me to swallow better was not uncomfortable at all. I ate my first apple in years yesterday, and just had it done on Wed. It would be worth it to at least bring this idea up to your doctor. I wish you all the best, and will be here for you if you have any more questions. ginnie

Hi Ginnie - were you sedated for either of your endoscopies?

I've had one when I was sedated and it wasn't too bad. This ENT seems to worry about the risks of sedation and he hasn't inspired me with confidence.

With sedation they've said they might want me to eat afterwards to check I'm ok ! (I can only swallow thin liquids so no chance)
Without sedation I'm a little nervous about someone ramming it down my throat when I often have trouble swallowing my own saliva!

Flash

Re: sedation question flashster
 

Hi, and I sure hope all goes well for you. I was sedated both times No mention of possible problems with sedation. I think they look at your whole medical situation. D you have other medical problems that could possibly put you into danger for sedation? I would not personnally do the proceedure with out it. I had enough trouble when they put a tube up my nose into my throat.
I had no ill effects eithor from the proceedure, or the sleep medication.
I had biopsy and throat dialiation at the same time, and no pain after. I would question your doctor alot since he has a concern, or expressed a negative comment about sedation. If he is not confident, seek another professional who you are quite sure is confident. Just a thought. ginnie

A quick update, and in case anyone recognises my test results!

All my bloods are now back from the tests the neurologist carried outin January.

The MG antibodies were negative, as was just about everything else.
>Apart from the voltage gated potassium antibodies which were at 122 (normal range 0-100)<

They are doing another EMG & Nerve Conduction Study today.

Based on those blood results, he mentioned something called Isaacs Syndrome, and also that whilst the antibodies to MG weren't detected, it doesn't mean its out of the frame yet.

If anyone has any comments I'd love to hear them. I presume they'll be looking for MG as well as Isaacs at the EMG later today.

I'm not taking any drugs or anything that can affect the EMG (I'm struggling to feed myself on liquids anyway so little I can take that will have any affect).

Wish I had something simple, and glad at the moment they are saying "ALS is out of the frame" (when I keep asking them).

Isaac's, aka Neuromyotonia
 

Do you have the fasciculations associated with Isaac's, a.k.a. Neuromyotonia? From what I've read I think it's not uncommon to have MG associated with VGKC and Isaac's.

Here's a link to the Isaac's forum if you wanted to read or ask questions:

http://isaacsyndrome.proboards.com/

Hi Geode

Yes I twitch alright. Tongue aswell now, hence my worries about ALS which the neuro says isn't in the frame.

I put it down to shear weakness. I gather swallowing issues can be a part of Isaacs also.

I have posted already on that forum - thanks.

Sorry to be jumping in late but thought I could add a little something that MAY help. My husband has MG and I used to be a speech-language pathologist. Many speech therapists specialize in swallowing therapy. If you haven't seen one I would suggest that you try to get a referral.
Depending on what the barium swallow study indicates, they may be able to suggest some compensatory maneuvers to help you with your swallowing difficulties. In some cases a chin tuck, head turn or making a point to hold your breath prior to swallowing may help. These are just a few of the techniques they may try, again depending on what they see on the swallow study. I am in NO way suggesting any of this may help, only a speech therapist who is evaluating you can make that determination.
Good Luck!!!!

Flashter - this is going to sound really crazy (but that's never stopped me before....):

Are you by any chance taking "calcium channel blockers" for high blood pressure?

The reason that I ask is that a few years before my MG diagnosis, my doctor tried switching my BP medicine for one that might work more efficiently. She mentioned in passing that some people start coughing a lot/gagging/etc. when they go on them.

I didn't think anything of it at the time, but a couple of weeks later, I started coughing a lot and noticed that I was gagging whenever I would take my meds in the morning. It got so bad that I couldn't take my meds easily because I had real problems swallowing.

I called my doctor, and she switched my Rx very quickly; as soon as I was off of them, those symptoms stopped. (My mom had the same reaction to CCB's, and had to be taken off of them as well)

Just thought I'd mention it, on the off chance that you are taking a CCB.

(As an aside, CCBs and MG DO NOT MIX; I don't know if a reaction to CCBs would warrant looking into the possibility of being monitored for MG or not - I am unaware of it being a "canary in the coal mine", but it's an interesting avenue of thought)

You might talk to your neurologist about doing a Mestinon trial - that can (in most cases) rule MG in or out pretty quickly. However, there are some forms of MG that don't respond to Mestinon, so it's not a foolproof "test".

People with MG are some of the only ones who are able to handle even a small dosage of Mestinon.

That's how I got "diagnosed" - all of the other tests came back negative, but I was able to tolerate 90 mg of Mestinon with no problem (120 mg put me on the couch in misery for a couple of hours) - most people can't tolerate 30 mg....

"normal" people don't tolerate mestinon?
 

That is very interesting--I'd not hard that before

I'm still on the diagnostic fence (MS or MG or residual cervical cord compression), but the 1 medicine I can't do without is Mestinon---I have problem swallowing thin liquids and mestinon seems to help

I just have intermittent penetration and likely chronic aspiration (evidenced by my frequent left sided lung infections) and taking the mestinon (60 mg every 4-6 hrs) seems to help

I've decided if it a placebo effect, I'm good with that b/c I don't get side effects (although things get loose if I bump it to 90mg)


I think neurologist's have to much fun putting people into boxes (diagnostic categories) and should focus more on actually helping people

Whatever the "answer" to you problems, just remember you are not alone