How does bulbar ALS progress?

[Searcher]

I have been having seemingly increasing speech and swallowing problems the past few weeks, along with some intermittent slurring, feeling of tongue weakness and feeling tongue twitches. Yesterday a rheumatologist (examining me for other things) agreed that my tongue has less tissue and more grooved on Left side and that side is weaker on pushing to the opposite side than going the other way. There appears to be slightly more atrophy than before, along lateral border of tongue. Others have noted some slurring in speech in past few days when I've asked them about this, though have had this before, over many years, on occasion.

All of this, after having speech, swallowing and breathing problems - as well as mild weakness in many other muscles (hands, legs, neck, shoulders, etc.) off-and-on, going back over 20 years now. Had 2 EMGS in 80s suggesting Motor Neuron Disorder, but many more recent ones that were normal or showed only minor, limited changes.

So with this background, I really believe that now the tongue/speech problems are progressing; may have been so for months now, perhaps imperceptibly slowly. I believe, for instance, that some of what I and the rheumatologist are seeing as far as loss of bulk on Left side of tongue was the situation at least as far back as May, 07, if not longer.

My question is, if this is bulbar ALS, what is the range of how fast and regularly these symptoms progress? I've read that bulbar ALS usually leads to death sooner, but also read or hear about those whose ALS starts in bulbar region which progresses slower than average. What can I expect if this is, in fact, bulbar ALS? Can symptoms fluctuate for some time and then evolve into a slow but steady progression?

Is there any other disease or condition which can cause tongue atrophy, weakness, twitching, somewhat labored eating (15' for bowl of cereal today) and swallowing, along with hyperreflexia (according to doctor), weak or no gag and at least mildly weak and twitching muscles elsewhere?

Thanks for any civil responses

Searcher

 

[Zaphoon]

Wow, you've been getting tested since the 80's? Thats a long time! My civil response is that if you've been looking for a diagnosed that length of time, you don't have ALS.

My neuro told me in July I don't have ALS and I believe him. Now, we're trying to figure out if its MS, MG, PLS, a neuropathy, myopathy or dreaded tinkerbell disease. I just happen to have mirroring systems of the dreaded disease. (clean EMG = NO ALS)

If your neuro has told you that you do not have ALS, why not believe him/her? There are folks on this forum that would LOVE to have heard, "Its not ALS!"

But, I must qualify this with the disclaimer, I'm not a doctor. Just a regular guy...

Zaphoon

 

[Searcher]

A world-renowned ALS expert (Dr. Munsat) once told me that I have something wrong with my motor neurons. If he was right then, and all Motor Neuron Disorders are progressive, than just maybe this is evolving into ALS.

Neuro did say I don't have ALS in July, but have had new flare up and apparent progression of symptoms since then, which is why that reassurance doesn't hold as much weight right now. I will see him in a month and see if he detects the changes I'm experiencing and seeing.

 

[BethU]

I believe I have slow bulbar progression.

March 06, slurred speech (which I couldn't detect. People told me)
Fall 06, problems chewing from extremely dry mouth. Also dry eyes. Speech deteriorated.
February 07, problems swallowing liquids. Speech deteriorated.
August 07, first EMG ("clean"). Swallowing and speech continued to deteriorate.
December 07. Respiratory failure and 3-week hospitalization with entubation, etc.
May 08. DX with ALS. Second EMG showed 3 areas of denervation.
Summer 08, speech REALLY deteriorated. Swallowing has remained about the same.

After 2 1/2 years, speech is almost gone. Swallowing requires care. Some shortness of breath all the time. Weakness is spreading to my hands and arms (esp. left one).

 

[Searcher]

Beth,

Thanks for sharing your experience. Once the slurring started, did symptoms basically progress or were there signficant fluctuations or plateaus?

Thanks,

Searcher

 

[BethU]

Searcher, My blurred vision started at the same time, and there were definitely plateaus there ! I'd be stable for up to two or three months, then the deterioration would start again.

I started speech therapy about 9 months after the slurring started, and made remarkable progress ... in fact, I was scheduled to give a talk to a group, and told them I'd have to put it off a month, but I was almost back to normal. Then I had to have a minor surgery on my ear, and when I came out of anesthesia, my speech was worse than it had ever been. And it's been a slow, steady deterioration since then.

 

[Jennifer51]

speech therapy?

Hello Bethu...I was interested in what sort of things you had to do in speech therapy that you think made your speech better. I had bulbar symtoms in Jauary of this year, went to the gp at the end of march and DX in April with bulbar. I see a speech therapist regularly, but she said she would not give me exercises as it was more important to ensure I keep my swallow for as long as possible, she said speech was less important...but I WANT TO TALK again, at least better than I do now.... I understand where she is coming from but my speech or lack of it, really gets me down....I dream of just opening my mouth and it comes out normal...but hey, I wake up to find it just as bad...darn...jennifer

 

[thelma313]

My Dad's progression

My father was diagnosed with Bulbar Onset in August of this year. His started experiencing slurred speech in February 08 and it has deteriorated since then however he is doing speech therapy and it does seem to help him quite a bit. He definitely fluctuates. Yesterday he sounded pretty great and it was so nice to chat with him but other days it's a bit more of a struggle for him to talk. He has some weakness in his hands but not all the time. He also has one drop foot and wears a brace. His attitude is pretty good. He is following a strict regimen of vitamin therapy and an all-organic diet. He's doing quite well and we are all so proud of him.

 

[kylisa]

My mother's bulbar symptoms became apparent in Nov 07. She was diagnosed in Feb 08. She steadily progressed to now. Now, she has little voice at all, she has not eaten or drank anything by mouth for over a week. She does have a peg so she is receiving Ensure Plus and water at regular intervals. She laughs and sometimes cries so now her diagnosed may become psuedo bulbar palsy. She is beginning to go down quickly now. She rarely wants to get out of bed except to use the bathroom.

 

[patricia1]

My speech started in 99 There is no exercise I have ever had that was helpful.
The more I abuse those muscles the worse it was. So I stopped the exercises she gave me
I gave up on worrying about my speech I found not being able to use my hands arms and legs way worse and that isnt so bad to live with after a year
I guess we can deal with things as they come.
My biggest fear is the breathing getting to a point where I will have to make a discision to get a vent. But hopefully that wont be for a while,

I want to believe that God is with me during this journey,and helping me cope.

One thing I must say is I have met the nicest people during this time of my life that I would have never met if not for ALS,

God Bless Pat

 

[awieleba]

zaphoon~

what is a tinker bell disease, should I google it? LOL

 

[BethU]

Jennifer ... I've had those dreams, too! In my dream, all of a sudden, I can talk, and it turns out I just hadn't been trying hard enough. Then I wake up. :-(

The speech therapy I was getting was regular speech exercises, like they give to stroke victims ... repeating words with sounds I was having trouble with, "exercising" my tongue. For the first six months, they worked beautifully, and my speech improved ... and then I had that stupid second little surgery, and woke up with almost unintelligible speech. After that, I continued with the therapy for another six months, but it only made my speech worse. As Pat says, (and neuros and other speech pathologists have told me since then), doing exercises with an atrophied tongue is like trying to exercise an atrophied bicep ... it makes it worse, and wears down the muscle. I would be exhausted after 5 minutes of reciting words.

I have just had a really wierd, funky progression that's been all over the map.

BethU

 

[Marjorie R. Wilcox]

comment

Tinker Bell is the tiny fairylike "butterfly" from the story of Peter Pan, that cannot speak, but shows her attitudes by making a tinkling sound with her wings. If she is annoyed she makes the noise loud and fast. You have to look at her facial expressions to see what she is feeling....not unlike someone with ALS who can't talk anymore. Not a true disease I don't think, but a good representation. M

 

[Searcher]

My speech started in 99 There is no exercise I have ever had that was helpful.
The more I abuse those muscles the worse it was. So I stopped the exercises she gave me
I gave up on worrying about my speech I found not being able to use my hands arms and legs way worse and that isnt so bad to live with after a year
I guess we can deal with things as they come.
My biggest fear is the breathing getting to a point where I will have to make a discision to get a vent. But hopefully that wont be for a while,

I want to believe that God is with me during this journey,and helping me cope.

One thing I must say is I have met the nicest people during this time of my life that I would have never met if not for ALS,

God Bless Pat

Pat,

When were you diagnosed with ALS? You seem to have a slow case, and especially slow of bulbar.

BTW, think I once read somewhere that women get bulbar ALS initially disproportionately to men (though men get ALS overall at a significantly higher rate).

 

[Searcher]

Searcher, My blurred vision started at the same time, and there were definitely plateaus there ! I'd be stable for up to two or three months, then the deterioration would start again.

I started speech therapy about 9 months after the slurring started, and made remarkable progress ... in fact, I was scheduled to give a talk to a group, and told them I'd have to put it off a month, but I was almost back to normal. Then I had to have a minor surgery on my ear, and when I came out of anesthesia, my speech was worse than it had ever been. And it's been a slow, steady deterioration since then.

Beth,

I take it the blurred vision was from the MG? If so, mighty odd that the MG and the ALS symptoms would start at the same time, just coincidentally. Think you will try Mestinon again at some point?

BTW, is Dr. King Engel still at UCLA? Saw him for a consult around 18 years ago.