Inquiry for bulbar onset ALS

[thedeu]

Greetings and blessings:

I was wondering about the bulbar onset of ALS, and how did the pathology of it unfold for those who have been diagnosed with this type of ALS?

Essentially, do people just "wake up one day" and they have slurred or difficulty speaking? Or does it unveil itself progressively via signs and symptoms prior to actually having issues, i.e; fasiculations in the throat/tongue, "weird" or "not normal" feelings in the throat or jaw muscles, stuttering over words for a period of time before slurring them, trouble pronouncing certain combinations of letters before eventually losing the ability to speak, etc.?

Thank you very much for any information. All's well that ends ALS!
God bless.

 

[ottawa girl]

Hello.

It's been awhile -

How's the baby? Still working in the ER? Has your insurance kicked in? Did you ever go back to your GP? What did the GP think?

 

[thedeu]

Hi Elaine,

Baby is doing great! He is now 15 1/2 months old, walking, and getting into everything possible! I no longer work in the ER, since Nov of last year, however I'm still working as a funeral director. Insurance is good to go and yes went back to my GP and was referred to neuro. Saw my neurologist, gave him the low down as to what was going on. 2 Brain cts, brain MRI, cervical neck MRI, 2 EEGs, and a left arm EMG w/ nerve conduction study later, I was given a pretty clean bill of health. The only real significant result was a C3-C4 disc bulge with "severe" left foraminal narrowing (which would explain my left arm/leg numbness and tingling).

I voiced my concerns with ALS, and he seemed pretty nonchalant about it. He started going down the list of what is typical of ALS patients and how ALS typically presents itself. I think conducting the EMG was the final step he wanted to take from a neuro standpoint, and since I passed it, he referred me back to my GP to address my cervical issues. He really had no explanation for the fasciculations (which have almost completely gone away), and believed the vertigo was an ENT problem.

Suffice to say, I had pretty much moved on with my life, doing my best not to give into any hints of hypochondria (easier said than done), hitting the gym, getting back in shape, and enjoying life. And then along comes this "buzzing", constant feeling of pressure or tightness of the back of my tongue, underneath my tongue, and underneath my chin. This has been going on for months, intermittently, and makes me feel as if I really need to concentrate on what I'm saying. I've found myself stuttering over words, and often sounding like I have marbles in my mouth, where I have to take a moment, recompose myself and get my words out properly. No slurring of speech or no obvious (to others) difficulty in speaking. Just the perceived struggle that I'm having with everyday conversation. I'm at the point of when I read, I do so aloud, just to hear myself and monitor if I'm having any significant problems.

Of course, I've committed the cardinal sin of consulting Dr. Google, and as usual, I cannot find any relevant information regarding bulbar onset of ALS, in it's presentation, other than the common symptoms of slurred, tired, fatigued, or difficult speech patterns. I'm trying to gather info on the sign/symptoms (if any) that lead up to a true bulbar onset.

I place faith in my neurologist of 30 years, who didn't seem the least bit concerned that what I'm going through is ALS, and the fact that I passed my EMG yields some level of serenity. Yet, you still hear about cases where ALS patients walked away from a neurologist's office thinking or told that it was totally something else.

Ok, enough from my end....I'll ramble on and on if you let me! I'm thinking this is more "paranoia" than anything else. How are you Elaine? I love the pics you've posted! Indeed you truly are an inspiration for others! I love reading your posts and find comfort in your words. Thank you so much for touching base with me. God bless!

 

[bowser]

My wife's came on very suddenly.

One day she had difficulty pronouncing certain words and she refused to see a doctor.
We presumed she had a very slight stroke.

She did go later on when more of her speech became difficult to pronounce.

 

[thedeu]

Thank you Bowser. Your wife and your family are in my thoughts and prayers.

 

[vickim]

I am so happy for you. Living large and enjoying it all. Good for you and good luck in all you do.

 

[ottawa girl]

Aaaaaaahhh 15 months - so much fun at that age. Keeping you hopping, I'm sure.

I'm thinking your fear is creeping back in. I've never heard of buzzing tongue as a symptom. Why don't you check back in with your GP? Maybe he/she can quickly assess it and put your mind to rest. Stress, as you know, can cause weird symptoms too.

Thank you. I'm no inspiration - just doing it day by day... Just like the other 15000 American and 3000 Canadian PALS & CALS on any given day.

 

[thedeu]

LOL Elaine....yes, stress indeed. Being able to read the stories from ALS patients, has truly raised my own level of awareness with this disease. It saddens me beyond words, however I wish to further unfurl the banner regarding ALS, and do whatever I can to help others. You may feel that you're an inspiration, but you are a rock star in my book!

 

[thedeu]

You may not (sorry) feel that you're an inspiration, but you are a rock star in my book!

 

[ECpara]

My husband's bulbar onset ALS diagnosis came after very gradual symptoms. His speech was ever so slightly slurred, he thought a nerve had been hit during a dental procedure in March, 2011. Made sense. But then the slurring became more pronounced about 6 months later. Unbeknownst to me, he had fasciculations of the tongue, biceps, and legs, pointed out by the doctor at the Duke ALS clinic. My husband never pointed these out to me, perhaps he didn't want to admit it or scare me. That was in February, 2012. Today he still has those fasciculations, and although he can no longer speak or eat (uses a PEG), his limbs are still completely mobile. I do see muscle atrophy in his biceps, and his right leg. But he still golfs in the high 70s, low 80s! Go figure. I know in a matter of time these will go, just praying the slow progression will continue.

 

[thedeu]

ECapra, thank you for the information. I'm glad to read that your husband is still doing his thing! That is amazing. The onset of ALS is so unique to each patient, and I'm learning so much from people within this support forum. I appreciate your willingness to share. Thank you again.

 

[jellis86]

My bulbar onset started with odd swallowing issues and then slurred speech, that was mid 2011. The swallowing issues kind of went away for a while, but my speech continued to get worse. I had fasciculations in my tongue starting in May of 2012, or at least that's when I noticed them. By Sept. 2012 my speech was greatly affected, but I continued to work until Feb. 2013.

These days my speech is almost gone, I can still makes sounds, but can't pronunciate words anymore. I have a nearly 100% atrophied tongue which affects eating and chewing and of course swallowing. I am now on a modifies diet and also have a feeding tube.

So things took time to get from slurred speech to no speech, almost 2 years. I still have full use of my limbs but my left hand is starting to get weaker.

 

[thedeu]

Hey Jellis86,

I appreciate you sharing your symptoms with me. Knowledge is certainly power. From the point of mid 2011 when you started to experience "swallowing issues" and slurred speech, when were you formally diagnosed? What was the deciding factor/exam/test that led to the conclusion of ALS? Also, when your speech started to slur, was it a consistent variable vs. having good days with speech and bad days with speech?

Allow me to express my sorrow for your diagnosis, however I'm glad to read that your still maintain the use of your limbs! I love the John Adams quote btw! Blessings to you and yours.

 

[jellis86]

thedeu...I was "officially" diagnosed on Feb. 14, 2013. But my neuro mentioned it as possible in Oct. of 2012.

My speech started slurred but I did have good days and bad days. Amount of sleep, how much talking, etc. affected it. But even on good days it was noticeable.
As far as swallowing that's a different deal. I actually went from having real problems with swallowing solids food in mid 2011, to no problem with solid food in mid 2012. Then in the fall of 2012 thin liquids started to give me an issue and later, solid foods again. But I can still deal better with most foods than I can with any thin liquids.
These days I'm on a fairly soft diet and take most liquids and meds via my feeding tube.

Thanks and never hesitate to inquire further. One thing I had a hard time finding on here at fist was what peoples symptoms were when they first realized something was wrong. I believe in telling my story as often as possible, not just to help people diagnose themselves (always a bad idea anyway) but maybe to help someone realize they don't have ALS!

 

[affected]

One of the odd things is that you don't always realise all the symptoms until you have the benefit of hindsight.

Well that's how our story goes anyway.

I first noticed he was always doing a funny throat clearing kind of cough, and I mean he was doing it a lot, and had never done it before. This started a good 8 months before any symptoms he actually noticed.

My PALS noticed that he couldn't whistle, and even on a hot day if a breeze hit him his jaw would tremor - like teeth chattering when you are cold, but he doesn't have to be cold.

Before even the real slurring started he would have trouble with cold water - he describes it that he would take a mouthful, his palate would shut down, the whole lot would just come spraying out.

He also would have uncontrollable outbursts of quite hysterical laughter, and have to walk out of the room to calm himself.

Neither he nor I connected these 4 things together at the time.

Then the slurred speech started and like Jellis in the first months some days it was better than others but it was getting progressively worse.

He also had night issues - teeth grinding, biting his cheeks, moaning and biting his tongue. He would wake up a wreck!

Within 3 - 4 months of the speech problems starting he also began twitching in the left arm just in one or two spots, then both arms until he was twitching from his shoulder blades down to his finger tips whether awake or asleep.

It took 9 months from the start of the slurring until we got a diagnosis, but I should say too that he would not go to a doctor until I insisted, 3 months after the slurring started to become really noticeable.

All the usual tests to exclude other issues were done during those months.

By the time of diagnosis his speech had deteriorated so that he was very difficult to understand, he had to be very careful with fluids in particular and eating was a slow careful process as well.

He had clinical weakness in his hands, particularly the left and brisk reflexes pretty much all over.

Today, 15 months after the beginning of the slurring he is on blended food, his hands are becoming claws, he has muscle wastage all over, very weak arms, legs are wasting and he has had some falls this week. He has resisted the feeding tube, but is now waiting for this to happen and having a hard time getting enough nutrition and fluids and is losing a lot of weight.

Hope that helps, if it is ALS, and bulbar onset, it is still different for many people and the progression times are unpredictable.