Questions about bulbar onset

Like many here, I come seeking advice. I have read all of the stickies and tried my best to "research" what I can about ALS. (I am a junior high science teacher, and the lack of answers and knowledge about the pathogenesis, etc. is baffling to me. But I should keep this short). Sorry).

About a year ago now, I developed severe chemical sensitivities which caused breathing problems, throat swelling, nose bleeds, etc. (For those who are unaware of what this is, as I was, it is a wacky neuroimmunological overraction to things that we mostly don't notice: perfume, detergent, etc. Not an allergy, per se). I was forced to stop working after 15 years (I was 38 at the time).

Well, the sensitivity improved (so I was able to return to work) but I started to get fasiculations. I had no idea what these were. Then neck weakness. To make a long story a little shorter, I finally ended up at UCSF where they did an EMG of my arm and leg. Did an exam. Brisk symmetrical reflexes +2, no clonus, normal arm/leg EMG. This happened in October.

I am still concerned, inspite of the clean EMG, that I may have early bulbar onset ALS. The doctor thought I'd be dead by now or seriously sick so no EMG of tongue, etc. (Twitching started Feb). But from what I've read here, some people had clean EMGS and had bulbar ALS. I guess time will tell, but I have an 8 year old daughter and my heart is breaking.

Here are my symptoms. (Yes, I told the doctor).
1. Fasciculations all over, but mostly in thigh by knee, upper arm, etc. mostly left side. And area on left side of mouth.
2. Increasing difficulty swallowing liquids. Not water, but anything like OJ, etc. just hangs out in the back of my throat. Did swallowing test, per dr at UCSF. Okay, except for thick liquids.
3. Lip "atrophy." Lips look smaller. Hard to whistle now, but can, sort of. But not loud anymore. And that is a great Junior High Teacher Survival Skill!
4. Uvula points to right. (To be fair, I never looked at it before this, so I can't say what it was up to!)
5. Aspirate after eating, especially (and sadly) chocolate. Got a fab case of pneumonia. Dr at UCSF ordered cystic fibrosis test for this reason. Negative.
6. Hiccup immediately after drinking Coke. One hiccup. Odd.
7. Increased gag reflex.
8. Crying episodes, decreased by Zoloft. (I'm not depressed. And I DON'T normally cry).
9. Severe shoulder/neck cramp on left side.
10. Exercise intolerance. (If I sit around I cramp less).
11. Jaw cramp.
12. Teeth chatter after I move my face muscles.
13. Losing bulk in hands, especially left.
Sorry. That was too long. But I would like to know if this sounds similar to anything you or your significant other went through.

Thank you.
"Lea Moore"

Somebody more familiar with bulbar will give you an authoritative answer, but in the meantime I'll tell you that I didn't see ANY ALS symptoms in your list.

chemical sensitivities; might be the cause of your problems.
normal arm/leg EMG; that's good.
some people had clean EMGS and had bulbar ALS; I'm not aware of this. What are their user names?
Fasciculations all over; could be caused by many conditions--no need to go to ALS
difficulty swallowing thick liquids; doesn't necessarily point to ALS.
Lips look smaller; Now that's a weird one. Never heard of this. Atrophy occurs after a muscle is unused.
Uvula points to right; Now there's a conversation starter! Oh...uvula. Nevermind.
Aspirate after eating; My recall of bulbar is that the tongue gets paralyzed, which would be really obvious.
Hiccup immediately after drinking Coke; Let's just forget you listed this.
Increased gag reflex; never heard of this being associated with ALS.
Crying episodes; This is important. But could be caused by your anxiety thinking about ALS.
Exercise intolerance: If you're exercising, I wouldn't worry about ALS.
Jaw cramp; anxiety
Teeth chatter; ? anxiety?
Losing bulk in hands, especially left; If a doctor can see this obviously, then it could be a problem, but not necessarily ALS.
Severe shoulder/neck cramp; You also mentioned neck weakness. Is it still bad, or does it get better sometimes?
In ALS, a weak muscle just gets weaker, it never gets better.

Well, Lea. I'm not seeing ALS in your future. --Mike

So what are UCSF's differentials, given your abnormal swallowing results and aspiration pneumonia? They haven't just said, "you're fine," have they? What's the diagnostic plan?

I would ask about myotonic/myasthenic syndromes, some of which have blood markers that are rarely falsely positive, like the AChR Ab test.

But as you have read, bulbar onset ALS that caused your sx would almost certainly show up in a limb EMG. I don't think MND is your problem. If UCSF is not on the case, there are other good neuromuscular centers in your area. You might read the sticky "Getting a diagnosis" for some pointers on the process. I can't attribute all the phenomena you describe to chem sensitivity.

Atsugi, thank you for your answer (and sharing your excellent sense of humor). Glad you don't see ALS in my future. Yes, the shoulder weakness is getting worse, slowly though. It is odd, but I'm mostly weak when I try to hold something out in front of me straight, with my left hand. Can't pick up a pot straight off the stove, etc. With the left. The doctor said I wasn't clinically weak, but I'm weak for me. But it does hurt sometimes. Actually most of the time, as I am darned determined to keep using it. The doc gave me klonopin (sp?) and that help a bit. I know it helps with the worry too. I'm sorry. I tried to remember who posted about having a clean EMG and having bulbar onset ALS. My memory is lousy now. I think one post was by a woman who lost her husband to the disease. Her user name started with co---, but that's all I can recall.

Igelbe, thank you for the insight. The plan was the CF test and swallowing test. I guess that is it. I must say, though, I have had almost every test known to mankind and "passed" except a bulbar EMG. (haven't had one). They tested me for all kind of of antibodies to everything neurological and probably all non neurological too. None present, except high anti TPO, but thyroid is still good, so they aren't concerned about that. Yes, I don't think it has to do directly with Chemical Sensitivity, but I feel both could be related to some chemical exposure.

Thank you. Have a great new year.

"shoulder weakness is getting worse, ...when I try to hold something out in front of me straight, with my left hand...it does hurt sometimes. "

Not an ALS thing.

In ALS, motor nerves in your brain are destroyed, so the nerves can't send a "go" signal to your muscles. Thus, the muscle doesn't move. That's weakness. There's no feeling involved, no feeling of weakness, no burning, no feeling of fatigue or hurting--the muscle just doesn't go anymore.

Good luck sorting this out with your doctors.

Thank you.

Bulbar ALS will show up in a limb EMG.

All that pain when trying to do things really points away from ALS.

My husband was bulbar onset.

Thank you Tillie.

Lea, Tillie is correct. My husband also had bulbar- and it showed in the limb EMG.

I would look for a new neurologist who specializes in neuromuscular disorders. Give them a copy of all your tests and see what they think. Go to a reputable doctor. If the tell you no als, believe it.

Many things you describe are sensory based. Als is a motor or movement neuron disease. You seem okay in that area. Have you been tested for vitamin defiencies, heavy metals,muscle enzymes, myasthenia gravis, had a brain mri? If not I would ask for the reasons the doctors don't think it's necessary.

I am almost certain you suffer from anxiety disorder of some sorts like I do. Most people don't (or are unwilling to) recognize it. It's especially hard on science orientated people, who are generally very rational, like you and me. I got master's on applied thermodynamics and I am director of department with 50 people and yearly budget of over 60 million euros. I can assure you that I very much believe in probabilities and being rational. Except when it comes to anxiety and fears it will bring.

Fasciculations, in this case most likely Benign Fasciculation Syndrome like I have. According to study done by Mayo Clinic is not only completely harmless long term but has strong correlation with history of anxiety. Did you know that muscle twitching is listed symptom of anxiety. Fasciculations are amazingly common and have variety of reasons, 99,9999% which are completely benign of nature. It's annoying but I wouldn't worry about it too much (although I do but it's easy to say to that someone else).

Swallowing problems, again typical anxiety symptom. Also more you concentrate on it the harder it gets. Trust me, I've been going through same fears. I also happen to have very bad case of reflux disease and I'm currently waiting for operation. The ALS specialist I met had me drinking handwarm water and said that it's one of the main indicators as both hot and cold water generate some sort of reflex but bodywarm water forces your muscles to do the swallowing.

Lips, could that have something to do with you paying attention to it? Sounds bit weird really. Whistling thing, I thought that myself as well but again, paying attention to it HAS to make it harder for you.

Had to check it, my uvula isn't symmetrical either. I'm sure any given doctor, who check probably dozens of mouths in a week, would have noticed if there was anything weird there.

The aspirate thing. Are you sure you don't have reflux disease? Extemely common and chocolate is the worst. Also can cause mild cases of pneumonia. Sounds like something to do with chemical insensitiviy as well as pneumonia.

Hicvup after Coke. Errr... I thought that happens to everyone. Again, reflux disease would make that worse.

Increased gag reflex. Is this something doctors have told you or something you have noticed yourself? I thought in bulbar ALS loss of gag reflex was the main problem.

Crying episodes. Are you ABSOLUTELY crtain you aren't suffering from generilized anxiety disorder or something similar? Who ordered you Zoloft? It is not only depression but also anxiety medicine. Also are you using it regularily as otherwise it's no use.

Jaw cramp/neck cramp/teeth chatter. I'm no doctor but I'm pretty sure you suffer from bruxism. I know I do and I get all those things regularily and have had them all my adult life. Again, bruxism has high correlation with anxiety.

Andy clearly put time and thought into this post. So while it is against our rule I have left it and messaged him to explain the reasons for our rule against posting on the thread of other DIHALS. I have great hope that both he and Lea , now reassured , can move on from here. Of course we wish them both well

Nikki J said:

Andy clearly put time and thought into this post. So while it is against our rule I have left it and messaged him to explain the reasons for our rule against posting on the thread of other DIHALS. I have great hope that both he and Lea , now reassured , can move on from here. Of course we wish them both well

Click to expand...

Thank you Nikki and duly noted

Thank you for your thoughtful and detailed post. Yes, I am definitely hoping it is health anxiety and that I can move on.

Other than the swallowing, reflexes, and fasiculations (which could definitely all be due to anxiety), the thing that worries me the most is my left hand/neck. (I'm not sure it's the neck. It is my left shoulder muscle/goes up neck). My left hand looks skeletal. This is within the last year. The students have asked me "what's wrong with your hand?" when I accidentally put it on the lumens/overhead. (Since I'm right handed, I can usually avoid this). Thenar muscle's still there, though, far as I can tell, but less than right. The hand works, but I have trouble doing stupid things, like passing out papers/separating them. (But the students love to help!). And the left arm is weaker. Shakes if I rest on it, shoulder/neck cramp, etc. (Could be anxiety). But I have a hard time blaming the hand on anxiety. I decided to go back to the doctor in Feb. at the als clinic so he can check it out.

However, since I have had more than one (read: a lot) ncs/Emgs over the last year, I am now worried that all that "shocking" might have led to/caused a mnd. Because I did start out just with twitching (after the chemical sensitivity went away). And I think (with no degree in medicine or expertise) ALS is probably caused by excitotoxcity from repeated low level neuronal insults.

(Yes, I know I'm probably going to get told 1. Too many tests. (True). 2. You don't know what causes mnd, as the experts still don't know (I agree). 3. You need to see a doctor and believe him. (true. But can't help feel something is wrong. I know my body. But still true). 4. Get help for your anxiety, no matter what the outcome. (Doing so. But every time I look at my hand I think "This doesn't bode well...:-?" 5. You are putting tremendous stress on your marriage. (yes)).

Once again, forgive me for being verbose. I will move on (unless the doc rules otherwise). Thank you.
Lea