Is this bulbar?

Status
Not open for further replies.

Jbarn

New member
Forum Supporter
Joined
Sep 23, 2021
Messages
9
Reason
Learn about ALS
Diagnosis
00/0000
Country
US
I would appreciate someone chiming in on this. This is my first time here, thank you for your time.

I’ve had laryngeal reflux for 13 years, I don’t get heartburn but a lot of sore throats, post nasal drip, etc. with that being said, in 2015, I had the LINX reflux management device (a bead of magnets the go around the esophagus to augment the lower esophageal sphincter) installed. This device is anchored 1mm from the vagus nerve bundle. Ok, fast forward to 2018, I started having some issues swallowing and had a EGD (camera that looks into your esophagus and stomach) that found I had presbyesophagus (esophagus forms corkscrew shape), the GI said it came from chronic reflux, but, the year before I had one done to check the effectiveness of the device and the esophagus was perfectly normal.

Fast forward to December 2019, I started having muscle fasciculations and in April 2020, had a EMG/NCV conducted at a MND clinic, both tests were normal. The device was causing heart attack symptoms (it was theorized it was hitting the vagus nerve causing heart attack symptoms because a thorough cardiac work up found no cardiac issues), and swallowing issues. In November 2020, I had the device removed and everything seemed to resolve.

In May 2021, I had another EMG done at the same MND clinic just to be sure I did not have ALS, and the tests were normal. At that time 17 months had passed since the start of fasciculations and 13 months since the first EMG/ NCV. None of the test up to that point tested the bulbar muscles.

In June 2021, I once again started having trouble swallowing and burping, and had another EGD and a High Resolution Manometry conducted. The EGD was normal but the Manometry revealed the esophagus was not functioning well, it showed the muscle was weak.

In September 2021, my VA doctor wanted me to have another EMG/NCV, because a head to tailbone MRI could not account for my swallowing issues and fasciculations. This time I went to UT Southwestern Medical Center, which is a certified ALS research hospital, and medical school. The doctor who did the study is a professor for MND at the hospital and the tests were once again normal. She tested many areas on the body and did test the 7th cranial nerve (for bulbar onset) but no other facial or neck areas.

So, 20 months, 3 EMG’s/ NCV’s, all by MND experts, swallowing issues, no breathing issues, no speech issues, but about 3 months ago I was tapping my right index finger on my knee and all of a sudden my finger went weak, no matter how hard I tried I could not tap any harder or faster. I was only able to tap around 40% speed and power of normal. It lasted about 30 to 45 seconds, then it went away, hasn’t come back since. When I mentioned it to the doctor she told me ALS doesn’t typically present that way, but she didn’t say never. So there it is, sound like als?

Thanks again
 
Last edited by a moderator:

Bestfriends14

Very helpful member
Forum Supporter
Joined
May 7, 2017
Messages
1,358
Reason
CALS
Diagnosis
05/2017
Country
CA
State
AB
City
CALGARY
Not in the slightest. This is because you don't have ALS symptoms and you've had clean clinical exams, plus unremarkable EMGs at more than one renowned ALS clinic. Is there a reason you don't believe this great news and the advice from so many prestigious doctors?
 

Jbarn

New member
Forum Supporter
Joined
Sep 23, 2021
Messages
9
Reason
Learn about ALS
Diagnosis
00/0000
Country
US
Thank you so much for your reply. The reason I’m struggling with this is because my swallowing is getting weaker and there has never been a full bulbar emg study done, despite me asking for one. The 7th cranial nerve innervates the cheeks and facial muscles, the 5th, 9th, 10th, and 12th, innervate the throat, esophagus, etc.

I told the doctor who conducted the last EMG that I was having trouble swallowing. She stuck her finger under my jaw, told me to swallow, then preceded to tell me she was not able to reach the area needed to check my swallowing. I thought it strange, seeing how it was UT Southwest and all.

I was having slight issues at that time with swallowing (33 days ago), but now, I find myself having choking episodes when eating and drinking. I feel as though it was missed. I would like to believe the doctor when she told me that if what I had was being caused by als, then it would have showed up on the EMG by now.

The problem with that thinking is that I have read on other forums where people had neurologist tell them the same thing, only for als to be diagnosed later. Then there’s the finger tapping issue. The doctor said it doesn’t typically present that way. I read a persons story where prior to diagnosis, they attempted to open a soda bottle, but couldn’t, and someone had to do it for them. They explained how it only happened that one time and that they were fine about an hour later. This was about 6 months prior to their diagnosis.

I’ve read many patients stories concerning this disease since ’20. It seems that there are many who had symptoms, saw neurologist, had emg’s, told they were ok, then later diagnosed with als.
 

lgelb

Moderator
Forum Supporter
Joined
Nov 5, 2009
Messages
10,532
Reason
Lost a loved one
Diagnosis
00/0000
Country
US
State
WA
City
Seattle
My first advice is to stop reading "patient stories," most based on biased and uncertain recall.

My second is to make sure you have been re-screened for GERD/EE/H. pylori, any of which which can damage your esophagus even after years of "stable" laryngeal reflux. Based on what you've said, you might also consider a second gastro opinion with someone who's more of a swallowing expert to reconsider the evidently equivocal imaging from a GI perspective. But none of this has anything to do with ALS.

You have presented no reason for testing more bulbar muscles on EMG. If you had ALS, it would show up elsewhere on the EMG. The hallmark of ALS is certain abnormalities even in areas with no issues.

Motor neurons that die in ALS don't flicker like a light bulb and come back to life after a few seconds. When they're dead, they're dead.

Best,
Laurie
 

affected

Guru status reached
Joined
Apr 26, 2013
Messages
13,781
Reason
Lost a loved one
Diagnosis
05/2013
Country
OZ
State
AU
City
lala land
I think you are confusing a few things.
there is no such thing as 'bulbar ALS', but there is 'bulbar onset ALS'.
Testing the 'bulbar' muscles exclusively is not relevant, as ALS, of any onset region, will show on an EMG in regions throughout the body.
This is a real problem when people who are not doctors decide to read some things online and diagnose themselves and suddenly know better than highly trained doctors and EMG technicians.
Please, return to your PCP and ask what could be happening and let them direct the next line of tests and specialities to consult.
 

Jbarn

New member
Forum Supporter
Joined
Sep 23, 2021
Messages
9
Reason
Learn about ALS
Diagnosis
00/0000
Country
US
As a follow up. In June of ‘20, I had to see a pulmonologist due to alleged reflux causing breathing issues. At that time my Forced Vital Capacity baseline was 6.77 liters. In April of this year I had to take the test again and did not look at the results because the doctor told me it was better than the one I did in June of the previous year. Yesterday, I compared my tests and saw that in 10 months my FVC dropped 1.3 liters. A high resolution contrast enhanced CT taken 3 weeks ago did not show any lung damage, at all. The MND doctors never tested my diaphragm, ever, just told me that if I had als it would have shown up by now on the EMG because my symptoms started in December of 2019. Lastly, just this past Thursday, my jaw went stiff on the left wide while eating, it was so stiff that I had problems opening it, and it’s still stiff, not as bad, but still there. What is your opinigiven this new information?
 

Nikki J

Moderator
Joined
Mar 22, 2012
Messages
11,012
Reason
PALS
Diagnosis
04/2014
Country
US
State
MA
City
Boston
The only time diaphragm emgs were done was to test for function when considering a diaphragmatic pacer. It was so unusual my clinic had directions posted in each emg room for the electrophysiologist to use as a reference. No one does them any more

that is still a normal value and respiratory function can change for many reasons and vary from day to day. Also it is only one part of the pfts and other values matter more. Even if your values were all worse that doesn’t mean ALS

and a suddenly stiff jaw doesn’t sound like ALS.

ask your pulmonary doctor about the pfts. Ask your primary and your dentist to evaluate the jaw issue
 

affected

Guru status reached
Joined
Apr 26, 2013
Messages
13,781
Reason
Lost a loved one
Diagnosis
05/2013
Country
OZ
State
AU
City
lala land
Try to remember that your doctors have had years and years of study, training, and practical experience.
So when they say things you don't understand, ask them to explain why.
When you get concerns like a stiff jaw and jump to ALS, immediately make an appointment with your doctor and get examined.
Every.single.time.

If that seems excessive, then yep, you are probably being hyperaware and googling too much.
If not, then an examination by a trained doctor who has a baseline to compare a new examination to, is the best way to see what has truly changed.

We truly can't tell you or advise you anything else, but I wish you the very best.
 

Jbarn

New member
Forum Supporter
Joined
Sep 23, 2021
Messages
9
Reason
Learn about ALS
Diagnosis
00/0000
Country
US
I have 2 sides to my symptoms, but had to wait to post. In the summer of 2019, my son and I were running laps in the swimming pool, the water was chest deep and It felt as though someone was digging their fingers under my left ribs. I asked if he could feel it and he said no. In December of 2019, I started having fasciculations. In April of ’20, I had my first EMG, leg, arm, hand, foot, etc, normal. No diaphragm or bulbar testing.

In July of ‘20, I noticed shortness of breath, so I went to see a pulmonologist for a pft. Everything was normal except where they could see a little restriction at the bottom of the lung bases, probably from my reflux. My FVC baseline was 122% of predicted, and I weighed 265.

In April of ‘21 I had a follow up pft, I weighed 255, and my FVC was 101% of predicted. I dropped 21% in 10 months. The pulmonologist wanted me to get a high res chest ct, which I did, it showed nothing.

As time went by I told my wife I could feel a fluttering under the left side of my rig cage, but chalked it up to digestion activity. Additionally, while in the gym, doing bench press, I would get cramps that I thought were in my back (now I know they were coming from my diaphragm), and would sometimes get them from slouching. In May of ‘21, just for good measure I had a second EMG conducted just to be sure I did not have ALS. Once again, arm, leg, foot, back etc. No diaphragm, no bulbar. All normal.

In September of this year a mri of the brain, and full spine revealed nothing, so i had a 3rd EMG done. Arm, leg, back, cheek foot, hand, etc. All normal. A few weeks after the EMG, another pft. The FVC baseline was 112% of predicted, but I weighed in at 242. I asked the pulmonologist if it looked like ALS, because my FVC had dropped from the year before, but it coming back up could just be from me losing weight. He told me I had no ”fatiguability” during the test, so he didn’t think so.

I have also noticed that I am currently having trouble saying certain words and am finding myself having to repeat them several times so i can annunciate them correctly. How possible is it I could have respiratory onset that started in the summer of ‘19, and 3 EMG’s over the last 22 months have missed it.

Thanks for your input.
 

ShiftKicker

Moderator
Joined
Mar 16, 2015
Messages
4,425
Reason
DX UMND/PLS
Diagnosis
06/2015
Country
CA
State
BC
City
Vancouver
Jbarn, I've moved your post to your existing thread. Keep on posting here, as it helps people see what's already been discussed.
 

lgelb

Moderator
Forum Supporter
Joined
Nov 5, 2009
Messages
10,532
Reason
Lost a loved one
Diagnosis
00/0000
Country
US
State
WA
City
Seattle
The answer is, it's not possible, with those robust FVCs (all above predicted values, which makes worrying about differences between years pretty silly), clean EMGs, and no loss of function.

I would seek a second opinion with an ENT and/or pulmonologist as regards your perceived shortness of breath, which has many possible causes, from atypical asthma to environmental toxins or allergies to cardiovascular conditions. The FVC is only one PFT value, and it's not the most reliable. But nothing you've reported suggests ALS, nor does the evidence support it in any way.

Best,
Laurie
 

Jbarn

New member
Forum Supporter
Joined
Sep 23, 2021
Messages
9
Reason
Learn about ALS
Diagnosis
00/0000
Country
US

affected

Guru status reached
Joined
Apr 26, 2013
Messages
13,781
Reason
Lost a loved one
Diagnosis
05/2013
Country
OZ
State
AU
City
lala land
Definitely no matter how much you add, it doesn't resemble ALS at all.
Did you read this post carefully? It might really help you.
 

Jbarn

New member
Forum Supporter
Joined
Sep 23, 2021
Messages
9
Reason
Learn about ALS
Diagnosis
00/0000
Country
US
I appreciate your reply, however, there is weakness. Last week I was eating and my left jaw became so tight I could barely open it. It went away after a couple of minutes and the soreness lasted two days. Today, my wife and I went out to eat, and two things happened that have never happened before. 1. My jaws were tired from just eating nacho, and my wife almost finished her nachos before me. I was in the military and have been eating fast since I was 18. Does that seem more consistent with bulbar als?
 

Jbarn

New member
Forum Supporter
Joined
Sep 23, 2021
Messages
9
Reason
Learn about ALS
Diagnosis
00/0000
Country
US
I did, but I think it’s only fair to remember that als is very heterogeneous, and the onset is widely varied. That is why I’m here, like many others, we are concerned that we may be facing the terrible disease that has taken so many loved ones. Here, we can have ones weigh in who have known, or been privy to others with atypical onset’s.
 
Status
Not open for further replies.
Top