Bulbar ALS with clean EMG?

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AMAMP

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Hi all, I’m posting because I’m very concerned about my mom. I have no way of speaking with her doctors and she/my dad don’t seem to ask any of the important questions! (Frustrating) So I’m turning to all of your knowledge and experience to get some clarification.
7 months ago from today (09-03-2020) my mom (age 68) began having constant twitching and spasms in her mouth (lips and tongue) and jaw/cheek area.. it immediately made her speech slurred and weak. Since then her speech has progressively become worse along with her coughing while drinking or eating. Also, she has excessive drooling and saliva.. she always has a napkin with her to wipe her mouth. She has since seen SEVERAL doctors, neurologists, ents, speech therapist etc. none of them know how to help her. Her speech is very low in pitch, slow and monotone.. not nasally. She makes an EXTREME effort to form words and is now at a point where most of her words are unrecognizable. She eats so slowly and has no appetite...a couple times she has choked and my dad has had to heimlich her... she has lost 20 lbs.
Here’s the thing.. SHE HAS NO OTHER WEAKNESS in her body. She just checked into the ER, was admitted and they did an MRI, Brainstem MRI and an EMG.. all were clear. They told her she doesn’t have ALS. However the dr told my dad separately out in the hallway at the hospital that BULBAR PALSY is not off the table, so to speak.. why is that the case when her EMT was clear. I keep reading (from knowledgeable people) on these threads that a clear EMT MEANS NO ALS.. is there an acception for bulbar ALS?
My mom has an appt with yet another neurologist on April 29, 2021 to possibly have another EMG (again, why? if first one was clean and clean = ALS free) .. I guess I’m asking for your thoughts on her first EMG.. they tested her arms, legs and under her chin into her tongue... and all all was normal a could she really still have ALS? I guess I’m in disbelief because of all the stories on this forum where people had one or two clean EMGs before being diagnosed with bulbar ALS. Thank you for any knowledge you could pass along.

Also! I meant to add that everything else seems to have been ruled out.. all the other things that go along with this process.. nothing was a match for her.
 

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Another thing I remembered is that it has been discovered in her endoscopy that her swallowing muscles are ok.
 

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Bulbar palsy is not actually ALS, and it could still be a few things which is why they are still investigating. It must be hard not to be able to ask directly.
 

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No, with a clean EMG at this point, especially presuming bulbar muscles were tested, it is unlikely that she has ALS, but she could have a related disease as you say.

Pseudobulbar palsy would not show up on a standard EMG since lower motor neurons are not involved. Its features include difficulty in swallowing, speech, and emotional lability (unstable mood/swings). But your report that swallowing muscles are normal argues against that, through the abnormal gag reflex that is also part of it could explain difficulty eating, as well as choking, and swallowing muscles per se might not be involved yet.

Emotional lability would be an important clue that she might be somewhere on this spectrum, given the rest of what you've mentioned.

Progressive bulbar palsy would show up on an EMG of bulbar muscles since it is a lower motor neuron problem.

Both are motor neuron disorders, like ALS, with various primary causes. It sounds like most of the timely ones to know about, like a tumor, have been ruled out, but you don't mention a spinal tap, which could identify some disorders that MRI and blood tests cannot.

Anyway, at this point, in addition to finding anything treatable, I would want to make sure she is getting proper supportive treatment, like medications to address her saliva, a feeding tube if she is not getting enough nutrition, baclofen or tizanidine for spasticity, Nuedexta for mood if needed, etc.

Even if your parents want to handle the situation on your own, you can always email them a list of questions they could consider asking, in a nondirective way. It can't hurt.

Best,
Laurie
 
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Foodie1

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Your mom sounds like my mom. My was was 70 years old strong as an ox but was slurring her words, facial and tongue twitching,drooling and choking on food. Went to doctor after doctor and nobody knew what was going on. Then she saw two different neurologists in St. Louis and got to clean 2 EMGs and still the both neurologists didn’t know what she had. Finally she went to Mayo clinic and was diagnosed with Bulbar onset May 2017 and passed away August 10, 2019.
 

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Sorry it took me so long to reply..
thank you all for your responses. You all provided such helpful information.
Laurie, my mom has not had a spinal tap yet.. perhaps that is next? I don’t believe she has psuedobulbar palsy as she has been in complete control of her emotions. I will send a list of suggested questions with my parents for her April 29th appt. Thank you for that great suggestion!
Foodie1, I’m sorry to hear about your moms experience. May I ask how long it took from the bulbar onset for your mom to begin experience als symptoms in her lower extremities?
 

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That is a good question AMAMP. Let me think .........???
My mom was diagnosed in May of 2017. She started with raspy voice and then slurred speech probably around 2016 or maybe a couple of months before that. She did not experience ALS symptoms in her lower extremities until about a year after diagnosis which would have been 2018. She was still walking 6 months before she passed but then needed a wheelchair to help get around. After the wheelchair things started to really accelerate because of her non movement.
 

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Thought I would update this thread with my moms appt from her neurologist today.. she has been diagnosed with PLS. Still don’t know much about all of this but for now we are just thankful to have an answer. Thank you for reading and replying to my post.
 

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You and your Mom are very fortunate to get a PLS diagnosis in just one appointment.
 
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rmt

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AMAMP, your Mom sounds a bit like my husband. He is currently diagnosed with PLS and was given that tentative diagnosis after just over a year of symptoms. He started with speech issues and his swallowing was OK. After about a year and a half of speech issues he started having swallowing issues. We are now 3 years from the start of symptoms and his speech is gone by he is still able to swallow, though he has to eat slowly and carefully. And like your mom, he has no symptoms so far anywhere except speech and swallowing. His EMGs have been normal so far, so they are tentatively calling it PLS. But they say it won't be an official diagnosis until after 4 years (with still no lower motor neuron involvement).

Where is your mom being seen?
 

TippiLeigh

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@AMAMP I’m not on here at lot, but I just happened to notice your post. I was going to ask if they had brought up PLS yet until I read further down in the comments and saw that they did. I’m in a few PLS facebook groups and there are a few with Bulbar Onset. They’ve all started with clean EMG’s also. (Unfortunately many ended up with a dirty EMG down the road)

PLS is a disease that has no test, so we’re usually put on this 3 to 4 year plan of “wait and see”. During the “wait and see” portion, they try every test imaginable to see if it’s something else. And they are also waiting to make sure that you don’t start having lower motor neuron symptoms and end up with a dirty EMG. At my first appointment my neurologist suspected MS. After my MRI’s, at my second visit, he told me he thought it was PLS. It was that quick for him. But he can’t officially diagnosis it for probably another year or so, because I’m progressing a little quicker than they’d like and are waiting to see if this changes to ALS.

Sometimes, people that start with PLS can convert over to ALS. But with that said, starting with PLS or with upper motor neuron dominant ALS, usually gives you slower progression. And many times a person with PLS can live a normal length of time and never really progress. Unfortunately every persons story it totally different. I keep hearing lately people compare patients with motor neuron disease to snowflakes, because we’re all different...symptoms, progression, etc.

I’m so sorry about your mom. This stuff isn’t easy for anyone. You sound like a blessing to your parents though ❤️
 
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AMAMP

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Thank you so much for taking the time to respond! I think it was a tentative PLS diagnosis today.. so also waiting to see if in 3-4 years things progress to ALS. (I guess she has 1year down bc she had been having symptoms since last may 2020 but never told anyone) I’m still processing it all.. I’m so very sad for my mama. Her speech is almost indecipherable and she struggles to eat. So far her swallow muscles are ok so we’re thankful for that.. she uses a fork to move the food around her mouth and she takes 3X as long to eat. I just want to support her emotionally.. it’s hard to carry bc she doesn’t seem know that it could move into ALS out of nowhere. Anyway, she is going to an als clinic next month for a speech assessment and physical therapy. Today she was seen at Manchester st UCI building neuromuscular ALS office.. she was prescribed Riluzole. Have you ever taken it? Any advice? I don’t have any other information than what my dad shares w me and what all of you kind people here on this forum. Thank you so much for your help in understanding all of this.
 

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AMAMP, sorry to welcome you here. The support I've gotten here has been incredibly helpful so I'm glad you found this forum.

How is your mom communicating currently? My husband uses white boards to write things down and we now have them in every room in the house and in the cars. He brings one into the store and on our walks. It is a cheap and simple solution that would work for your mom since her arms aren't affected. He also has a tablet with a text to speech program and a little blue tooth speaker (that I keep with me) so that he can "talk" to me from across the room. It makes the conversation more "normal" but he is a slow typer so he usually just uses the white boards. And he has a text to speech app on his phone, but he mostly uses that if he forgets his white board.

Have they talked to your mom about a feeding tube? My husband doesn't have one yet, but I wish he did so that he wouldn't have to struggle to get enough food and liquids every day. You can have a feeding tube and still eat by mouth if you are able. My husband is still hoping he won't have to get one. I constantly have to remind myself that his health care is his decision and that I need to find a way to be OK with whatever decision he makes. In all honesty, I'm not there yet! It is so hard to watch somebody you love struggle and be powerless to fix it.

My husband was prescribed Riluzole but decided he didn't want to take it, so I don't have any advice on that. But I'm sure others will chime in.
 
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