Another is this ALS

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Hi,

Just came from the EMG. Was verbally told that it was abnormal and consistent with ALS. I will post the results when I get them (likely in the next couple of days). The neuro mentioned that the person who ran my prior emg was a physical rehabilitation doctor and these sorts of tests really need to be done by neuros and thus the prior emg was more or less useless. Neuro reccomended a new second opinion, as she views the first opinion I got as worthless. She stated this isn’t really one of the cases where she has much doubt, though. I have one blood test outstanding and then all the rule outs are done (as noted most of the other mimic tests had already been done as part of prior testing).

I guess my story is looking to be more the exception than the rule. I will point out that literally everyone besides my initial neuro was at least somewhat concerned I had ALS. This included two speech pathologists, an ENT, a psychiatrist, a sleep doctor and neurological resident who were all quite concerned. A second neurologist said she wasn’t that concerned, but still referred me for a review by their ALS clinic. I just got exceedingly unlucky with a neuro who said it was stress and I should try talk therapy and a low dose antidepressant to help me sleep.
 
I should note during the delay by the original neuro, my voice quality has declined a good amount. I can no longer bank my voice, but could have several months ago if I had been taken seriously. I also could have visited family while I was still largely verbal, etc. I’m more than a little irritated. I’m going to do my best to get over it for my mental health, but the behavior seems quite outrageous (to me at least).
 
I am very sorry it is looking this way. Do you have any voice ? They are able to do more with less these days and can get a surprising amount from a little. Search for John Costello Boston Childrens. He runs the ALS communication clinic and there are videos on their website. I have been told they do remote consults too. I know Childrens sounds odd but that is where Mass General sends people and they have a robust program. Also this was a recent webinar from Les Turner foundation if you want to pursue some kind of voice preservation
 
Thanks, man. I have some voice left, it’s just not very good and takes a ton of effort to speak. I had looked into some voice banking options and they did not seem realistic (no way am I getting through 1600 intelligible phrases in less than months of daily work). I’ll check out the resources you’ve posted. I really appreciate the support.
 
There are definitely options with far far fewer phrases. Listen to the webinar I posted and maybe reach out to one of the people on it or to the clinic at Childrens. Asap because as you know bulbar als only gets worse Like do it before the weekend to try to get the ball rolling
 
Thanks. I reached out today. EMG is below:

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First, you are under no obligation to post any test results on this forum.

However, since you did, it looks like the only abnormalities are fasciculations. That is pretty much a clean EMG with a questional issue in your right tricep. Just because your extremities show clean, doesn't rule out bulbar onset ALS. My bulbar area showed nothing but all four extremities showed fibs, PSWs and reduced recruitment long before I experienced weakness.

If I were you I'd get a second opinion. I would think they would have done an EMG on your bulbar area. They did on me at both Mayo Clinic and Johns Hopkins. I wasn't having any bulbar symptoms and my swallow test was normal and that was reflected on my EMG.

Sadly, ALS is tough to diagnose but if your clinical exam demonstrated bulbar issues, I would want an EMG of the bulbar area proving that's what I have.

Best wishes.
 
I am getting a second opinion at Northwestern on the 26th. They tried for Bulbar but it didn't show anything (fasiculations or otherwise). Glad to hear a second opinion might be helpful in my case. My family is devastated. I won't tell them as I don't want to engender false hope. The neuro said the combination of the IA and fasiculations were consistent with ALS, but it sounds like there is room for disagreement there.
 
For what it's worth, I also have noticeable weakness in my right arm that is progressing, but really not that concerned about it unless it is indicative of ALS. I workout pretty strenuously, so something is frequently a little off in one of my muscle groups.
 
That is not the kind of EMG we see with ALS. Has a doctor diagnosed clinical weakness in your right arm?
This must be very stressful, I'm glad the second opinion is soon, you sure need a neuromuscular specialist to go through this.
IA and Fasc are not ALS, we would see lots of Fibs and PSW. It is the chronic and acute dennervation that is indicative and that is not there at all.
Let us know the results after the 26th, and you can always do the voice banking suggestion from Nikki in between.

May I ask, do you sound very drunk when you speak?
 
No. The only clinical weakness that has been diagnosed is in my tongue, lips and facial muscles. I don't have clinical weakness in my arm and it would be awhile before it shows up, I think. I do a good amount of weightlifting, but my right arm is just getting weaker and I'm having to use less weight as I have difficulty gripping and controlling it. Again, we are talking about high weights here, so definitely not clinical and is likely explainable other ways. It is a neuromuscular doctor who I saw for this. I'm seeing another neuromuscular doctor on the 26th. I don't sound drunk. I sound kind like a mixture of the people below with a good amount of nasality thrown in as I tire out. I've had an ENT, speech pathologist and neuro independently describe it as Spastic flaccid. A few months ago, it was "sub-acute" and now its "moderate" dysarthria. Forming the words is physically exhausting and I find myself getting winded and have difficulty coordinating my breathing (but I'm fine if I go running or biking).



I am new to all this, and definitely not a dr., but I thought the ALS criteria were revised to include Fasc as something like 22% of ALS patients were dying without ever making it to an ALS diagnosis supported by EMG so they were trying to fix that issue. Not trying to argue, I just don't know much about this process. I definitely want anything other than ALS.
 
The pre gold coast criteria were incredibly confusing. They had possible probable and definite ALS categories which made it sound like the first two were not ALS but they were / are and patients should have been told their diagnosis was ALS when they met the criteria for possible because possible meant ALS in one area. Probable was 2 and definite three. Some neuorlogists didn’t explain this and their patients had false hope it was something else. Gold coast fixed this. Meeting ALS criteria in one area is simply ALS The people who died before a definite ALS diagnosis weren't Really undiagnosed they just had stupid misleading label. Logically if you had bulbar and respiratory involvement but ok limbs you would be probable ALS by those standards but could easily die quickly. supposedly gold coast criteria are otherwise slightly more lenient particularly for bulbar. I am not sure how true this is in practice

the issue of fasciculation potentials is more complicated but you still need widespread involvement and chronic as well as acute denervation on emg.
 
It sounds like you are saying that's not what any of you see. I realize none of you have identified yourselves as neuromuscular neurologists, but value the opinion anyways.
 
FWIW, I've had suspicion of some sort of autoimmune reaction. I had an absolutely horrible case of hand, foot and mouth disease a few weeks before this started (lasted two weeks, couldn't eat for days, fever around 104, had to crawl for a few days because the blisters were so bad, etc.). I also had about 10 other illnesses in the 3-4 months preceding that (we had just started our one year old in daycare). No such luck on any autoimmune test, though providing results that could explain the severity of my symptoms per the docs. I realize this isn't the random illness symptom forum, so will discuss with the other doctors when I see them in a couple of weeks. I'll keep you guys updated.
 
You are right I do not believe there is a neuromuscular physician on this thread. Bulbar can be hard to diagnose. I am struck though the one tested bulbar muscle was completely normal which seems strange. I am happy you are getting another opinion You could of course have upper motor neuron bulbar disease. Some physicians if they diagnose that and see some abnormalities on emg call it upper motor neuron dominant ALS did you not get a summary page with this?
 
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