Bulbar progression question

[moosey]

Greetings,

I am scheduled for an EMG in October and a swallow study in September. They've been monitoring me for the past 10 months due to fasciculations and atrophy in my legs. My doctor doesn't think it's progressive neuromuscular because everything has been bilateral, with no focal atrophy. I'm not eligible for an MRI.

I was wondering if my current symptoms would be typical or atypical bulbar progression (I understand there's no absolute typical progression) but if I'm concerned for no reason, it would be nice to know...

My first bulbar symptoms were in December '23, muscle tightness in my throat made swallowing feel strange and incomplete. No dysphagia.

The tightness subsided but swallowing still felt off. There was a secondary flare of tightness in March, felt lower in my throat than the first.

For the past 3-4 weeks my throat has been very tight, and I noticed last week that the two side arches in my upper throat/mouth aren't there any longer. I am getting slight dysphagia. The swallowing works but feels weak. The tightness seems to have migrated from the upper to the lower throat.

I am not having any tongue atrophy/fasciculations, or slurring of words. Voice can be a bit hoarse intermittently.

I try to explain it away to myself as peripheral neuropathy, and or autoimmune, but I am well aware PN rarely affects cranial nerves.

It's strange to say, but this has been ongoing for so long that I am fully expecting that I will be diagnosed in October.

 

[lgelb]

On what you've said, I don't share that expectation. I anticipate your studies will be reassuring as regards ALS.

Not sure what "side arches" means.

Flail leg is a bilateral leg onset but doesn't include bulbar issues. And it definitely includes weakness, not just atrophy, like all MND.

I would see an ENT to rule out structural disorders, but no, for me, you're not describing bulbar damage in ALS.

 

[moosey]

I had a swallow study and it returned as normal. I expected it would as my muscle issues are higher up and I am not experiencing dysphagia or any level of aspiration. The technician noted that I have scalloped tongue, usually indicative of muscle tension.

The mouth muscles that i was referring to are the arch muscles in the back of the mouth cavity. They are almost nonexistent now, they have recessed to almost flat.

My legs are declining but bilaterally and very slowly. The tightness hasn't been an issue the last few months, they're just slimmer in the quads and uncoordinated for anything akin to jogging. I have almost tripped a few times lately from scuffing my feet.

I am not exhibiting any brisk reflexes, or lack of reflexes. No abnormal signs with the standard clinical neurological tests that doctors do.

The throat and bilateral leg decline made me wonder about PLS, but wouldn't there be other neurological signs like positive babinski or hyperreflexia?

I have an ICD with a non-mri compatible lead, so no scans to rule out MS. My doctor said all we can do is monitor.

My EMG is late October, it's been a long time coming, and I was quite ambivalent about getting the results, but now I just need to get some answers.

 

[lgelb]

PLS is very rare and you have not described it. Has an ENT seen the changed musculature you mention? And what did they say?

As to the ICD, a CT can provide some measure of reassurance as to MS and many other conditions.

 

[moosey]

I haven't been referred to an ENT. The throat changes are relatively new, approx 6 weeks, and the Healthcare system in my province is crippled. There's a 6 year wait for Neurologist appointments referrals. You only get access really during emergencies. My family doc is making an honest effort, but the internist was the best he could get me, and he's the one saying wait and see. I expressed my frustration to him back in April when my legs and feet were declining and my emg wasn't until October, he replied that there's no cure for those diseases implying why would I be in a hurry to find out. It kind of shocked me into silence and I haven't spoken with him since.

Thanks for all of the great service you all provide to people worried or afflicted with MND.

 

[moosey]

I had my EMG appointment today. The neurologist did a very thorough clinical exam, and they did a NCS. The Neurologist said the NCS looked normal. I guess he didn't see much need for an EMG following the clinical exam, he said "I could stick a needle in you if you want?"

I was a little surprised as I was booked for an emg and ncs. I said yes, and instructed that the only unilateral issue was my right arm. He put a needle in my shoulder joint area and said everything looked normal. The technician said to him no denervation.

Honestly that's all I needed to hear, combined with the fact my swallowing feels slightly better after trying PPIs, I'm looking forward to putting this behind me and disregard MND as a cause for my leg issues.

Thank you all for your time and help and you are all in my thoughts.

 

[lgelb]

Thank you for closing the loop, with all best wishes for your health.

 

[moosey]

Apologies for returning after my previous message but as a bit of tike has passed and I've had time to process everything, I have a couple more questions that maybe someone can shed some light on.

Wouldn't the clean NCS be more supportive of ruling out other potential causes, and be more motivation to do an extensive emg?

I find it really strange he only did one needle in one shoulder?

Trying to undrstand/reconcile his thoughts for doing that. From a diagnostic standpoint, considering the 8 month wait, why not just investigate?

I know there weren't any common clinical signs, but I have been experiencing muscle tightness and my swallowing coordination been declining since July. My tonsils have disappeared, and my facial muscles are weakening. The last few days I've began to notice drool seeping out of the corner of my mouth at random times. Last night I woke up and I felt like I was oxygen deprived despite wearing my cpap.

Does anyone know any possible mimics that could be explain my throat muscles?

It's very unsettling.

 

[lgelb]

Your first question presumes that a normal NCS somehow points more to ALS than no NCS at all, which isn't true.

You acknowledge that based on the "very thorough" exam, the neuro saw no reason for an EMG. So it's not a shock that he didn't waste resources on more than a "dot the Is" measurement, which was negative in a symptomatic area.

It's also not a shock when CPAP settings need adjustment, or you need a different mask, a new cushion, to replace your filters, whatever. Drooling, waking short of breath, sore throat, etc. can easily be down to apnea that isn't being treated optimally.

I would schedule a DME appointment and make sure they have seen all your machine data (which it's wise to track yourself as well; it's your sleep). You can also video your night and track your respiratory rate, pulse, etc. with a smart watch or fitness tracker for more data.

If the tonsils are inflamed, your PCP can see and treat that. If they aren't sure if something else is wrong with them, that's what the ENT is for. But the notion that after a normal neuro exam, ALS has made them "disappear" within this short timeframe doesn't make sense.

 

[moosey]

I think maybe I sound like I'm arguing that it is ALS, which is not the case whatsoever. On the contrary I just want this all behind me.

I'm not sure when my tonsils disappeared or how long it took, definitely not the past few days. About a month or two ago I just discovered that they have completely receded to my throat walls.

I tried not using my cpap for 2 weeks to see if I noticed any throat improvement but I didn't and it had noticeable adverse effects on my general health so I stopped that. My cpap sends reports to my app every morning and it looks like things are OK, my events/hr are usually 0 to 0.5. It doesn't have 02 saturation data though.

I guess im trying to rationalize a clean emg, clean blood tests, clean ncs, no myopathies, with the swallowing struggles I'm experiencing. Drool slipping out of my mouth yesterday watching TV was a new thing too.

Figured I'd share and fish for some logical reassurance that these symptoms can be explained away.

Thanks again for taking time to respond to myself and the countless others you're providing a valuable contribution to people like myself.

 

[lgelb]

Glad to hear you are not stuck on ALS. Not using the CPAP is only going to make your sleep worse, as you found. What I meant was, things might need adjustment. It's like any other kind of therapy -- if it's not right, things can go worse than they were.

And events are not the only kind of meaningful data. Your sleep can be totally torqued with no-to-minimal event frequency as you cite. I know many people in this category. It can really help to have more biometric data.

A month is a fly-by in ALS time. But bear in mind that the things you see as related may not be, either.

 

[moosey]

I received a letter today notifying me of an EMG appointment in July 2025. Considering I just had one Oct 15th, where he didn't even do an EMG after my clinical exam and normal NCS, is this typical? I'm guessing the intention is to do another clinical exam and determine if an EMG is warranted at that stage?

The fact that it's 9 months from now makes it unlikely that it's related to something he saw and wanted to investigate further, which is where the mind goes at first.

I'm aggressively self treating a suspected/undiagnosed silent reflux with diet and OTC products, and am noticing a slight intensity reduction in my throat swallowing problems.