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.