Status
Not open for further replies.

GaborBP

Member
Joined
Sep 11, 2017
Messages
10
Reason
Lost a loved one
Country
HUN
State
Budapest
City
Budapest
Hi,
I started a discussion here last September, right after having a mildly (but widespread) dirty EMG. I'm sincerely grateful for the support and encouragement I received from the PALS and other people in this forum, they helped a lot to get me trough a very dark phase of my life. I'm checking in, because in the last 2 months my symptoms started to change in strange ways. A quick recap of my history

  • 29 yo male, father passed away from bulbar at 56, after 2 years. No family history, but head trauma and toxin exposure. SOD1, C9 negative
  • '15 August subjective left arm weakness, September: widespread but sporadic twitches, except for forehead over my left brow, it's going on non-stop (!) since '15 Nov. This is the only place where Lyrica doesn't work
  • MRIs, autoimmune bloodwork essentially clean (single T2 lesion)
  • Episodic trigerminal neuralgia, abducens palsy, muscle stiffnes. Gone now
  • '15 EMG clean, '16 NCV: some sligthly reduced CMAP, mild temporal dispersion, possible mild myelin damage
  • '17 EMG dirty: fib and slightly increased amp, mild poly in tibialis/forehead, inc. insertional activity in deltoid, a single fib in the thoracic paraspinals. CMAP slightly better, but more marked temporal dispersion, normal recruitment - not specific enough for ALS

The following things are strange/inconsistent to my neuro:
  • My left arm basically regained all strength about a year ago. I think that's what the EMG showed: irritability, no fibs, slight poly -> healing nerves?
  • Normal clinical, only UMN sign is myclonus, most likely from antidepressant.
  • Leg stiffness, weakness, joint cracking comes and goes
  • Slightly short MUAP combined with slightly increased amp, bordeline abnormal poly and good recruitment in TA. This is a weird combination

My concern is that in the last 2 months I started to get bulbar symptoms, mostly for swallowing water, rarely food. It started with throat gurgles and nasal congestion, which I can't clear (I still have those) now I often gulp when drinking water and swallow air/can't breath after drinking. I don't choke or regurgitate, and I do have both anxiety and signs of silent reflux. In fact, I've been coughing after meals, and having nausea in the morning for many years. I also had episodes of hypersalivation, but I can swallow that just fine. My tongue is a bit scalloped, but my neuro said it was no big deal. I'm still waiting for the swallow study appointment.

I sometimes have episodes of perceived slurring, but people say I speak normal. However, I had a scary episode after I had taken Mestinon, which was prescribed for me in a kinda ad-hoc way: I objectively slurred for about 30-60 minutes, and my tongue and around my eye I twitched like crazy for a couple of hours. It absolutely scared me, since I've never had tongue twitches, and I know that they are rarely benign.

My questions are the following
  • Can you have a short but serious slurring "attack" in ALS? For folks who tried Mestinon: can it cause dysarthria and a crazy tongue/eye twitching episode?
  • Are my swallowing problems consistent with how bulbar works?
  • And most importantly, have you ever seen anybody with similar symptoms being diagnosed with either an MND or anything else? My neuro is utterly perplexed.

I'm really thankful for those who took the time to read my post and I'd be grateful for any insight.
 
Weakness and twitching are known side effects of mestinon so the answerto question 1 appears to be yes
2 not in my family’s experience
3 same as # 2

Comment: your statement tongue twitching is rarely benign is wrong. I believe this common false belief stems from a comment by Professor Turner at Oxford that was taken out of context. He was speaking of people who already had MND. He said when asked that outside of that group tongue twitching( like twitching anywhere) is most often benign
 
Since your neuro is perplexed, I'd get a second opinion, but I don't see ALS in what you have reported.

Best,
Laurie
 
Weakness and twitching are known side effects of mestinon so the answerto question 1 appears to be yes
2 not in my family’s experience
3 same as # 2

Comment: your statement tongue twitching is rarely benign is wrong. I believe this common false belief stems from a comment by Professor Turner at Oxford that was taken out of context. He was speaking of people who already had MND. He said when asked that outside of that group tongue twitching( like twitching anywhere) is most often benign

Thank you! So if I understand correctly, a short episode (less than an hour) of slurring is not consistent with how bulbar works?
 
Yes you are correct. Bulbar can get worse if the person is tired so they might sometimes sound a little better first thing than late in the evening but a transient one time episode? No. Not anything I have seen or heard of.
 
Since your neuro is perplexed, I'd get a second opinion, but I don't see ALS in what you have reported.

Best,
Laurie

Hi Laurie,

Thank you for sharing your opinion, do you perhaps know of good neuros specializing in MND (and -hopefully in my case - mimics) in Europe, or some resource where I can find a list of such specialists?

My main problem with my neuro is not that she's clueless (she's not), I think she's perplexed because she doesn't really have the time to throughly follow up her patients. She's more or less the SINGLE specialist of ALS, SMA, Kennedy's, myopathies, dystrophies, etc., in a country of 10 million people. Oh, and she has to deal with little things like the swallow study department REFUSING to return any calls... :x

Best,
Gabor
 
Gabor, here is a list of a few centers in Europe. Depending on where you are willing to travel, wishmobbing in DE and others may be able to assist you as well.
 
Status
Not open for further replies.
Back
Top