New thread- Concerns about symptoms aligned with Bulbar ALS/MND

[BeckyB]

Hello PALS & CALS,

I posted here in July 2022 after experiencing a range of concerning neurological symptoms. Could this be Bulbar Onset ALS/MND?

I underwent further testing, which thankfully came back negative and was told to “forget about MND/ALS”. I continued to follow up with my neurologist every 6-12 months. The plan was to “keep an eye on things”, “wait to see if and how things progress” and to “come in for a review if symptoms worsen”.

My symptoms did not improve, I just learned to live with them and adjusted my lifestyle so that they did not bother me as much.

I noticed that my muscle twitching became worse when I exercised and when taking certain supplements, so avoided these to the detriment of my overall health.

This year (2024) I started noticing further changes in my symptoms.

My current symptoms are as follows:

1. Fasciculations: muscle twitching all over body, but has recently increased in intensity, particularly in shoulders.
2. Swallowing issues: sensation of a swollen throat. Coughing after consuming liquids.
3. Vocal changes: this started as having a tired throat with a hoarse voice, but recently has progressed to a loss of my upper vocal range. Either no sound comes out or the sound that does is completely off key to what I intended. My voice is now lower and raspy. The problem feels at the back of the throat. The “ka ka ka” sound is difficult to do. There is no slurring of words currently.
4. Left hand: Clumsy. Occasionally dropping things. The last 3 fingers curl up, but I am able to stretch them out & still have use of the hand.
5. Perceived weakness
6. Left leg: cramping in calf, unsteady on left leg.

I saw my neurologist who performed a clinical exam, somewhat dismissed the vocal change concerns and advised me to see an ENT. He did not want to do a follow up EMG.

My last EMG was in July 2022 (4 limbs + hypoglossus) and I also had a videofluroroscopy around this time too to assess my swallow, which was thankfully fine.

Given it’s been over 2 years and my symptoms have increased, do you think a follow up EMG should be ordered?

I can’t explain away the vocal changes. I’ve had a scope down my throat previously and there’s been no signs of reflux or damage to my vocal cords.

I feel terribly guilty to be back here and bothering you about this, but please, I would so appreciate your suggestions on next steps.

Please note: that I’m NOT located in the USA, so do not have access to the ALS specialist doctors or hospitals often mentioned in this group.

Many thanks to those who have read this and decide to reply.

 

[lgelb]

It sounds like the major potentially concerning change is in your voice/swallowing, so I would follow the recommendation to see an ENT. I don't think it's ALS, but something like that should be checked out by a specialist, even though you had testing 2y ago.

 

[BeckyB]

Hello,

Thought I would leave an update.

I saw my GP regarding weakness in my left hand. It had initially been a feeling of uncoordination- fumbly & clumsy and some curling of the outer fingers, but in recent months, I’d noticed my grip becoming weaker, especially when using my thumb and first finger. I have been experiencing cramping in the palm of my hand and twitching in the hand itself in multiple regions including the thenar.

My doctor says there is mild wasting in the thenar eminence, reduced grip strength & thumb extension and has referred me for another EMG (my last one was in July 2022).

My husband & I have cried today. My doctor hasn’t suggested any alternative diagnoses. I had 2 clear EMGs in 2022 and I was told by my neurologist to forget ALS and move on with my life. I can’t wrap my head around how this can be happening now…

 

[lgelb]

I wouldn't make assumptions based on your GP's not having an alternative to ALS, which is more of a specialized area. Nothing is settled as yet.

 

[BeckyB]

Thanks for your reply, lgelb. You’re absolutely right of course.
EMG scheduled for later in the month.

 

[BeckyB]

Hello PALS & CALS,

I’m back with an update.

I saw my neurologist last week who performed a clinical exam & NCS/EMG.

He said the most important part of the appointment is the clinical exam and following that, he wasn’t concerned but would perform the EMG as I had been referred.

He did note that my thenar eminence was quite flat, but claimed it wasn’t atrophy in his opinion. The NCS/EMG was only performed in the one limb and apparently came back with no problems (although I have not personally seen the report). Only 4 muscles were tested via EMG, but did include the APB & FDI.

Do you think this enough to rule out ALS/MND as a cause for my symptoms? He had no alternative suggestions as to a cause for my left hand issues (beyond vaccine triggered FND). I have seen no improvement in my symptoms since the testing. I am still experiencing weakness, stiffness & twitching.

Thanks once again for your time and advice.

Becky

 

[lgelb]

Yes, I do, 100%.

I would ask your GP about a referral to an orthopedist or physio. Sometimes it takes digging into the signs to better understand what can be done to improve things, whether a cause can be identified or not.

 

[BeckyB]

It’s Saturday night and I’ve just finished organising Christmas gifts for my pre-schooler daughter’s classmates…

My left hand FDI muscle is visibly twitching. My thumb has been getting more progressively weak for months now.

My neurologist acknowledged to my GP that I have flattening in my thenar eminence.

But the EMG was apparently clear (tested 4 muscles of arm only, but included APB & FDI).

I don’t understand. I am so scared.

I was told 2 years ago to see how things progress… they have done, but still… no answers.

I am actively investigating other avenues- having an X-ray & ultrasound next week and booking an appointment with an occupational therapist.

Do I need another referral a different neurologist? Is there any chance the EMG is missing something? Can an EMG miss ALS/MND?

 

[lgelb]

Is your voice still affected? What about the ENT?
I would also ask about a sleep study.
No, I don't see how the EMG as described could miss damage in areas of concern.
OT and physio trials make sense, to get more information and possibly improvement. Are you right or left-handed?
What did the neuro say regarding next steps?

 

[BeckyB]

Thank you so much for your reply, Laurie.

My voice is still affected- lower range, raspy sounding & tires after talking a while. However, I did undergo an upper endoscopy in October which identified a tiny erosion that could cause mild reflux. I am currently not having any slurring, so decided if things worsen further with my voice, I’ll reschedule my ENT appointment.

Yes! Agreed. I certainly need a sleep study. Are you suggesting apnea?

I’ve been reminding myself that the EMG was performed in the most important hand muscles involved in ALS/MND, where I’m experiencing weakness AND fasciculations. Therefore surely (if it has been performed correctly) it would have shown abnormalities, yes?

I still have not seen the results (not sure I want to either because it will just lead me to Google & stress more), but I did see my neurologist’s letter to the GP stating: motor = normal , sensory = normal.

I cannot understand how I can be having these progressive & visible symptoms though, and the results not even show fasciculations. It casts doubt…

To answer your question- I am right-handed, thus am experiencing symptoms in my non-dominant hand. Over the last few months, my right hand has become more clumsy (like the left was initially), but so far I am not experiencing weakness in the right.

No real next steps provided by neurologist. I have a scheduled follow up in March.

 

[lgelb]

Re the sleep study, there are multiple possibilities for yield, including breathing and movement disorders.
You can get an rx for a basic one at home, often via telehealth if need be.
Yes, if the EMG is normal in your hot spots, that's pretty much a red X through ALS as a differential.
If the neuro said motor=normal, doubtless that's what they meant, over their legal signature.
EMGs do not often match up with what you think are fascics, cramps, weakness, what have you. They're measuring damage, not what you feel or how you move.

Seems like we're going in circles a bit, so please don't post again until you have a report from the OT or new test results. I would also ask your GP if you should be undertaking treatment relative to the erosion.

 

[BeckyB]

Hello. I am back with an update.

Saw the hand therapist & underwent assessment.

Left hand grip strength weaker than dominant right by 20%.
Left hand pinch test weaker than dominant right by 25%.
Should be no more than 10% difference between dominant & non-dominant hands.

Therapist confirmed issue is with the small hand muscles (mentioned FDI & APB specifically).

Hand splinted to see if rest improves things. I noticed the splint I’ve been given is one of the same types given to PALS.

I would really value your thoughts on this update.

 

[BeckyB]

AN UPDATE

Hello PALS & CALS,

Please allow me to share another update.

1. Second opinion neurologist

I attended a second opinion appointment with an experienced neurologist. He has requested repeat MRIs & will potentially do another EMG depending on the results of the MRIs. He reported to my GP that he “could not detect any significant facial muscle weakness” and that my reflexes were “depressed throughout”. He also wrote that he did not detect fasciculations, but he did not specifically look, nor did he inspect my weak hand. It was a very expensive appointment, but unfortunately I don’t feel like it yielded much.

2. Progression of symptoms

My left hand weakness (in the thumb & index finger) remains. It mildly affects my day to day living. For example- I struggle to hold my hairdryer with my left hand, lift a jug, use a towel to dry myself.

Over the last 2 months I have also developed twitching over the lower half of my face. It is very subtle. It started in my chin, then onto my upper & lower lips, next to the nose, then inside the nostril and along the bridge of the nose. Does this sound associated with ALS?

I have also had a couple of incidents of tripping over my left foot this past week, which has concerned me greatly. Neither stumble ended in me hitting the ground, so while I’m shaken up & very disheartened at the development of yet another potentially aligned symptom, I thankfully have not been hurt.

My next neurology appointment with my regular specialist isn’t until next month.

I do have more to report, but I’ll leave it there for now. My mental health is struggling through this diagnosis process. I’m feeling very isolated & family has said they don’t want to hear about it, because it makes them upset to hear that I’m not doing well. So I would appreciate any kind responses.

 

[lgelb]

A lot of people get hand splints.
No, subtle facial twitching is usually dehydration or too little sleep.
Speaking of which, what's happening re a sleep study?

A strength test showing something is up with one hand is not a shock. You're dragging in ALS when it's at the very bottom of the list based on findings so far.

Tripping can be those same things and anxiety.
But an ALS dx requires clinical-level impairment and your EMG didn't suggest ALS, either. I told you in November that was a red X through ALS and it still is.

There may not be a single grand explanation for everything you're experiencing, so presuming the neuro has no new findings, it's still time to attack the possibilities (including sleep) one by one. Your GP should be a help in this.

I would get on to that. Please don't post again until after your neuro appointment, and only if there are new findings.