Bulbar ALS questions

[Plucky81]

Hi All,

Seeking some guidance here, thanks in advance for your replies.

I am a 35y old male, Starting in October 2016 I initially had numbness and tingling in my right arm leading to an NCS/EMG, NCS was normal EMG showed chronic partial denervation in a C7 distribution consistent with a chronic radiculopathy. Numbness and tingling resolved but was replaced with symptoms of mild speech issues which no one else noticed consisting of a slight slur and general feeling of more effortful speech. In late January 2017 I went to my regular physician, who is extremely capable and thorough, with my concerns. He referred me and I had a second EMG at a clinic experienced in Neuromuscular disease along with a very thorough clinical exam. EMG was of both arms and left leg, many insertions and very thorough. EMG results were normal told no ALS. Clinical exam normal apart from assymetric triceps reflex, less in left arm told no ALS. Told EMG Neuro and Neuro who did clinical about speech concerns both seemed unconcerned and told me no ALS, dont need to see me again. Also I have had a ENT evaluate my mouth and tongue and a full head/spinal MRI both of which were unremarkable.

Since this time I have continued to have mild speech issues, only I notice and some mild swallowing issues. I think speech has gotten slightly worse over 6 months but its assessment is complicated by the long time frame, certainly has not improved. My most concerning issue is my tongue which is very assymetrical and very scalloped and looks altogether messed up, this has gotten worse over the 6 months. I do not see obvious tongue fasciculations.

My questions are:
1.How unusual would a clean clinical and clean limb EMG be in early Bulbar ALS?
2.Taking into account my young age (ALS wise), is minimal change over 6 months for Bulbar very unusual?
3.Can tongue atrophy ever occur without obvious fasciculations at rest?
4. Can tongue atrophy occur without gross deterioration in speech and tongue mobility?

Thanks in advance for any replies, I am very concerned about these symptoms and their insidious development. I have read widely to find out as much as I can but am reaching out in the hope that someone might be able to shed some light on the above.

 

[Plucky81]

I'm aware the above post is quite long, my apologies for that.

Could someone provide some insights

3.Can tongue atrophy ever occur without obvious fasciculations at rest?

Thanks

 

[affected]

1. won't happen, EMG will show ALS
2. highly unlikely indeed
3. probably and it means nothing - has a doctor diagnosed atrophy or is that what you are calling scalloping?
4. no

hope that helps - please understand the folk here are terminally ill, paralysed or caring for someone in that situation.

 

[Plucky81]

Hi Tillie

Thanks very much for your response.

In terms of your response to my first question I have to disagree that an emg of the limbs would always show up Bulbar ALS. It is certainly well described in the literature that the limbs do not always show neurogenic changes in Bulbar ALS. I know that many would say that because I had earlier limb symptoms that a clean limb emg excludes als however I believe my arm numbness was an unrelated symptom caused by a seperate pathology.

Thanks for your answer to my second question, I was coming from that one along the lines of I have heard it contended that those with a younger age of onset have a more robust pool of neurons and as such can show a slower progression. It is helpful to hear in your experience you believe in my situation Als is highly unlikely.

In response to your follow up question regarding what I believe may be atrophy. No it has not been diagnosed by a doctor but I also am not referring to the scalloping on the edges of my tongue. I am seeing very definite assymetry of my tongue with one side being much smaller than the other. I was hoping to hear here that LMN disfunction of the tongue resulting in atrophy much necessarily be accompanied by fasciculations.. but appreciate you letting me know that this is not the case.

Finally, appreciate your response the the final question in regards to speech quality and its relation to tongue atrophy.

Thanks again for taking the time to answer, it was helpful.