Question: Hi,

I am 35 and male. I initially had numbness and tingling in my left arm and hand in Oct 16, an NCS/EMG was ordered which showed mild chronic partial denervation in a C7 distribution consistent with a chronic cervical radiculopathy. Cervical MRI did not show any structural explanation for the symptoms but the issues got better and I was left with just some residual hand numbness.

In XXXXXXX 17 i started to experience percieved speech issues (only I notice) and fasciculations in many areas of the body. I had a full head and spine MRI which showed no structural issues or demyelinating lesions. A follow up NCS/EMG was conducted at a reputable clinic, they checked left and right arm and left leg multiple insertions in each limb. The results came back normal. I informed neurologist performing emg of my speech issues but he did not test my bulbar muscles. A second neurologist conducted a very thorough clinical and deemed it normal apart from assymetric triceps jerks, left very depressed, she said that in her opinion this lacked clinical significance. I also mentioned my speech issues to which she did not attribute any significance, she daid my voice sounded fine. This occurred in March 2017.

Fast forward to late July 17 and my speech issues have progressed, I percieve more effortful speech and mild articulation issues. My biggest concern is that I have experienced what i believe to be tongue atrophy as well as the mild deterioration of speech and I am very concerned about Bulbar ALS.

My questions are
1. Is a negative limb EMG very unusual in Bulbar ALS? I have seen it described in medical papers but how common is it?
2. Can tongue atrophy precede major speech disturbances?

MY MAIN QUESTION
3. Can tongue denervation atrophy caused by Bulbar ALS occur WITHOUT fasciculations?
I have what I believe is atrophy but no tongue fasciculations, is this possible in Bulbar ALS?

Thanks in advance for your time

Thank you for your answer doctor.

1. To follow up are you saying that in a patient with Bulbar ALS you would always see neurogenic changes in the limbs or have you seen patients with clean LIMB emgs that when their bulbar muscles have been subsequently tested show denervation in the bulbar region in the absences of any changes in the limbs?

2. is atrophy preceeding speech disturbances the usual phenotype? I understood that the usual course is for speech changes to occur before any noticable atrophy.

3. I would not say that the atropy/wasting I am seeing is significant. It is definitely noticable but I do not see a major reduction in function, speed of movement, strength etc. Given this is the absnece of fasciculations in the tongue suggestive that ALS is not the cause?

thanks for you time

Thanks for your response doctor.

i have uploaded 4 images of my tongue as requested.

Image 1 shows my tongue fully protruded which to my mind looks fairly normal and symmetric.

Image 2 shows the large indentation on the left lateral border and teeth indentations at the front on both sides
Image 3 shows the large indentation on the left again and the teeth indents, you can also see in this image that the left posterior of the tongue is much higher than the right exhibiting definite asymmetry.
Image 4 shows a more centered view of the tongue illustrating the above mentioned asymmetry from another angle.

Do these images look to be something of concern?

Also, considering that I first experienced perceived speech issues in January and it is now July, is that an inordinately long time to experience limited progression of bulbar dysfunction?

Thanks again in advance once again.

Thank you very much doctor for your responses, it very help and much appreciated.

In response to your last answer, Are you saying you are seeing "unilateral" tongue atrophy? If so, is this the typical presentation of tongue atrophy in Bulbar MND, I was under the impression that bilateral wasting is the typical presentation?

Also when protruded I do not have any deviation of the tongue. Am I incorrect in thinking that true unilateral atrophy would result in tongue deviation?

Given my limited progression over 7 months from symptoms onset is that unusually slow if it were Bulbar MND?

Any finally you say it may not be MND, Is MND the most likely diagnosis in your opinion with the information I have provided? And what are the differential diagnoses?

Apologies for the excessive follow up questions, I am just very anxious about this and am looking for reassurance that it isnt this horroble disease.

Thanks again in advance and for your patient explanations.