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What Are The Symptopms Of Myasthenia Gravis?
Question: I have been diagnosed with bulbar palsy. However I wonder if myasthenia gravis could still be a possibility. 2Hz repetitive stimulation was done pre and post exercise on right trapezius muscle and facial (nasalis ) nerves. No decretment noted. Nerve conduction studies on right arm were normal. Nerve conduction studies on left leg, left and right arms were normal. Needle EMG demonstrated increased insertional activity in the right tongue and one complex repetitive discharge in the right trapezius, otherwise was normal. Interpretation: No evidence for diffuse lower motor neuron process or a defect of the neuromuscular junction on this study. MuSK antibody and Achr antibody tests came back negative. My only symptoms is difficulty with speech articulation, slower tongue movement in terms of moving food around in my mouth. Dr. didn't do Tensilon test. This has been going on for about 5 months. No weakness in limbs.
Brief Answer:
There is no requirement of Tensilon test.
Detailed Answer:
Hi Ms. XXXXXXX
Thanks for your query.
1. Firstly, Bulbar Palsy is not the name of a precise disease, but a conglomeration of signs and symptoms, due to weakness/paralysis of muscles involved in speech, swallowing, chewing and, articulating or words.
2. Bulbar Palsy is one of the manifestations of some neurological conditions, including Motor Neuron Disease (abnormality of nerves supplying the muscles); Syringobulbia - a congenital dilatation of the inner tube of the spine; or, Myasthenia Gravis- an abnormality of the immune system.
3. All of the above-mentioned possible conditions progress very slowly. In your case, it is apparently a very early stage of one of the above-mentioned conditions, whereby which, only the nerves- which supply tongue and muscles involved in speech- have been affected so far, while sparing other nerves of the body.
4. You have already undergone tests for Myasthenia Gravis. These diagnostic tests have already RULED OUT Myasthenia gravis. Hence, THERE IS NO INDICATION FOR CONDUCTING TENSILON TEST, which is relatively a less-specific test, done to rule out or suggest Myasthenia gravis.
5. Hence, I am of the personal opinion that an MRI of upper spine should also be done to rule out/detect Syringobulbia as the cause of Bulbar Palsy.
Consult your Neurologist and apprise him of my opinion. I am certain that he will agree with me and would actively consider ordering MRI of upper spine.
Hope I have addressed to your concerns to your complete satisfaction and you find my response helpful and informative. I will be happy to answer further queries, if any.
Fond regards and have a nice day,
Col (Dr.) Rakesh Karanwal
Above answer was peer-reviewed by :
Dr. Chakravarthy Mazumdar
I guess I have two follow-up questions. I don't understand how he came to the conclusion of bulbar palsy. If the EMGs are normal with the exception the tongue and trapezius comments....how did he conclude it was bulbar palsy which he described as an upper motor neuron disease? His findins included "Motor finds are limited to the oral facial region with questionable proximal right upper extremty weakness. There is relative hyperreflexia in the upper and lower extremities with a right Hoffmann sign, but there are no definite fasciculations and no definite focal atrophy aside from possible the tongue. " Do you have any thoughts on how he came to that diagnosis based on what I have told you?
Also, I am completely confused as to the progression of bulbar palsy. My doctor said it differs from person to person, that my condition seems non-aggressive. He gave three options -- it could stay as is, it could progress further as a upper neuron disease or it could go into a lower neuron disease that leads to ALS. How will I know? What should I expect? But I have read articles that bulbar palsy prognosis is poor - 1-3 years. Can you help me understand what the options really are?
Thank you for any advice you can provide.
I guess I have two follow-up questions. I don't understand how he came to the conclusion of bulbar palsy. If the EMGs are normal with the exception the tongue and trapezius comments....how did he conclude it was bulbar palsy which he described as an upper motor neuron disease? His findins included "Motor finds are limited to the oral facial region with questionable proximal right upper extremty weakness. There is relative hyperreflexia in the upper and lower extremities with a right Hoffmann sign, but there are no definite fasciculations and no definite focal atrophy aside from possible the tongue. " Do you have any thoughts on how he came to that diagnosis based on what I have told you?
Also, I am completely confused as to the progression of bulbar palsy. My doctor said it differs from person to person, that my condition seems non-aggressive. He gave three options -- it could stay as is, it could progress further as a upper neuron disease or it could go into a lower neuron disease that leads to ALS. How will I know? What should I expect? But I have read articles that bulbar palsy prognosis is poor - 1-3 years. Can you help me understand what the options really are?
Thank you for any advice you can provide.
Brief Answer:
Outcome/rate of progression difficult to predict
Detailed Answer:
Hi Ms. XXXXXXX
Nice hearing back from you.
I fully agree with the three options, as mentioned by your doctor. The progression depends on the cause.
In your case, the fact that Bulbar Palsy- common to all the possible causes enumerated by me- being the ONLY neurological deficit present, poses a great challenge in coming to a conclusion.
Out of the three commonest possibilities enumerated by me, Myasthenia gravis has already been ruled out conclusively.
On the other hand, Syringobulbia can easily be ruled out/diagnosed by MRI scan of the spine.
Therefore, if Syringobulbia too is excluded, the only possibility is that of ALS.
Amyotrophic Lateral Sclerosis (ALS) is a very difficult disease to diagnose. To date, there is no one test or procedure to ultimately establish the diagnosis of ALS.
ALS has a variable progression : it may remain static with Bulbar Palsy as the only disorder; or, may progress gradually. The rate of progress varies from person to person.
May I inform you that the so-called prognosis of 3-5 years is outdated.
The current data shows that about twenty percent of people with ALS live five years or more and up to ten percent will survive more than ten years and five percent will live 20 years. There are people in whom ALS has stopped progressing and a small number of people in whom the symptoms of ALS reversed.
Hence, at this stage, it is impossible to predict the outcome or prognosis.
I am afraid that there is nothing that can be done at this stage. Perhaps, if at all, there is occurrence of a fresh symptom, it may give your Neurologist a valuable clue to the possible diagnosis and thereby, its prognosis.
I find myself unable to comment further.
Fond regards,
Dr. Rakesh Karanwal
Above answer was peer-reviewed by :
Dr. Chakravarthy Mazumdar
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