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Progressive reduction in speech and swallowing ability
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Progressive reduction in speech and swallowing ability

About a year ago we started to notice a slowing and slurring in the speech of my 75 year old mother.  Since that time her speech has continued to worsen to the point of severely limiting her ability to communicate.  Initially her primary care physician felt she had a stroke.  This has since been ruled out.  A neurologist specialist felt that she may have ALS and conducted a number of test, MRI, swallowing test, and an EMG.  All tests to date have not identified what would be causing these symptoms.  They have since ruled out ALS, Parkinson, myasthenia gravis, lyme disease and progressive bulbar palsy.  With time as the speech problem has continued to get worse it also started to affected her ability to swallow causing her to choke while eating and drinking.  Tongue strength is also greatly reduced.  All other muscles appear to be fine when checked by the specialist.  
At the moment they are out of ideas and we have been told to wait and see.  I
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Avatar_n_tn
First of all, keep in mind that I am unable to diagnose you because I am unable to examine you, this forum is for educational purposes.
   The symptoms and story that you have presented indicate that your mother is having progressive bulbar dysfunction.  The causes of this condition are very broad, but the most common are myasthenia gravis, bulbar ALS, polymyositis, thyroid disease, Chiari malformations, structural lesions and strokes of the brainstem and oclupharyngeal muscular dystrophy.  These conditions can be difficult to diagnose, and they are often not completely worked up.  I am not aware of what testing you had to rule out the conditions you mentioned, so I will mention some common pitfalls.  Myasthenia gravis (MG) is diagnosed with antibody tests (binding, blocking and modulating), but there is also seronegative MG.  EMG should also be conducted to evaluate for this condition, specifically a single fiber EMG of a facial muscle in your case.  ALS has specific features on EMG in the arms and legs, but may only involve the bulbar muscles.  In this case an EMG on the laryngeal muscles (cricothyroid muscle, tongue, etc) need to be evaluated, and are often missed.  I woudl recommend a muscle biopsy, thyroid studies and genetic tests for oculopharyngeal dystrophy if the above were negative.
I hope this has been helpful.
5 Comments
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Avatar_n_tn
Hi...I read your post. Although I do not know what is wrong with your mother, I read that Lyme disease has been ruled out.  Many people and most doctors don't know that you can have a flaming case of Lyme disease and still test negative.  It is ultimately a clinical diagnosis made by a Lyme specialist by way of history of symptoms and exclusion of other diseases.

I am being treated for neurological Lyme disease and I did have visible throat paralysis and was beginning to have problems with my tongue.  In the middle of a conversation, it would feel like it turned to stone for a few seconds and then go back to normal. I had episodes where food and drink actually came out my nose. Very bizarre.  My Lyme titers were negative.  Of course, I had many other symptoms and I don't know if your mother does or not.

I am not saying this is what is wrong with your mother but if you don't get any answers, you may want to revisit the issue.  I felt I had to pass this information along to you.

Good luck and I hope you soon get some answers.
Carol
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144586_tn?1284669764
Is she sunlight sensitive? Progressive deterioration suggests an intracerebral bleed, usually caused by simple vitamin k deficiency.
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Avatar_n_tn
How is your mom doing now? Any more info? We have a similar situation in our family. Thanks.
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Avatar_n_tn
Lately i've been having numbness and weakness in my left arm.  I haven't exactly experienced a loss in muscle strength in that arm, it just doesn't feel right.  I have been suffering through alot of stress and anxiety lately about other stuff, so i'm hoping it's that thats causing my symptoms.  Also, my arm feels like its burning and at other times my hands get really cold when the rest of my body is warm.  I'm scared I might have Ms, but mostly i'm scared about ALS.  If anyone can help me please, and let me know your opinion that would be awsome.
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