Re: Dysarthria and L'hermitte's, etc.

Posted By CCF MD mdf on March 30, 1998 at 12:02:11:

In Reply to: Dysarthria and L'hermitte's, etc. posted by Carl on March 27, 1998 at 15:57:18:






I confess to an overwhelming if variable fear of ALS, despite having been told by 4 neurologists that they don't think so.  Two EMGs with nerve conduction as well as needle studies have been negative.  These were done 6 and 14 months after symptoms were noticed.  Also a negative brain MRI.  Symptoms include fasciculations all over (but this is clouded by the fact that I have had fasciculations since age 20 and am now 55; it's just that they are much more frequent now).  Also weakness in the left hand (objectively verified by neurologist recently) and forearm (not biceps).  The major symptom, which has slowly progressed over the past 16 months, is weakness and partial paralysis around the left side of the mouth and then tongue.  It has begun to interfere with speech, although this varies with time of day (worse later) and from day to day.  I would also say that there has begun to be a slight problem with swallowing, maybe better to call it a lump in the throat.  Finally, there is the rather severe L'hermitte's sign: bending the neck, and many times even when I just breathe in, there is a shooting electrical sensation down the spine and into legs and arms.  Recently I had a viral infection for 3 days during which the l'Hermitte's was almost constant.  
My questions are 1. whether the l'Hermitte's rules out ALS?  2. Do the 2 negative EMGs (done by an EMG expert at a major university clinic, BTW) rule out ALS?  3. What should I do next to get a diagnosis?  4.  What are the possibilities as to what is going on with me.  Is MS likely given the symptoms, or is it probably spondylosis, or could it be a viral infection.  Or, my nemesis ALS?  I seek reassurance, but truth above all.
Carl
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I don't know if I can say for sure that Lhermitte's sign rules out ALS, but ALS is certainly NOT the first thing I'd think of as far as a diagnosis. Lhermitte's sign is a reflection of cervical spine irritation or dysfunction. Commonly it is seen in MS, though I've seen it also with vitamin B12 deficiency and with compressive lesions of the cervical spinal cord (spondylosis). In general, Lhermitte's sign is an indicator of CENTRAL nervous system dysfunction, and ALS is a mix of central and peripheral problems.
Fasciculations can be benign. The fact that you've had them for 35 years is an indication of that.
Weakness is important to investigate (so is Lhermitte's sign). It's not so easy to speculate where your problem is originating since you have combination of problems above the neck (mouth) and below (left hand). It is reassuring that the brain MRI is normal, but be sure that the scan has been examined closely. If you have not had MRI of the cervical spine, it seems a good idea. Bring this up with your doctor.
This information is provided for your general medical education only. Any specific comments regarding your diagnosis, prognosis, or treatment options must come from your doctor after careful interview, examination, and thoughtfully selected tests. Hope this helps. CCF MD mdf.