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Re: Primary Lateral Sclerosis

Posted By Barrie Mulkern on September 23, 1997 at 21:09:43:

In Reply to: Re: Primary Lateral Sclerosis posted by ccf neuro M.D.* on August 18, 1997 at 19:03:59:

: : I am a 5 ft-7 inch, 180 lb, 63 year old male, born in Holland in 1934 and emigrated to Canada in 1953.
My appendix was removed in 1952 and my gallbladder in 1987.
I have been diagnosed with Primary Lateral Sclerosis, but let me backup a little. On October 5th, 1994 I had heart surgery (6 bypasses). 6 Months after my surgery I went back to playing Badminton (which I have played for thirty years at a competitive level) I noticed that I wasn't moving as quick as before and that I couldnt tap my right foot, only when I tried really hard I could move it a little.
I went to my doctor and asked to see a Neurologist, he ordered a Myelogram which was normal, then a CT which showed that I had a Pituitary Tumor, further tests revealed that is was a non secreting adenoma. In the mean time my right leg got worse and I started to get cramps specially at night, and swelling of my legs and feet. My legs are really spastic and I need two canes to walk.
Many more test were done including CTs, MRIs nerve conduction tests etc, all were deemed as normal (except for the tumor of course) They say that there is no way that this tumor could be the cause of the PLS.
The following is the list of drugs I am on at the moment;
- Acetylsalicylic Acid 325mg daily
: : - Accupril 10 mg (I have a blood pressure of 140/80)
: : - Baclofen 10 mg (twice a day to control Spasticity, it doesnt seem to do
  much good)
: : - Pravachol 10 mg
: : - Swelling of feet (left leg had vein removed for bypasses, so it was swollen
        already) I am now using compression stockings, which help a lot to reduce the
: : - Right foot drop
: : - Muscle cramps in my calfs at night
: : - Muscle tightness in my legs, specially right one
: : - Slightly high sugar level of 125
: : - Head aches
: : - Hypertension
: : - Dry skin
: : - Rosacea (minor)
: : - Warts on hands, and other areas of the body
In PLS is the myelin distroyed or the muscles?

: : I am willing to setup a data base to see if there is a common thread and even setup a website if there is enough interest.
Thank you in advance, for any suggestions.
: : Regards
: : Frank Reyerse
Primary lateral sclerosis is an extremely rare but well recognized disorder of the nervous system in which the lateral columns of the spinal cord undergo an unexplained or "primary" degeneration or "sclerosis", hence the origin of the name. Contained in these so-called lateral columns of the spinal cord are nerve tracts known as the lateral corticospinal tracts, which are nerve endings that come all the way from the surface, or cortex, of the brain, to connect with , or synapse upon, other nerve cells whose main cell bodies are located in the spinal cord. It is these second nerve cells in the spinal cord that actually connect to the muscles of the body, and when they are destroyed, along with the fibers that degenerate in primary lateral sclerosis, you get a much more commonly known disorder caleed AMYOTROPHIC lateral aclerosis, or ALS (Lou Gehrig's disease). The "amyotrophic" means "without muscle" since in that disease, the muscle cells waste away due to the nerve fibers that normally constantly send them signals being destroyed by the disease. This same process occurs in polio. In primary lateral sclerosis, these cells stay alive and well, and it is the brain's fibers connecting to them only that are affected by disease. They lose their myelin or insulation coating, and the net result is that the spinal cord cells they would normally be connecting to and influencing become overactive, since the brain's influence is usually a regulatory  or negative one, Hence, spasticity or stiffness and hyperactive contraction of the muscles (which remain alive and well) occurs. This can unfortunately be very disabling and painful if it becomes severe. Other symptoms can include a loss of coordination, especially for things like handwriting and shirt-buttoning, as the brain also sends down signals that help refine movements ands make them precise. Unfortunately, there is no treatment or cure for the primary disease process itself, save for the rare instance when it is secondary to an unrecognized vitamin B12 deficiency (in which case, of course, it is not true primary lateral sclerosis). Various medications, however, can be quite effective in limiting and ameliorating some of the symptoms it causes. For spasticity, in addition to benzodiazepine-type drugs such as Valium (diazepam) or Klonapin (clonazepam), baclofen (Lioresal), there is a new, sometimes dramatically effective medication known as tizanidine (Zanaflex), used to treat spasticity mostly in multiple sclerosis patients who often get disease in these same lateral corticospinal tracts (in addition to many other tracts, hence its name "multiple" sclerosis). I would suggest you're inquiring about this drug, especially if your walking is becoming more difficult due to the spasticity. A second opinion from a nearby major medical center may also be very worth pursuing in your particular case, again, because true primary lateral sclerosis is exceptionally rare. If you would be interested in being evaluated at the Cleveland Clinic specifically, the neurology department's appointment number is 216-444-5559 or 1-800-223-2273 extension 45559. Information provided in the Neurology Forum is intended for general medical informational purposes only. Actual diagnosis and management of your specific medical condition should be strictly in conjunction with you treating physician(s).  We hope you find this information interesting and useful.
Dear Frank,  my name is Barrie Mulkern and I have been diagnosed with Primary Lateral Sclerosis.  As this disease is so rare - even my Neurologist at a top medical center in Boston - has only seen a handful of cases in his career, and not one exactly like mine.  I started having slowness in my right arm at first, then my entire body became rigid, and now I've lost most of the ability to use my left arm and I need assistance walking at all times.  I have been looking for someone with anything similar to what I have for about a year now.  I would enjoy corresponding to you and others on a database or posting info that will help anyone on a website.  I am currently sporting an intrathecal baclofen pump which distributes about 450 mcg to my spinal column in a 24 hour period.  This is really the only type of medication that seems to help the spasticity - it is very severe - but pain is relieved from baclofen pump.  
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