Primary Lateral Sclerosis (PLS) is a group of rare, degenerative, neurological disorders. They are sporadic, meaning there is no clear familial link, although there are hereditary forms of PLS.
PLS is caused primarily by degeneration of the upper motor neurons in the brain and spinal cord, which results in increasing spasticity and weakness of voluntary muscles. It is often referred to as a benign variant of Amyotrophic Lateral Sclerosis (ALS, Lou Gehrig’s disease). The primary difference between the two is that in PLS the spinal motor neurons or lower motor neurons stay intact. Thus, there is no muscle wasting (amyotrophy), which is the symptom that ultimately causes fatal complications in ALS.
The disorder usually begins in the legs but can begin in the upper body or bulbar (speech and swallowing) muscles. The age of onset is generally between 35 and 66 years of age, with a median age of 50.
The incidence rate for PLS is difficult to determine. One study puts it at 500 individuals in the United States. However, many researchers feel this is inaccurate, and that the actual incidence rate is closer to 2000. The issue is further complicated by the fact that researchers also believe a good portion of those initially diagnosed with PLS may actually have ALS or HSP.
PLS was first identified in France in the 1850s by Dr. Jean-Martin Charcot. During his landmark analyses of ALS patients, Dr. Charcot encountered a woman with severe spasticity and limb contractures but no muscle wasting. After her death in 1856, the autopsy revealed extensive scarring in the lateral columns of her spinal columns, or primary lateral sclerosis.
Here’s an Overview Summary Chart.
Giving Tuesday was a great start on our year end goal. So far we have raised over $37,000. Our anonymous donor will match every donation dollar for dollar up to $175,000 until January 15, 2017. That's right, every donation you make will be doubled so you will receive twice as much satisfaction and double the number of people you will help with your wonderful and most appreciated generosity. This is 40% more than was matched last year so we are asking everyone to stretch your giving as well to make sure we use every nickel of this match. We can do it!
When Clinical Trials begin in 2017 it may require as many people as possible for the Clinical Trial to be effective. If you have HSP or PLS, please add your information to our data base so we can keep you informed. We are missing a lot people's email address, so if you just want to add your email address. just enter your name, address and email and we'll add it. That way, we will be able to reach you quickly at a lower cost. Your privacy is very important to us. We will never share any of your information with any individual or company without your permission.
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