HSP and PLS are caused by degeneration of the upper motor neurons. “Upper motor neurons” are the nerve cells that control voluntary movement. They are located in the motor cortex of the brain and carry messages from the brain to the brain stem and spinal cord. They connect to (synapse with) other nerves called “lower motor neurons”, which carry the messages out to the muscles to tell them to move.
When there is degeneration in the upper motor neurons, messages (impulses) cannot adequately reach the lower motor neurons and their messages to the corresponding muscles are impaired. This causes the muscles to be weak and muscle tone to be increased, resulting in stiffness or spasticity.
In both HSP and PLS, nerve degeneration occurs in the lower part of the spinal cord, resulting in weakness and spasticity in the lower body. In PLS, degeneration also occurs at higher regions in the spinal cord and brainstem, causing weakness and spasticity in the arms, slurred speech and difficulty swallowing. This can also happen to a smaller degree in HSP.
Here are some diagrams to help:
In 2015 almost a dozen HSP Patient Advocacy Organizations from all over the world met in Madrid Spain to join forces to establish an international connection and resource to make sure a first rate, well established and impeccable HSP Patient Registry is set up. This will allow our next Clinical Trials to go forward smoothly and effectively. Good communication channels have also been established so as to allow for a smooth flow of information among scientists and organizations such as The Spastic Paraplegia Foundation.
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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