Where does Lou Gehrig's Disease come from?

This week we explored the pathways leading to neuron cell death which can lead to various diseases including Parkinson’s disease, Alzheimer’s disease, and Amyotrophic Lateral Sclerosis (ALS) in particular.  ALS or Lou Gehrig’s disease is a disease in which there is a loss of motor neuron function. Motor neurons allow for voluntary muscle control. Because of cell death of motor neurons, ALS patients lose normal motor skills such as walking, writing, swallowing, which progresses to eventual paralysis and death (usually 3-5 years after diagnosis). ALS affects around 30,000 people in the United States alone. In this blog I’m going to delve into the pathway leading to ALS and try to determine what actually causes the disease and how its progression can be slowed.

 

Image from: http://www.bioresearchonline.com/article.mvc/Neurons-From-ALS-Patients-Skin-Cells-0001

What exactly causes ALS?
Well, the truth is we do not know exactly what triggers ALS. Research is still being conducted to determine the exact cause of ALS. Only about 10% of cases are passed down genetically within families, so the other 90% of cases are still a mystery as to why ALS occurs. So far researchers have two leads to this rapid motor neuron destruction—both are linked with gene mutations leading to abnormal proteins. The first mutation is in the gene of a protein called ubiquilin-2 which degrades old and abnormal proteins to make room for new ones. When it is not working properly ubiquilin-2 loses its normal function and abnormal proteins build up in the body. Recently a second relation to ubiquilin-2 was discovered as well. Ubiquilin-2 protein itself, can also build-up specifically in the spinal cord without a mutation in the ubiquilin-2 gene of patients with ALS, possibly aiding in the progression of motor neuron loss. This is an important discovery because the ubiquilin-2 protein is increasing the possibility of cell death, even if there is no mutation or abnormality occurring in the protein.
A second mutation is in superoxide dismutase (SOD1) which is a protein that helps in decreasing oxidative stress to neuronal cells. Oxidative stress can lead to the death of motor neurons (apoptosis) that are still healthy. There are over 150 different genetic mutations in SOD1 that have been linked to ALS—some are more common than others.  All tend to lead to the same outcome–loss on motor neurons, resulting in ALS.
 
How can the progression of ALS be delayed?
Unfortunately, this devastating disease has no cure. Scientists are working diligently to figure out a cause and cure. Right now there is a drug called Riluzole which slows the progression of ALS.  The exact mechanism of how the drug works is unknown, however researchers do know that Riluzole helps keep levels of glutamate down. Glutamate-a natural chemical in the body that helps control motor neurons- builds up in ALS patients. At high levels in the body, glutamate can become neurotoxic and harm neural cells. The Riluzole protects these motor neurons by preventing overexposure to glutamate, which helps extend the lives of ALS patients. ALS patients can also take anti-spasticity drugs that help keep muscles loose and relaxed. These include baclofen and diazepam. These drugs do not slow the progression; they only make the patients more comfortable by treating ALS symptoms. More research is in progress to find a cure and hopefully we will find one, and wipe out Lou Gehrig’s disease.

1 Comment

  1. What is called als, or more generally motor neuron disease, can be seen as cascading neurodegeneration which is a trigger response cycle.
    In order for a self perpetuating trigger response cycle to occur, we need triggers, and a certain type of response.
    In the case of als, the trigger can be physical trauma, emotional trauma, some combination of the two or something else.
    Common triggers include, among others, substances in food such as pesticides, head trauma, spinal anesthesia, blood pressure medication, vaccines and infections, such as with Borrelia burgdorferi,the spirohete associated with what is called Lyme disease. Persistent or acute life stress can also be seen as triggers.
    The response is cellular freaking out and disconnection. This is a form of microbehavior.
    So for instance, a person undergoing regular vaccinations may trigger inflammation of the nervous system, which develops into a self perpetuating freak out and disconnect response. In other word, the response of the neurons to the vaccine triggers further response and this triggers further response.
    So while the initial trigger may be over, the response becomes the new trigger and keeps it all going.
    To understand how this works one could start by looking into the description of the action of neurons and astrocytes that Jeffrey Rothstein describes in his work.
    To further understand how emotional stress is connected with degeneration and chronic health issues, one could review my work and the work of Gabor Mate, among others.
    So how is so called als then solved or prevented?
    Given that it is a trigger response cycle, the answer is to reduce triggers and change cellular responses.
    Trigger reduction includes improving diet, relieving life stress, reducing medication and vaccine use and somehow mitigating problems involving infection.
    Changing cellular response is done using mind body methods such as mindfulness meditation, micro matches macro methods and hypnotherapy.
    In addition, one can improve cellular response by improving diet.
    Through comprehensive application of trigger reduction and response adjustment, one can prevent the occurance of motor neuron disease and if it is already occuring stabilize and improve the condition of the person experiencing it.
    Examples of people who have publicly discussed their success in using this type of approach to prevent and solve motor neuron disease include, among others, Craig Oster, Steven Shackel and Theresa Lee.
    The use of mindfulness meditation and stress management counseling to reduce inflammation and stabilize and improve the health of people experiencing various forms of disease is widely discussed and has been the subject of numerous studies.
    Also, I discuss all of this in a more comprehensive and detailed way in my work.

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