Amyotrophic Lateral Sclerosis (ALS): Introduction & Causes

Amyotrophic Lateral Sclerosis (ALS): Introduction & Causes

Amyotrophic Lateral Sclerosis (ALS) also known as Lou Gehrig’s disease has become more well-known to the general public over the past several years.  The famous ice bucket challenge has been used as a means to raise awareness and funding for further research of this devastating disease.

The disease was actually discovered by Dr.Jean-Martin Charcot in 1869, it became widely known when the famous baseball player Lou Gehrig developed the disease.  Since then, the disease has been commonly known as Lou Gehrig’s disease, but its formal medical name is Amyotrophic Lateral Sclerosis (ALS).

ALS is a progressive neurological disease that has devastating effects on those who are diagnosed. ALS (Lou Gehrig’s disease) affects the upper and lower motor neurons of the brain and spinal cord.  It is a disease that actually destroys the cells that comprise each of the nerves, and the results are devastating.

The condition begins with minor changes in normal functionality, including muscle weakness or muscle twitching.  As the nerve cells continue to die the disease becomes more progressive and the symptoms become worse.  As the disease progresses, the patient will begin to lose functionality of their muscles and be unable to move.  It also affects the patient’s ability to speak, and in later stages, breathe.

Causes:

There are two common types of ALS (Lou Gehrig’s disease).  Those types include: sporadic and familial. Sporadic means that the cause is unknown, but medical experts have concluded that this form is not inherited. Sporadic ALS is the most common form of ALS in the U.S.  It actually is responsible for more than 90% of ALS cases.  Familial ALS accounts for the other small 5 to 10% of patients.  Familial means that the disease is inherited. A specific gene mutation in an affected patient’s genome can be passed down to his/her children.  The gene mutation has a 50% probability of being passed down from parent to child.  

Through research, it has been identified that patients who have acquired this disease tend to have increased levels of Glutamate, resulting in a chemical imbalance within the body.  Glutamate acts as a messenger within the brain, cerebrospinal fluid (CSF) and nerve cells.  When there are increased levels of Glutamate within the Central Nervous System, it can actually be toxic to specific types of nerve cells.  The toxicity can lead to ALS.

Other research has indicated that some autoimmune like functions can result in ALS.  In individuals who have a disorganized immune system, the immune cells begin to attack themselves, resulting in nerve cell death. This type of dysfunctional immune system activity has been associated with the development of ALS in the patient population affected by this disease.

Finally, protein mishandling has also been associated with the development of ALS.  When there are dysfunctions present within the protein synthesis process, proteins can begin to accumulate within the cells.  Accumulation of these proteins can then result in nerve cell death; and that is how this process can be associated with ALS.

The entire process of how ALS is acquired is unknown.  It is known that there is both an inherited and non-inherited means by which this disease is transmitted.  Significantly more research must be conducted in order to better understand how patients are acquiring this disease.  It is through continued research that the different causes discussed above have been identified, and now appropriate measures can be implemented to prevent the disease from developing through these processes.