Amyotrophic Lateral Sclerosis

There are many types of diseases that attack the muscles, and doctors are still discovering new ones each day. This year there has been a wide spread of different challenges going around. One of the challenges was the ALS Ice Bucket Challenge, this challenged spreaded awareness of the muscle disease Amyotrophic Lateral Sclerosis (ALS). ALS has often been referred to as Lou Gehrig’s Disease. It is a progressive disease that affects the nerves in the brain and the spinal cord.

The name of this muscle disease may sound really hard to understand, but really it is quite simple. Amyotrophic can be broken down into three parts and looked at separately. “A” means no, “myo” means muscle and “trophic” means nourishment. When these three are put together it means no muscle nourishment. When a muscle has no nourishment, it begins to waste away and become weak. The lateral in the name is stating the position of where the disease is located on the spine. Since it is later this would be the disease is found on the outer sides of the spinal cord. The sclerosis part of the name means the hardening of the muscle in that area.

In the body there are motor neurons; which are located in the brain, brain stem, and spinal cord. These motor neurons are control units and communication links between the nervous system and the voluntary muscles in the body. The motor neurons in the brain send messages that are transmitted to motor neurons in the spinal cord and then from there to a particular muscle. In Lou Gehrig’s Disease, the neurons in the brain and spinal cord die and stop sending messages to muscles. This causes the muscles to weaken and have fine twitches. Eventually the brain is unable to control the voluntary movements in the body.

There are three different forms of ALS; there’s sporadic, familial, and guamanian. Sporadic is the most common form of ALS found in the United States. It can affect anyone, anywhere in the US. Up to 95% of all ALS cases are in the form of sporadic. While familial can only been passed by genetics. There are a very small amount of cases in the US where ALS has been passed down genetically, 5 to 10%. With families who have passed down the genetic mutation of ALS, there is only a 50% chance that each offspring will inherit the mutation or develop the disease. The last form, guamanian, is an extremely high incidence of ALS was found and observed in Guam and the Trust Territories of the Pacific in the 1950’s.

There are always signs and symptoms to any disease; ways that the doctor will know what type of disease a person has. This allows for quick identification so the patient can get the treatment for the disease as soon as possible (if there is one available). The signs of ALS are; persistent weakness in a limb, and the inability to speak or swallow. In more advanced stages of ALS there could be shortness of breath and difficulty breathing or swallowing. Each person’s early symptoms are different. One person might have trouble with their legs, another with their speech, and another with their arms.

There is no real way to diagnose ALS, doctors usually do multiple test to rule out other diseases/disorder. The reason they do this is because there are other diseases that mimic ALS and the doctors do not want to misdiagnose. One of the type of test doctors use is electrodiagnostic test; these include electromyography (EMG) and nerve conduction velocity (NCV). Doctors also use; blood and urine test, spinal tap, x-rays, and magnetic resonance imaging (MRI), just to name a few. Patients who believe they have ALS are asked to seek a second opinion from an ALS ‘expert’.

As of today there is still no known cure for ALS, but there have been developments in the past 20 years that could possibly diminish the severity of the symptoms. The FDA has approved of the first drug treatment of riluzole (Rilutek) in 1995. This drug has been deemed to reduce damage to motor neurons by decreasing the release of glutamate. Glutamate is found in glutamic acid and is short for monosodium glutamate. There have been trials with ALS patients that show that riluzole extend survival by several months. This is mainly with patients who have trouble swallowing.