ALS

The reason I chose to research this is once a year usually my work raises money and awareness for research of this disease. So I figured I could learn some information about it.

What is it?

Amyotrophic lateral sclerosis (ALS) is a progressive neurological disease that attacks motor neurons (nerve cells in the lateral brain and spinal cord that reach muscles all over body and are responsible for voluntary movement). The neurons are affected and degenerated until they eventually die. Basically, the brain cannot send messages to muscles in order for them to move. ALS is also referred to as Lou Gehrig's disease, named after a famous baseball player who died of the disease in 1941.

There are an estimated number of 30,000 cases of ALS in the U.S. at any given time. It does not affect some races and ethnicities over others, but it is much more common in males than females. People from the ages of 40 to 60 are most commonly affected, but there have been cases in people much younger than that. Most of the time there are no risk factors that can conclude whether a person will have ALS; it is very random. ALS can be inherited from parents in about 5 to 10 percent of cases. The familial cases usually are a result of one parent carrying the gene encoded for ALS.

Detection of Corticospinal Tract Compromise in Amyotrophic Lateral Sclerosis with Brain MR Imaging: Relevance of the T1-Weighted Spin-Echo Magnetization Transfer Contrast Sequence

What are some symptoms?

Symptoms include weakness of the limbs such as the arms, hands, legs, and feet. Twitching and cramping of the muscles is usually seen. Difficulty speaking (slurred speech) and swallowing can occur. In more advanced stages, difficulty breathing can result and a ventilator is needed to help keep the person alive. Symptoms can progressively get worse, leading to paralysis, and spread to other parts of the body with time.

How is it diagnosed?

There is no one exact test that can verify a person has ALS. A series of tests are given and the diagnosis results as other diseases are ruled out. Exams that the physician usually orders along with reviewing symptoms and signs are:

• EMG (electromyography)- detects electrical activity in muscles using fine wire electrodes

• NCV (nerve conduction velocity)- electrodes send a small shock to muscles to determine function; usually rules out other diseases

• MRI (magnetic resonance imaging)- uses radio waves and a strong magnetic field to visualize brain and spinal cord; used for detection of tumors and other abnormalities

• blood and urine tests
  
• muscle biopsy- removal of piece of muscle if muscle disease is suspected
  
• spinal tap- test the fluid surrounding brain and spinal cord

Detection of Corticospinal Tract Compromise in Amyotrophic Lateral Sclerosis with Brain MR Imaging: Relevance of the T1-Weighted Spin-Echo Magnetization Transfer Contrast Sequence

How is ALS treated?


Today, nothing has been found to reverse or cure ALS. Treatment focuses more on slowing down the stages of ALS and making patients more comfortable as they have to continue to deal with the disease. Physicians can prescribe drugs to reducepain, fatigue, twitching and cramping of muscles, and also for the relief of depression that can sometimes go along with the disease. Physical therapy and speech therapy are offered to help the person try to stay mobile and learn to adapt. Many times, ventilators are needed to inflate and deflate the lungs after advanced stages of ALS have set in and the muscles of the diaphragm are no longer to do so on their own.

There is one FDA approved drug that slows the disease's progression by reducing damage to the motor neurons. Riluzole (Rilutek) reduces the level of glutamate (a chemical messenger in the brain) in the body. The drug does not reverse the stages of ALS, but studies have shown that people who take the drug have longer survival rates by several months. It also prolongs the time before which a person would need to be placed on a ventilator.





References:

http://www.alsa.org/als/what.cfm?CFID=2685063&CFTOKEN=e844aa327d6427ab-87842C7C-188B-2E62-8090905205824399

http://www.ninds.nih.gov/disorders/amyotrophiclateralsclerosis/detail_amyotrophiclateralsclerosis.htm#126484842

http://www.mayoclinic.com/health/amyotrophic-lateral-sclerosis/DS00359/DSECTION=causes