Amyotrophic Lateral Sclerosis

Proper Name: Amyotrophic Lateral Sclerosis

Common Name: ALS, Lou Gehrig's Disease

Age of Onset: ALS may occur from as early on as teenage years to the late 80s; however, the peak age at onset occurs from 55-75 years, with a mean age of 62 years at diagnosis.

Duration: Amotrophic Lateral Sclerosis is a terminal, progressive neurotransmitter disease that once diagnosed, since there is no cure, death is the final result. ALS affects people in many different ways, for example:

-50% of all victims live 3 years or more,

-25% live 5 years or more, and

-10% live up to 10 years or more

Males/Females/Equal: ALS is slightly more common in males than females, which is represented in this ratio of 1.5-2.0 males: 1 female. This ratio, however, diminishes with increasing age where the number of men and women suffering from ALS is nearly equivalent.

Particular Ethnic Group: Amyotrophic Lateral Sclerosis exhibits zero proven ethnic, racial prevalences on the mortality charts. ALS however according to ALS CARE Database shows that "93% of the victims were Caucisan". Although ALS has no patterned relationship to a singular, particular, more-affected ethnic group, according to the Mayo Clinic, "people living in Guam, West New Guinea and parts of Japan have an increased risk of developing ALS. Dietary factors may be to blame." There is some evidence that just by serving in the military, people have an increased chance of suffering from ALS.

Cause: The cause of ALS is yet unknown, but new technological advances allow the disease to be further researched and explained. ALS affects its victims in many ways: some patients experience a slow, deteriorating journey, while others may experience episodes of the disease and then go into remission. Possible causes of ALS include:

1. "Free radicals. The inherited form of ALS often involves a mutation in a gene responsible for producing a strong antioxidant enzyme that protects your cells from damage caused by free radicals — the byproducts of oxygen metabolism.


2. Glutamate. People who have ALS typically have higher than normal levels of glutamate, a chemical messenger in the brain, in their spinal fluid. Too much glutamate is known to be toxic to some nerve cells.


3. Autoimmune responses. Sometimes, a person's immune system begins attacking some of his or her body's own normal cells, and scientists have speculated that such antibodies may trigger the process that results in ALS."

Although the specific cause of Lou Gehrig's Disease is unknown, scientific observation in recent years has yielded a wealth of knowledge as far as the physiology of the disease goes. A few diseases that can evolve into ALS are:

- Progressive bulbar palsy

- Progressive Muscular Atrophy

- Primary Lateral Sclerosis

- Adult-onset Spinal Muscular Atrophy

Symptoms: Since ALS is a terminal, progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord; the disease exhibits early symptoms such as:

• increasing muscle weakness, especially involving the arms, legs, and ankles, leading to decreased movement
• slurring of speech
• trouble swallowing
• hand weakness
• clumsiness
• muscle twitching in arms, shoulders, and/or tongue
• muscle cramps
• difficulty lifting the front part of your foot and toes (footdrop)

As ALS progresses throughout the body, the patient is left paralyzed and often times loses the capability of chewing, swallowing, speaking and breathing.

Prognosis (progression of the disease): The progression of ALS begins with the motor neurons in the body deteriorating. These motor neurons make voluntary muscular movement possible as they branch from the brain to the spinal cord to the skeletal muscles, which allow us to walk and talk. When the motor neurons die, the ability of the brain to initiate, control, and send impulses for muscle movement is lost. Once the muscle fibers are no longer stimulated because of the dead motor neurons and the scarred, hardened (sclerosis) spinal cord, the muscle fibers begin to atrophy and become smaller because they no longer have any function. Loss of muscular movement leads to the more severe symptoms such as paralysis and inability to eat, breathe, and talk; even though the victim's intellectual ability remains intact throughout the disease. The ultimate fatality behind ALS triggers from the victim's inability to breathe any longer---respiratory failure.

Treatment Options (Rx): Since there is no cure to Amyotropic Lateral Sclerosis, the only treatment used is a medication called Rilutek, which slows the progression of ALS. Rilutek is in fact the first and only drug approved to treat ALS by the FDA, as it decreases the levels of glutamate in the brain.

Victims of ALS often times get the option of therapy to help decrease the effects of ALS in addition to the Rilutek in their prescribed medication.

• Physical therapy for low-impact exercises to maintain muscle strength and range of motion as long as possible, helps the victim preserve a sense of independence.
• Occupational therapy to help the victim be accustomed to a brace, walker or wheelchair and may be able to suggest devices such as ramps that make it easier for them to get around.
• Speech therapy to help teach techniques to make the victim's speech more clearly understood. Later in the disease, a speech therapist can recommend devices such as speech synthesizers and computers that may help the victim communicate.

Links:

• http://www.hyscience.com/Stephen%20Hawking.jpg
• http://emedicine.medscape.com/article/306543-overview
• http://www.alsa.org/
• http://www.neurologychannel.com/als/index.shtml
• http://www.ninds.nih.gov/disorders/amyotrophiclateralsclerosis/detail_amyotrophiclateralsclerosis.htm
• http://www.atsdr.cdc.gov/NEWS/alsreport.html
• http://www.mayoclinic.com/health/amyotrophic-lateral-sclerosis/DS00359