what is ALS?
Amyotrophic lateral sclerosis (ALS), often referred to as “Lou Gehrig’s Disease,” is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. The progressive degeneration of the motor neurons in ALS eventually leads to their death. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively affected, patients in the later stages of the disease may become totally paralyzed. When a muscle has no nourishment, it “atrophies” or wastes away. “Lateral” identifies the areas in a person’s spinal cord where portions of the nerve cells that signal and control the muscles are located. As this area degenerates it leads to scarring or hardening (“sclerosis”) in the region.
As motor neurons degenerate, they can no longer send impulses to the muscle fibers that normally result in muscle movement. Early symptoms of ALS often include increasing muscle weakness, especially involving the arms and legs, speech, swallowing or breathing. When muscles no longer receive the messages from the motor neurons that they require to function, the muscles begin to atrophy (become smaller). Limbs begin to look “thinner” as muscle tissue atrophies.
Types of ALS:
Sporadic: the most common form of ALS in the United States – 90 to 95% of all cases.
Familial: occurring more than once in a family lineage (genetic dominant inheritance) accounts for a very small number of cases in the United States: 5-10% of cases
Guamanian: an extremely high incidence of ALS was observed in Guam and the Trust Territories of the Pacific in the 1950′s.
The most common form of ALS in the United States is “sporadic” ALS. It may affect anyone, anywhere. “Familial” ALS (FALS) means the disease is inherited. Only about 5 to 10% of all ALS patients appear to have genetic or inherited form of ALS. In those families, there is a 50% chance each offspring will inherit the gene mutation and may develop the disease.
Statistics about ALS:
- There are currently 30,000 Americans living with ALS.
- One person every 90 minutes is diagnosed with ALS.
- The average life expectancy of an ALS patient is 2-5 years upon diagnosis.
- It has been more than 70 years since Lou Gehrig gave his famous farewell speech at Yankee stadium and there is still no cure for ALS.
- ALS knows no racial, ethnic or socioeconomic boundaries