About ALS
ALS(Amyotrophic lateral sclerosis) is a progressive disorder of the nervous system which causes degeneration in both upper and lower motor neurons and results in muscle weakness.
Why is it known as Lou Gehrig's Disease?
Lou Gehrig, a famous baseball player in the U.S. during the 1930's, became afflicted with ALS. He was known as baseball's "Ironman". Strength, agility, excellent health - Lou Gehrig had everything it took to become a baseball legend. But Lou Gehrig had something else. At the peak of his career, he was diagnosed as having Amyotrophic Lateral Sclerosis (ALS). He died at the age of 38.
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 lead 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. Partial text taken from ALS Connecticut Chapter
More than 30,000 Americans have ALS, according to the ALS Association, a nonprofit organization that supports ALS research and public and patient education about the disease. Around 3,000 to 5,000 new cases of the disease are diagnosed every year.
Partial text taken from U.S. Food and Drug Administration
Onset -usually adulthood
Symptoms - generalized weakness and muscle wasting with cramps and muscle twitches common
Progression - ALS first affects legs, arms and/or throat and mouth muscles but ultimately affects all voluntary muscles, resulting in paralysis. Usually progresses rapidly with 3- to 5-year average survival.
Partial text from MDA DivisionSporadic ALS and Familial ALS
Sporadic ALS can strike any adult, anywhere in the world. ALS is not contagious. Ninety to 95% of all ALS is without any family history and is called Sporadic ALS. However, approximately 10% of all people who have ALS are found to have a family history of the disease, called Familial ALS (FALS). For a diagnosis of FALS, it must be identified clinically in another member of the family.4 FALS is inherited as an autosomal dominant trait, with a high penetrance after the sixth decade. An autosomal dominant inheritance pattern means that the gene flaw is on an autosome, a chromosome (DNA) that is not the sex (X or Y) chromosome, and a person needs to inherit a flawed gene from only one parent to cause the disease. About 15% to 20% of those with FALS have a mutation in the Cu/Zn superoxide dismutase (SOD1) gene on chromosome 21.5
Partial text from ALS NetworkFacts You Should Know About ALS
Although the life expectancy of an ALS patient averages about two to five years from the time of diagnosis, this disease is variable and many people live with quality for five years and more. More than half of all patients live more than three years after diagnosis.
Approximately 5,600 people in the U.S.are diagnosed with ALS each year. The incidence of ALS (two per 100,000 people) is five times higher than Huntington's disease and about equal to multiple sclerosis. It is estimated that as many as 30,000 Americans may have the disease at any given time.
Partial text from the ALS Association