What is ALS

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.

ALS, is a fatal neurodegenerative disease the effects adults of all ages. It is closely related to Alzheimer’s Parkinson’s and Huntington’s.

ALS targets large brain cells residing in the brain and along the spinal cord called motor neurons. In ALS, as motor neurons get sick and die, a person progressively loses the ability to move: to walk, speak, swallow, and breathe. ALS is usually fatal within 2-5 years of diagnosis.

Facts You Should Know About ALS:

  • The onset of ALS is insidious with muscle weakness or stiffness as early symptoms.
  • Progression of weakness, wasting and paralysis of the muscles of the limbs and trunk as well as those that control vital functions such as speech, swallowing and later breathing generally follows.
  • It is estimated that ALS is responsible for nearly two deaths per hundred thousand population annually.
  • Approximately 5,600 people in the U.S. are diagnosed with ALS each year. The incidence of ALS is two per 100,000 people, and it is estimated that as many as 30,000 Americans may have the disease at any given time.
  • 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.
  • About twenty percent of people with ALS live five years or more and up to ten percent will survive more than ten years and five percent will live 20 years.
  • ALS occurs throughout the world with no racial, ethnic or socioeconomic boundaries.

In 1991 a team of ALS Association-funded researchers linked familial ALS to chromosome 21. In 1993 the research team identified a defective SOD1 gene on chromosome 21 as responsible for many cases of familial ALS. Further study indicated over 60 mutations (structural defects) in the SOD (superoxide dismutase) enzyme which alters the enzyme’s ability to protect against free radical damage to motor neurons. These studies open possibilities for future therapies or strategies to effectively mediate both familial and sporadic ALS. But much more research on the SOD enzyme is needed. Also, researchers have not ruled out other gene involvement (on other chromosomes) in ALS.