Amyotrophic Lateral Sclerosis (ALS)

 

Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord.  It is also called Lou Gehrig's disease, after the baseball player who was diagnosed with it in the 1930s.  A French doctor named Jean-Martin Charcot discovered the condition in 1869.  A-myo-trophic comes from the Greek language.  "A" means no.  "Myo" refers to muscle, and "Trophic" means nourishment -- "No muscle nourishment."  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.  There are two different types of ALS, sporadic and familial.  Sporadic, which is the most common form of the disease in the U.S., accounts for 90 to 95 percent of all cases.  It may affect anyone, anywhere.  ALS usually strikes people between the ages of 40 and 70, and approximately 10,000-15,000 Americans can have the disease at any given time (although this number fluctuates).

 

Early symptoms of ALS usually include muscle weakness or stiffness.  Gradually all muscles under voluntary control are affected, and individuals lose their strength to speak, eat, move, and even breathe.

 

ALS is a progressive disease, which means it gets worse over time.  It affects nerves in your brain and spinal cord that control your muscles.

 

ALS is considered a motor neuron disease.  These nerve cells send messages from your brain to your spinal cord and then to your muscles.

 

You have two main types:

Upper Motor Neurons -- Nerve cells in the brain.

 

Lower Motor Neurons -- Nerve cells in the spinal cord.

 

ALS occurs as a result of the death of motor neurons.  Individuals in the later stages of the disease may become totally paralyzed, yet in most cases their minds remain sharp and alert.

 

ALS is not contagious.

 

In May 2017 Radicava was approved for the treatment of ALS.

 

Familial ALS:

Approximately 5-10% of ALS cases

Multiple family members are or have been diagnosed

There is a disease-causing gene

 

Sporadic ALS:

Approximately 90% of ALS cases

Only one person in your family is or has been diagnosed

Suspected interplay between "susceptibility" genes, exposure and aging