Amyotrophic Lateral Sclerosis (ALS)
What is amyotrophic lateral sclerosis (ALS)?
ALS is a disease that causes gradual loss of control and
weakness of muscles. The mind is usually not affected,
despite worsening weakness of the body.
ALS is also called Lou Gehrig's disease, after a well-known
baseball player who died of ALS. It is rare. About 1 new
case per 100,000 people is diagnosed each year. Symptoms
most often appear in people who are between 40 and 70 years
old. The symptoms worsen more rapidly in some people than
in others, eventually leading to death.
How does it occur?
ALS affects the nerves in your brain and spinal cord that
control your muscles. These nerves break down and
disappear. Your muscles then become weak and waste away
because the nerves that stimulated them are gone.
The cause of ALS is not known. It seems to run in some
families. Also, some ethnic groups, such as Pacific
Islanders, appear to be at greater risk. However, ALS most
often occurs in people who have no family history of the
disease.
What are the symptoms?
The symptoms are similar to other chronic (long-lasting)
conditions affecting the central nervous system (brain and
spinal cord). You may have:
- muscle cramps and twitching
- weakness in your hands and painless difficulty with small
tasks, such as fastening buttons, turning a key, or
opening a door
- weakness in your feet and ankles, causing your feet to
drag when you walk (called foot drop)
- stiffness in your arms and legs
- slurring or slowing of speech
- difficulty swallowing
- decreased ability to make facial expressions
- fatigue
- weight loss.
The disease begins slowly, most often affecting just one
limb, such as a hand, at first. As time passes, more limbs
and muscle areas become affected.
As your disability increases, you become more dependent on
others. Eventually you may be able to move only your eyes.
To others it may look like you are in a coma, but you
usually remain fully conscious and aware of your
surroundings. You are still able to see, hear, taste,
smell, and recognize touch. Bowel and bladder control and
function are usually not affected. You may become too weak
to breathe without the help of a ventilator. Respiratory
failure, pneumonia, or blood clots in the lungs may cause
death.
How is it diagnosed?
There is no specific test for ALS. The doctor examines
you, takes your medical history, and orders tests of your
brain and nervous system. An electromyogram (EMG) may be
done to test your muscles and nerves. Other tests done to
rule out other diseases may include a computed tomography
(CT) scan, magnetic resonance imaging (MRI) scan, muscle and
nerve biopsies, and blood tests.
How is it treated?
There is no cure for this disease. Treatment includes
relieving symptoms and providing supportive care.
Researchers are actively developing and testing new drugs
that will improve the treatment of ALS.
Emotional support from friends and family is very
important in helping you cope with this disease.
Early in the course of the disease, try to remain moderately
active. Physical therapy can help keep muscles active and
delay weakness. You may need braces or splints to support
weakened ankles or wrists.
As the disease progresses, you may need to stay in a nursing
home if you cannot be cared for at home.
Discuss with your family, friends, and health care providers
how much should be done to maintain your life during
advanced stages of the disease. What medical measures do
you want to be taken at various stages of the disease? Do
you want feeding tubes and ventilators to be used? Many
people fear becoming unable to move, eat, or communicate at
the end of life and may choose to limit life-sustaining
treatments. These are difficult decisions. You and your
family may want the help of clergy or other counselors to
help you with these discussions.
What can I do to help myself?
Take care of yourself. For example:
| 
