Amyotrophic Lateral Sclerosis
(ALS or "Lou Gehrig's Disease")
What is amyotrophic lateral sclerosis?
Amyotrophic lateral sclerosis (ALS), sometimes called Lou
Gehrig's disease, is a rapidly progressive, invariably fatal neurological disease
that attacks the nerve cells (neurons) responsible for controlling voluntary
muscles. The disease
belongs to a group of disorders known as motor neuron diseases,
which are characterized by the gradual degeneration and death of motor
neurons.
Motor neurons are nerve cells located in the brain, brainstem, and spinal
cord that serve as controlling units and vital communication links between the
nervous system and the voluntary muscles of the body. Messages from motor
neurons in the brain (called upper motor neurons) are transmitted to motor
neurons in the spinal cord (called lower motor neurons) and from them to
particular muscles. In ALS, both the upper motor neurons and the lower motor
neurons degenerate or die, ceasing to send messages to muscles. Unable to
function, the muscles gradually weaken, waste away (atrophy), and twitch
(fasciculations). Eventually, the ability of the brain to start and control
voluntary movement is lost.
ALS causes weakness with a wide range of disabilities
(see section titled "What are the symptoms?"). Eventually, all muscles under
voluntary control are affected, and patients lose their strength and the ability
to move their arms, legs, and body. When muscles in the diaphragm and chest wall fail, patients lose
the ability to breathe without ventilatory support. Most people with ALS die
from respiratory failure, usually within 3 to 5 years from the onset of
symptoms. However, about 10 percent of ALS patients survive for 10 or more
years.
Although the disease usually does not impair a person's
mind or intelligence, several recent studies suggest that some ALS patients may
have alterations in cognitive functions such as depression and problems with decision-making and
memory.
ALS does not affect a person's ability to see, smell, taste, hear, or
recognize touch. Patients usually maintain control of eye muscles and bladder
and bowel functions, although in the late stages of the disease most patients
will need help getting to and from the bathroom.
Next: Who gets ALS? »
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