Amyotrophic Lateral
Sclerosis
(ALS or "Lou Gehrig's Disease")
What is amyotrophic lateral sclerosis?
Amyotrophic lateral sclerosis (also called ALS or "Lou
Gehrig's
disease") is a classic motor neuron disease. Motor neuron
diseases
are progressive chronic diseases of the nerves that come from
the spinal
cord responsible for supplying electrical stimulation to the
muscles. This
stimulation is necessary for the movement of body parts.
ALS is progressive and fatal. The usual causes of death of
patients with
motor neuron diseases are not directly related to the disease,
but result
from simultaneous additional illnesses which ultimately occur
because of
weakness of the body. These illnesses are often infections.
ALS occurs most often in adults in the fifth through seventh
decades of
life. It progressively leads to death in 2 to 7 years. The
cause is
unknown.
What are the types of ALS?
There are 3 types of ALS: classic sporadic, familial, and the
Mariana
Island forms.
Classic ALS accounts for 90-95% of ALS patients in the U.S.
This form is
"sporadic" in that it cannot be traced to ancestors
with the
illness. The infrequent familial form is genetic, or inherited
and tends
to affect somewhat younger individuals (average 47 years of
age). The
Mariana Island form is a rare form of ALS described in Chamorro
indian
patients from Guam.
What are symptoms of ALS and how is it diagnosed?
All forms of ALS cause progressive muscle weakness and wasting.
Spontaneous tiny local areas of muscle twitching, called
fasciculations,
are characteristic in most patients. These may be sensed by the
patient as
muscle cramping. Lower extremity muscle wasting (atrophy) and
weakness
generally follows wasting of the arms, hands, and shoulders.
Spastic
muscles can be present.
Other muscles diseases are considered in the evaluation of
patients.
Blood testing and muscle electrical testing with
electromyography (EMG)
and nerve conduction velocities (NCV) can be helpful for a
diagnosis.
How is ALS treated?
Treatment measures in patients with motor neuron diseases are
largely
supportive, treating the complications, such as infections and
general
health. The treatment of ALS is also directed toward
suppressing the
immune inflammation felt to play a role in the degeneration of
the nervous
system of these patients.
While there is no cure for ALS or a proven therapy that will
prevent or
reverse the course of the disorder, the U.S. Food and Drug
Administration
(FDA) has approved riluzole (RILUTEK), the first drug that has been
shown to
prolong the survival of ALS patients. Patients may also receive
supportive
treatments that address the symptoms of the disease.
For further information, please visit the following site:
Amyotrophic Lateral Sclerosis Association
(http://www.alsa.org/)
ALSA National Office
27001 Agoura Road, Suite 150
Calabasas Hills, CA 91301
(818) 880-9007
Last Editorial Review: 4/16/2002
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