AMYOTROPHIC LATERAL SCLEROSIS
(ALS or "Lou Gehrig's Discease")
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.
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.
SYMPTOMS OF
ALS AND HOW 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.
TREATMENT
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.
If you have
access to the internet, for further information, visit the following site:
Amyotrophic
Lateral Sclerosis
http://www.familyvillage.wisc.edu/lib_als.htm
or contact:
Amyotrophic
Lateral Sclerosis Association
21021
Ventura Boulevard
Suite 321
Woodland
Hills, CA 91364
(818)
340-7500 (Voice)
(800)
782-4747 (Voice)
(818)
340-2060 (Fax)