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Lou Gehrig's Disease (ALS)
The etiology and pathogenesis of amyotrophic lateral sclerosis (ALS) are not known, although a number
of hypotheses have been advanced. One hypothesis is that motor neurons, made vulnerable through
either genetic predisposition or environmental factors, are injured by glutamate. In some cases of familial
ALS the enzyme superoxide dismutase has been found to be defective
Amyotrophic lateral sclerosis (ALS), often referred to as "Lou Gehrig's Disease," is a progressive
neurodegenerative disease that affects nerve cells in the brain and the spinal cord. Motor neurons reach
from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. The
progressive degeneration of the motor neurons in ALS eventually leads to their death. When the motor
neurons die, the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle
action progressively affected, patients in the later stages of the disease may become totally paralyzed.
The physician bases medication decisions on on the patient's symptoms and the stage of the disease.
Some medications used for ALS patients include:
Riluzole — This is the only medication approved by the U.S. Food and Drug Administration
(FDA) to slow the progress of ALS. Though it does not reverse ALS, riluzole has been shown to
reduce nerve damage. Riluzole may extend the time before a patient needs a ventilator (a
machine to help breathe) and may prolong the patient's life by several months.
The mode of action of RILUTEK is unknown. Its pharmacological properties include the following,
some of which may be related to its effect: 1) an inhibitory effect on glutamate release, 2)
inactivation of voltage-dependent sodium channels, and 3) ability to interfere with intracellular
events that follow transmitter binding at excitatory amino acid receptors. Riluzole has also been
shown, in a single study, to delay median time to death in a transgenic mouse model of ALS.
These mice express human superoxide dismutase bearing one of the mutations found in one of
the familial forms of human ALS.
Baclofen or diazepam — These medications may be used to control muscle spasms, stiffness
or tightening (spasticity) that interfere with daily activities.
Trihexyphenidyl or amitriptyline — These medications may help patients who have excess
saliva or secretions, and emotional changes