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. 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, and twitch. Eventually the ability of the brain to start
and control voluntary movement is lost. Individuals with ALS lose their strength and
the ability to move their arms, legs, and body. When muscles in the diaphragm and
chest wall fail, individuals lose the ability to breathe without ventilatory support.
The disease does not affect a person's ability to see, smell, taste, hear, or recognize
touch, and it does not usually impair a person's thinking or other cognitive abilities.
However, several recent studies suggest that a small percentage of patients may experience
problems with memory or decision-making, and there is growing evidence that some may
even develop a form of dementia. The cause of ALS is not known, and scientists do
not yet know why ALS strikes some people and not others.
DIAGNOSTIC TESTING AND CODING
There is no one test or procedure to establish the diagnosis of ALS. The diagnosis
of ALS is based on history, neurological findings consistent with the diagnosis of
ALS and eletrophysiological and neuroimaging testing to rule out other impairments
that may cause similar signs and symptoms. The diagnosis may be supported by electrophysiological
studies (electromyogram [EMG] and nerve conduction study [NCS] but these tests may
be negative or only suggestive of the diagnosis.
No cure has yet been found for ALS. However, the FDA has approved the first drug treatment
for the disease-riluzole (Rilutek). Clinical trials with ALS patients showed that
riluzole prolongs survival by several months. Riluzole does not reverse the damage
already done to motor neurons, and patients taking the drug must be monitored for
liver damage and other possible side effects. However, this first disease-specific
therapy offers hope that the progression of ALS may one day be slowed by new medications
or combinations of drugs. Other treatments for ALS are designed to relieve symptoms
and improve the quality of life for patients. Multidisciplinary teams of health care
professionals can design an individualized plan of medical and physical therapy and
provide special equipment aimed at keeping patients as mobile and comfortable as possible.
Physicians can prescribe medications to help reduce fatigue, ease muscle cramps, control
spasticity, and reduce excess saliva and phlegm. Drugs also are available to help
patients with pain, depression, sleep disturbances, and constipation.