DESCRIPTION
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Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease, is a rapidly progressive, invariably fatal neurological
disease that attacks the nerve cells (neurons) responsible for controlling voluntary
muscles.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND
ICD-9-CM/ICD-10-CM
CODING
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Diagnostic testing: 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 such as electromyogram (EMG) and nerve conduction study (NCS) but these tests
may be negative or only suggestive of the diagnosis.
Physical findings: 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.
ICD-9: 335.20
ICD10:
G12.21
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TREATMENT
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There is no cure for ALS. However, the FDA has approved two drugs to treat ALS. These
are riluzole (Rilutek) and edaravone (Radicava) or (Radicut). 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. Radicava is an
intravenous infusion given by a health care professional. 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.
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* Adjudicators may, at their discretion, use the Medical Evidence of Record or the
listings suggested to evaluate the claim. However, the decision to allow or deny the
claim rests with the adjudicator.
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