AMYOTROPHIC LATERAL SCLEROSIS (ALS) – ADULT

Aran-Duchenne; Gehrig's Disease; Lou Gehrig's Disease; Motor Neuron Disease

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.

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

Regardless of the part of the body first affected by the disease, muscle weakness and atrophy spread to other parts of the body as the disease progresses. Most individuals with ALS die from respiratory failure, usually within 3 to 5 years from the onset of symptoms.

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.

Suggested MER for Evaluation:

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  Clinical history and examination that describes the diagnostic features of the impairment;

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  Neurological findings consistent with the diagnosis of ALS; and

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  Results of any electrophysiological and neuroimaging testing.

Suggested Listings for Evaluation:

REMARKS

Amyotrophic lateral sclerosis.