Phoenix Neurological Associates,
Ltd.
5090 N. 40th Street
Suite 250
Phoenix, AZ 85018
Phone: (602) 258-3354
Fax: (602) 258-3368
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January 2013: New publications by our physicians and staff. Read more
ALS Symptoms | ALS Diagnosis | Causes of ALS | ALS Treatment | ALS Cure | ALS Research | ALS Resources
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.
A-myo-trophic comes from the Greek language. "A" means no or negative. "Myo" refers to muscle, and "Trophic" means nourishment–"No muscle nourishment." When a muscle has no nourishment, it "atrophies" or wastes away. "Lateral" identifies the areas in a person's spinal cord where portions of the nerve cells that signal and control the muscles are located. As this area degenerates it leads to scarring or hardening ("sclerosis") in the region.
As motor neurons degenerate, they can no longer send impulses to the muscle fibers that normally result in muscle movement. Early symptoms of ALS often include increasing muscle weakness, especially involving the arms and legs, speech, swallowing or breathing. When muscles no longer receive the messages from the motor neurons that they require to function, the muscles begin to atrophy (become smaller). Limbs begin to look "thinner" as muscle tissue atrophies.
The diagnosis of Amyotrophic Lateral Sclerosis or Lou Gerhig's Disease is a devastating moment. The disease is universally fatal and at present there is no known cure. However many patients receive the diagnosis from physicians who also feel that they have nothing to offer patients. At Phoenix Neurological Associates we recognize a few important facts about ALS. First of all, it is vital to be sure of the diagnosis and to be sure that all other possible diseases have been excluded. This requires physicians with years of experience in neuromusculaar diseases. Secondly, once the diagnosis is reached we believe that this is the beginning of our care for the patient, not the end. We use a multidisciplinary approach to help patients with ALS remain as independent and active for as long as possible. We are proud to be one of only 33 certified ALS clinics in the country.
This condition is often abbreviated as ALS, but is also known as Lou Gehrig's disease, named after the famous baseball player who died of the disease. ALS is a motor neuron disease that destroys the nerve cells responsible for carrying signals from the brain to the muscles. When these cells die, one loses the ability to control the muscles that enable us to move, speak, breathe, and swallow, and the muscles gradually weaken and waste. The majority of the symptoms of ALS are directly related to muscle weakness. This weakness can begin in any part of the body and progresses very slowly. There are often muscle twitches, called fasciculations, and muscle cramps. Numbness, tingling, and pain are not common. The disease does not usually affect the nerves in the brain, so that one’s thinking is generally not affected.
The diagnosis of ALS is one of exclusion because at the onset ALS may mimic other disorders like strokes, carpal tunnel syndrome, or other neurological conditions. There is no specific, definitive test to diagnose ALS. Thus a physician will typically perform many different tests to rule out other diseases, including magnetic resonance imaging (MRI) of the brain and spinal cord, electrodiagnostic studies, including needle electromyography (EMG) and nerve conduction studies (NCS), along with a variety of blood and urine tests. A diagnosis of ALS is reached after evaluating the results of all the tests, along with the patient's medical history, and physician's physical examination.
The EMG is the most important test in this work-up because it gives the physician accurate information about what is happening within the nerves. The needle EMG results can also be used to rule out others diagnoses. Once the diagnosis of ALS is made, it is appropriate to get a confirmatory second opinion given the gravity of this disease.
There is no known cause for ALS. Researchers have looked into theories including environmental exposure and a variety of infections and have not yet discovered the source for the disease. In 10% of patients with ALS there is a family history of the disease. Approximately 20% of these familial cases of ALS have a specific gene, called Superoxide Dismutase 1 which is abnormal. This gene can be tested for through a blood test. Otherwise there is no other specific genetic relationship for the majority of the cases of ALS.
There is one FDA approved drug to treat ALS, Rilutek. This drug was shown to increase survival of patients by approximately three months. Other than Rilutek there are no known drugs or supplements that have been proven to increase survival.
The majority of the treatment for patients with ALS is focused on managing a patient’s symptoms. We believe this is best accomplished through a multidisciplinary clinic (Link to clinic) which brings together a variety of specialized therapists, such as physical therapy, occupational therapy, speech therapy, and respiratory therapy. We have established this type of a clinic at Good Samaritan Hospital in Phoenix, Arizona. Here we can manage symptoms that arise by providing specific equipment to compensate for the weakness that develops. Trials have shown that patients followed in these multidisciplinary clinics live 6-12 months longer.
As there is no known cause for the disease we do not have a cure yet. Once the disease begins the average life expectancy is 3-5 years. Patients pass away from an inability to swallow effectively or breathe effectively. At present there are numerous research trials looking to explore avenues to find cures. Much of this research focuses on a concept known as excitotoxicity. This theory implies that certain levels of chemicals that excite muscles and nerves are necessary to be able to move. However too much of these chemicals causes nerve death. Unfortunately there is still a great deal of work that needs to be done before we will have a cure for this disease.
ALS Clinical Trials