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What is Amyotrophic Lateral Sclerosis?
This condition is often called 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, we lose the ability to control the
muscles that enable us to move, speak, breathe, and swallow, and the muscles
gradually weaken and waste. ALS is also often associated with spasticity,
muscle spasms, and exaggeration of the reflexes. The majority of the
symptoms of ALS are directly related to muscle weakness and spasticity.
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Who Gets ALS?
ALS most frequently affects people between the ages of 40 to 60, affecting
men about 20% more than women. It is estimated that 5 to 7 people out of
100,000 are likely to have the disease. ALS is typically a rapidly
progressive, ultimately fatal condition. The five-year survival rate
following diagnosis is approximately 25%. In a small percentage of patients,
progression is slower and survival is much longer. Death is usually the
result of respiratory complications.
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What Causes ALS?
The cause of this disease is unknown. The most currently accepted theory is
that it is a combination of factors. The vast majority of ALS occurs
sporadically but ALS is inherited in about 10% of cases. No cure is
currently available for patients with ALS. Only one drug, riluzole, has FDA
approval for the treatment of ALS but it is minimally effective, prolonging
survival slightly but without improving muscle strength or function. The
primary goals of treatment are aimed at helping the patient cope with the
effects of the disease and at preventing or relieving complications. A great
deal of research is devoted to finding an effective treatment for the
disease.
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How is ALS Diagnosed?
The early diagnosis of ALS can be difficult, because many other conditions
may cause similar manifestations. An extensive evaluation is usually done to
exclude conditions that may be confused with ALS. When the disease is well
established the diagnosis is usually very apparent just by clinical
examination. There is no single specific, definitive test to diagnose ALS.
The 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 muscles and
may be performed several times over a period of months to monitor for
changes. The EMG can also help rule out other diagnoses.
Because of the rapidly progressive nature of the disease, ALS requires close
physician follow-up and appropriate interventions to help maintain quality
of life and prevent complications. Difficult decisions regarding nutritional
and respiratory support are inevitably necessary.
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