Lou Gehrig's Disease (ALS)

Joseph Scott

What is Lou Gehrig's Disease?

Lou Gehrig's Disease, or ALS (Amyotrophic lateral sclerosis) is a neurodegenerative disease that progressively affects nerve cells in the brain and the spinal cord. It causes damage to motor neurons that allow the brain to initiate control of muscle movement.
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Who Has it?

ALS usually affects those from ages 40-70. For unknown reasons, military veterans are twice as likely to fall victim to the disease. The above graph displays the prevalence of ALS per 100,000 in The United States. It is estimated that approximately 20,000 people will have the disease at a given time.

Symptoms

ALS can cause different symptoms in different people, and the time it takes for the affects to become apparent. Victims may experience fatigue, paralysis, and breathing problems. These symptoms vary in intensity in different people. For example, some people may need permanent ventilatory support, and some may have trouble walking. Others may experience muscle cramps and twitches, or uncontrollable instances of laughing or crying.

How is it Caught?

Motor neurons are cells that are vital for allowing the brain to control body movements. They help the brain send signals to muscles to allow them to function. The mutant protein, cooper-zinc superoxide dismutase, causes the motor neurons to become defective. It is caused by faulty genes, so it could be a substitution or frameshift mutation.


There are two types of ALS: sporadic and familial. Sporadic is 90-95% of all cases, and it can be caught by anyone. Familial, on the other hand, takes up only 5-10% of cases. Familial ALS is hereditary. Below is an image of a karyotype of someone with ALS. Familial ALS is most often a dominant trait.

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Diagnosis

There is no one test or procedure that can ultimately confirm the diagnosis of ALS. Some of the diagnostic procedures include electrodiagnostic tests (electomyography, nerve conduction velocity), studies of a patient's blood and urine (to search for thyroid and parathyroid hormone levels), spinal taps, X-rays (including MRI scans), a myelogram of the cervical spine, muscle and/or nerve biopsy, and a thorough neurological examination. These tests are conducted at the discretion of a physician, and each test may be based on results form a previous test.

Treatment

There is no cure or reversal for ALS, so treatment focuses on slowing the progress of symptoms and preventing complications. Many victims require a wide array of treatment from various doctors and physicians. Treatment commonly includes physical and mental therapy, allowing patients to be supported by other victims and attempt to regain control over their bodies. The only medication approved by the FDA is riluzole, which slows the disease in some people. Doctors may also prescribe medication for cramps, spasms, constipation, fatigue, and other side effects of ALS. There are certain organizations that push for certain method of treatment, usually including encouraging doctors to look for more ways to treat carriers of ALS, and more interactive treatment of victims of the disease. Overall, there is little major controversy surrounding the ethics of the treatment.

Bibliography

"Scientific Program of the AMERICAN ACADEMY OF NEUROLOGY."Neurology 21.4 (1971): 392. Web.


"Amyotrophic Lateral Sclerosis." (ALS) Treatments and Drugs. N.p., n.d. Web. 20 Mar. 2016.


"The ALS Association Greater New York Chapter | New York | New Jersey."The ALS Association Greater New York Chapter. N.p., n.d. Web. 20 Mar. 2016.