ALS disease is an invariably fatal neurological disease that progresses from the muscles of the arms and legs to the mouth, tongue and throat as a patient begins to lose more and more control over his body. ALS can only be slowed with some medications, although research to find an effective treatment is ongoing.
What Is ALS?
ALS, or Amyotrophic lateral sclerosis, is known as a "progressive neurodegenerative disease," according to the ALS Association. Based on evidence, there appears to be a link between smoking and ALS. Another possible cause (although not definite) is a gene mutation called SOD1. Researchers do not know what role the mutation has in causing the disease to develop. This disease damages neurons in both the brain and spinal cord, leading to a hardening of the spinal cord. This damage causes the neurons to shrink and disappear, which means the muscles in the body stop receiving messages. Eventually, the muscles atrophy (become smaller and weaker). The better-known name for ALS is "Lou Gehrig's Disease," after the New York Yankees baseball player who developed the disease in 1939.
Symptoms
Initially, a person notices muscle weakness (in the legs, hands, arms or in the muscles that control swallowing, speech or breathing). Later, he notices that muscles that still work twitch (fasciculate) and cramp. Sometimes, this fasciculation is a precursor to the loss of function in those muscles. As the disease progresses further, a patient loses the ability to use his arms and legs. In addition, his speech becomes slurred and thick as he begins to lose control of his tongue and lip muscles. In later (more advanced) stages, he experiences shortness of breath and difficulty swallowing and breathing. The loss of ability to swallow can lead to choking episodes.
Who Are Its Patients?
The profile of an ALS patient indicates that he is between the ages of 40 and 70; average age at onset is 55. There have been cases where patients were in their 20s and 30s. ALS does not discriminate between men or women. Both genders develop the disease, although it occurs 20 percent more in men than in women. As members of both genders grow older, the occurrence becomes more equal.
Genetics
Heredity plays a role in a low percentage of families. Most patients diagnosed with ALS have no family history of the disease. These patients are said to have sporadic ALS (SALS); it is rare to have a familial connection (FALS), but it has happened. The incidence of multiple cases of ALS in one family can sometimes be "hidden" (if the patient was adopted or if his parents died at a young age). Most cases of ALS are not hereditary.
Various Forms
Currently, three forms of ALS have been classified. Sporadic is the most common form, accounting for 90 to 95 percent of all cases. The second form is Familial, which takes place more than once in a family's lineage (this is called a genetic dominant inheritance). This only occurs in approximately 5 to 10 percent of all cases. The third form is Guamanian. In the 1950s, a very high disease rate was noted in Guam and the Trust Territories in the Pacific.
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