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Depending in which part of the world someone is located, ALS is known by various other names. In some parts of Europe, it is referred to as Charcot's disease after the physician who first wrote detailed notes about it. In the United Kingdom, a physician might say you have motor neuron disease. (Even though it is called motor neuron disease, this phrase is a little misleading, as ALS belongs to a larger group of Motor Neuron Diseases.) In France, ALS is known as Maladie de Charcot. In the United States, ALS is called Lou Gehrig's disease after the baseball player who retired in 1939 after being diagnosed. Gehrig died in 1941, but used his fame as a celebrity to educate and raise awareness in the general public about ALS.
Since the 1960s, there were several theories written about the etiology or possible causes for Lou Gehrig's disease. In 1875, Charcot put forth the possibility of the poliovirus being responsible for these symptoms. This was debated until 1907, when Wilson raised the theory of an environmental cause, such as lead poisoning causing ALS. Increased levels of aluminum, manganese, and mercury, and decreased levels of calcium and magnesium were also suspected. Since Wilson's first theory was made, it was discovered lead causes pseudo-ALS symptoms, which are reversible.
Even though it was believed the environment was the sole cause for ALS, instead it is now thought ALS might result from general causes, such as an altered immune system, dietary deficiency, toxicity, vascular disease, physical injury, and inflammatory conditions. Examples of other possible causes include antibodies manufactured against neurons, structural defects in the membranes of neurons, enzyme deficiencies, syphilis, prior surgery and malignancy. So trace metal toxicity is not the sole underlying cause for this disease.
In 1993, it was announced a link was discovered in one form of ALS. Scientists in North America were able to isolate a genetic mutation on chromosome 21, which causes 15 to 20 percent of familial ALS cases.
Afterwards in 1995, another possible cause was found. A glutamate transporter protein deficiency was discovered in the brain and spinal cord of some ALS patients.
Even though we now know ALS is a neurodegenerative disease, the main cause for the disease is unknown, many scientists believe the causes for ALS are multifactorial.
Many people believe Amyotrophic Lateral Sclerosis (ALS) is a new disease, but reports were published about this disease in British medical literature since the early 1830s. The next account describing ALS was written in 1853 by Cruveilhier, who observed a circus owner with ALS symptoms. However, the most detailed description of ALS was written between 1869 to 1874, and credited to Dr. Jean Charcot. Charcot is known as one of the pioneers of neurology. One of his maids had ALS, and Charcot was able to make observations, and write notes on the clinical findings of this disease.
Over the past three decades, ALS has developed an equal distribution in all major countries worldwide. The disease has an incidence rate of 2 per 100,000 people. The occurrence of ALS is higher in men than in women, and 5,600 new cases occur each year in the United States. However, the prevalence rate for men and women is almost identical, as we get older. It is estimated the prevalence rate of ALS in the United States is 5 per 100,000 of the total population. This would mean if you were gathered in a crowd of 100,000 people, at least five of these people would be diagnosed with ALS within this specified population, and during this particular time.
The number of patients diagnosed with ALS is high in the United States and England compared to other major countries with the same population. Countries such as Italy and Poland were discovered to have lower incidence rates than other countries such as the United States.
However, during the early to mid 1990s, there were a number of clusters cases diagnosed in Japan, Micronesia, and Indonesia with a local incidence rate at least fifty times higher than the worldwide incidence rate.
Usually people diagnosed with ALS are 40-70 years old, with an average life expectancy of 2-5 years. Twenty percent will survive for five years, and 10 percent of ALS patients will live for at least ten years.
|Jennifer Mathes, Ph.D.|