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The Nature of a Beast: Understanding ALS

By Arleen M. Kaptur
(Page 1 of 3)

There are so many different situations which can make someone a caregiver, like a loved one getting into a horrible accident and being rendered immobilized. The years can begin to take a toll on the mind and body, and help is needed with the simplest of things. Then there are the ravages of a horrific disease, ALS (Amyotrophic Lateral Sclerosis), also known as “Lou Gehrig’s Disease” in the United States (after the baseball great), MND (Motor Neurone Disease) in England, and Maladie de Charcot in France, which robs mobility and is eventually fatal for those who are in the prime of their lives. ALS is a neuromuscular disease which begins as muscle weakness, and then slowly progresses into total and complete paralysis throughout the entire body. ALS becomes a bit easier to understand when it is broken down by its Greek meaning:

  • A—without
  • Myo—muscle
  • Trophic—nourishment
  • Lateral—side (of the spinal cord)
  • Sclerosis—hardening or scarring

Although ALS was first described by French neurologist Jean-Martin Charcot as long ago as 1869, there is still no cure for this devastating disease. About 50 percent of ALS patients die within the first 18 months of diagnosis, with 20 percent of ALS patients surviving five years; however, people who go on a ventilator to help them breathe may be able to live for many years. The average age for onset seems to be around 55, but people as young as 12 and as old as 98 have been diagnosed. Around 80 percent of all ALS cases usually begin between the ages of 40 to 70. In recent years, more and more people in their 20s and 30s are being diagnosed with ALS.

This terrible disease does not usually discriminate between sex or race; however, men are more likely to be diagnosed than women. The cause of ALS is still unknown, but environmental factors are a strong, suspicious factor as a possible cause, with a higher incidence among people who have been exposed to agricultural chemicals and solvents. Whatever the cause, medical researchers have discovered that an excess amount of a neurotransmitter called glutamate is produced, clogging the synapse nerve cells, preventing transmission of neural impulses. This clogging of the synapses causes eventual death of these nerve cells, resulting in motor neuron damage and muscle atrophy (shrinking), specifically to nerve cells in the brain and spinal cord that control voluntary movement.

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