ARTICLES / General /The
Nature of a Beast: Understanding ALS/
Other ArticlesBy Arleen M. Kaptur
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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