ARTICLES / General /The
Nature of a Beast: Understanding ALS/
Other ArticlesBy Arleen M. Kaptur
The motor nerves that are attached to muscles
gradually degenerate and die from lack of receiving
nerve impulses, and as a result of this nerve death,
the muscles atrophy and waste away. By the time
someone with ALS first notices neurological
symptoms, more than half of the motor neurons may
already be dead. Muscle paralysis is the end result
of this type of nerve death.
Symptoms of ALS may include tripping, stumbling
and falling, loss of muscle control and strength in
hands and arms, difficulty speaking, swallowing
and/or breathing, chronic fatigue, and muscle
twitching and/or cramping, and drooling. Both upper
and lower motor neuron damage is experienced, with
the symptoms of upper motor neuron damage resulting
in stiffness, muscle twitching, and muscle shaking,
and the symptoms of lower motor neuron damage
including muscle weakness and muscle shrinking
(atrophy). The most common path for ALS to take
first is to weaken either the arms or legs, making
this known as “limb-onset” ALS.
While the symptoms of ALS are not generally
painful, some of the effects may be, such as
pressure sores, muscle cramps, joint contractions,
constipation, burning eyes, swelling feet, and
muscle aches. Surprisingly, there are a few muscles
that remain untouched by ALS, enabling people to
retain their bowel, bladder, and sexual functions.
In a cruel twist of fate, the mind also remains
unaffected and remains quite sharp, even when there
is total paralysis. Eating can also become more
difficult as muscles begin to weaken, with a loved
one’s diet including softer foods and the
eventuality for a feeding tube, but this can greatly
extend the quality and quantity of life.
Three types of ALS exist, with the most common
type called sporadic ALS. This type
is not hereditary, but does account for about 90
percent of all ALS cases. Only about 10 percent of
ALS is recognized as hereditary (familial ALS),
which is passed on by a dominant gene. The third
type of ALS is called “Guamian,” named for the
unusually high incidence of ALS on the island nation
of Guam. Diagnosis is made usually through a process
of elimination and can take several weeks or even
months to detect and confirm existence, since early
symptoms can mimic many other neuromuscular
diseases. The usual diagnostic tests include MRI
(magnetic resonance imaging), EMG (electromyogram),
muscle biopsy, and blood tests. A definitive
diagnosis of ALS can only be made when the existence
of damage is evident in both upper and lower motor
neurons, and at least three limbs are sufficiently
affected.
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