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The Nature of a Beast: Understanding ALS

By Arleen M. Kaptur
(Page 2 of 3)

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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