Amyotrophic Lateral Sclerosis: Merciless Disease
The muscle and nerve disease ALS is merciless. Step by step, different muscle groups fall out, until eventually the respiratory muscles give out. Death is the result. The disease is diagnosed annually in about 500 Dutch people. And they sometimes feel quite misunderstood, because it is a disease that is very unknown and for which there is no medicine yet.
Amyotrophic Lateral Sclerosis (ALS) is a fatal nerve/muscle disease. It is one of the most serious and debilitating disorders of the nervous system, leading to the inadequate functioning of the muscles. This is because the motor nerve cells in the spinal cord and the lower part of the brain – the so-called brain stem – die.
Amyotrophic Lateral Sclerosis comes from the Greek. A (meaning none), myo (referring to muscle), trophy (meaning nutrition); muscles that receive no or insufficient nutrition or nerve impulses. Muscles that do not receive nerve impulses ‘atrophy’: the muscle mass disappears. Lateral refers to the area in the spinal cord where the nerve cells that transmit impulses to the muscles are located. When the nerve cells in this area die, this leads to scarring or hardening: sclerosis.
muscle movements
Because those nerve cells die, the signals from the brain no longer reach the muscles. The brain can then no longer initiate muscle movements. Patients die on average three years after the first symptoms, often due to weakness of the respiratory muscles.
In the Netherlands, the diagnosis of ALS is made in about five hundred people a year. Every year, 125,000 people worldwide die from the disease, in our country around 450 per year. This means that the number of patients in our country remains more or less stable, at around 1,500.
Who gets it?
ALS can develop in anyone in adulthood, but it usually develops between the ages of 40 and 60. Only 5 percent of cases are familial; or hereditary.
The gradient
The disease usually causes no pain and does not affect the mind. The senses (feeling, taste, sight, smell and hearing) usually remain intact, as well as the functioning of the intestines and bladder.
However, gradually more and more muscles are involved. Except the heart muscle. The failure of the respiratory muscles is usually the cause of the death of someone with ALS. How fast that happens varies from person to person. But the average life expectancy of an ALS patient is only three to five years.
Cause unknown
The exact cause of ALS is not yet known. Nor are there any drugs that can stop or cure the disease.
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