Definition and causes of ALS
Amyotrophic Lateral Sclerosis - ALS is the most serious of the motor neuron diseases as it is always fatal involving a degeneration of the nerves in the central nervous system (the brain and spinal cord) that control muscular activity. - Very few victims live longer than 5 years from the diagnosis. - See additional comments below under Outlook.
ALS - is a disease where the nerve cells in the anterior section of the spinal cord and cerebral cortex (the alfa motor neurons) slowly degenerate. These cells control the muscle movements, and it is therefore the muscles that are affected by ALS.
Men are affected more frequently than women and it is usually middle-aged people (50-60 years of age) who are affected. ALS is a rare disease, with only 1 new case annually per around 100.000 inhabitants in western countries. The cause is unknown but a small proportion of cases are hereditary.
- The nerve degeneration results in decreased muscle force and wasting. Usually the first symptoms occur as weakness in a hand, an arm or a leg. Difficulty in writing is often the first sign of the disease.
- Difficulty with swallowing or speech due to the impairment of the muscles controlling the neck and throat muscles and the tongue.
- Cramps and pain in muscles.
- The symptoms are gradual, and more and more of the body's muscles will be paralyzed. In later stages also breathing will be affected. The eyes are often the last to be affected.
There is no exact test for motor neuron diseases such as ALS but rather a comprehensive examination by a specialist involving a number of tests including blood tests, biopsy, and scanning.
Treatment and outlook of ALS
There is no curative treatment. Supportive therapy and physiotherapy is important. Some drugs can delay the process but not stop it. The disease is fatal, and the disease duration is usually less than five years but some may live longer with the disease.
In rare cases the disease evolve as early-onset, slow progression and extended longevity. One such high profile case is the English theoretical physicist and cosmologist Stephen William Hawking, born in 1942 and diagnosed with ALS at the age of 23 years. In early 2015, at the age of 73 years, Stephen Hawking is still alive and active, yet paralyzed for decades.
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