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Last updated:08-19-2009
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Amyotrophic Lateral Sclerosis - ALS
 

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.  
 

Definition and causes
 

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. 
 

Symptoms of ALS 

  • 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. 
 

Definition and causes

 
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


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.

 

Related articles:

ALS - Amyotrophic Lateral Sclerosis
Chorea (including Huntington's Chorea)
Dementia & Alzheimers disease
Neuromuscular diseases
Parkinsons disease (Paralysis agitans)




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