A disease in which the neurons of the nervous system are destroyed, losing their function of sending stimuli to the different muscles of the body. It affects neurons in the brain, brain stem and spinal cord.
It is most common in men between the ages of 50 and 70, but can affect both sexes at any age.
The cause is unknown, although it is known that there is a hereditary component influenced by an environmental component. The following risk factors have been described: smoking, exposure to environmental toxins such as lead and having served in the armed forces (repeated traumatic injuries, intense physical exertion, viral infections and possible exposure to toxins).
It manifests with muscle weakness that worsens over time. They typically have difficulty walking with frequent trips and falls, difficulty getting up from a chair, weakness and clumsiness in the hands that makes it difficult to carry out everyday tasks, cramps and spasticity of the muscles, etc. In more advanced stages, it affects the muscles of the mouth, making chewing difficult, and the swallowing muscles, causing frequent choking. Finally, it affects the respiratory muscles causing progressive and lethal respiratory failure. This can be accompanied by cognitive changes, mood and behavioural changes.
The slow onset of symptoms and confusion with other neurological diseases may delay diagnosis. Once suspected, an electromyogram should be performed to analyse the electrical activity of the muscles, and a nerve conduction study.
It is a disease that cannot be cured. There are medications that delay the progression of the disease, such as Riluzole and Edaravone. Combined with physiotherapy, speech and swallowing therapies, nutritional support and psychological support they can slow down the progression of the disease.
Life expectancy is 3-5 years from the onset of symptoms, although there are reports of survival of more than 10 years.
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