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 more common in men between 50 and 70 years of age, but can affect both sexes and 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. Patients typically present difficulty walking with frequent trips and falls, difficulty getting up from a chair, weakness and clumsiness of the hands, making it difficult to perform daily tasks, cramps and spasticity of the muscles, etc. In more advanced stages it affects the musculature of the mouth causing difficulty with chewing and the swallowing muscles, leading to frequent choking. Finally it affects the respiratory musculature causing a progressive and lethal respiratory failure. This can be accompanied by cognitive changes, and changes in mood and behavior.
The slow onset of symptoms and confusion with other neurological diseases may delay diagnosis. Once suspected, an electromyogram should be performed to analyze the electrical activity of the muscles, as well as a nerve conduction study.
It is a disease with no cure. There are drugs that delay the evolution of the disease such as Riluzole and Edaravone. Combined with physiotherapy, speech and swallowing therapies, nutritional support and psychological support can slow the progression of the disease.
Life expectancy is 3 - 5 years from the onset of symptoms, although there are reported cases of more than 10 years of survival.
- Amyotrophic lateral sclerosis among patients with a Medicare Advantage prescription drug plan; prevalence, survival and patient characteristics. https://doi.org/10.1080/21678421.2019.1582674
- Amyotrophic lateral sclerosis. https://doi.org/10.1016/S0140-6736(17)31287-4
- Current issues in ALS epidemiology: Variation of ALS occurrence between populations and physical activity as a risk factor. https://doi.org/10.1016/j.neurol.2017.03.035
- An assessment of treatment guidelines, clinical practices, demographics, and progression of disease among patients with amyotrophic lateral sclerosis in Japan, the United States, and Europe. https://doi.org/10.1016/j.neurol.2017.03.035
