In addition to the possible symptoms already mentioned above, which are progressively worsening, in the usual forms of amyotrophic lateral sclerosis, inadequate respiratory capacity is to be expected after three to five years after the initial onset of the disease, resulting in death either through pneumonia or through suffocation. Amyotrophic lateral sclerosis is a chronic degenerative disease that progresses continuously. It occurs in most cases in patients between 60 and 70 years of age and has an extremely poor prognosis with an average life expectancy of 3 years.
Exactly when the first symptoms appear and to what extent cannot be predicted. A cure for the disease is currently not yet possible. Therefore the duration of the disease is difficult to predict and can vary greatly between individuals.
The therapy of amyotrophic lateral sclerosis is currently limited to alleviating the symptoms that occur. A therapy that follows a curative approach does not yet exist at the current state of research. However, there are now a number of approaches as to what such a therapy could look like.
ALS is triggered by the death of so-called alpha-motoneurons in our spinal cord and brain stem. The task of these nerve cells is to transmit information from the brain to the muscle. If these nerve cells now go under, no more movement commands can be passed on from the brain to the muscles and paralysis results.
The focus of research is now on promoting the new growth of such nerve cells, which could then replace the destroyed cells and thus make information transmission possible again. However, these approaches are still at too early a stage to be able to make statements about their effectiveness. The average life expectancy of patients with amyotrophic lateral sclerosis is greatly reduced.
One assumes a survival time of 3 years after diagnosis. However, one in ten of the patients survive for more than five years. Only five percent of those affected live longer than ten years after diagnosis. Probably the most famous example of a significantly above-average life expectancy is Steven Hawking.
The extent to which amyotrophic lateral sclerosis is a hereditary disease is very controversial in science. It has been established that similar genetic changes are present in almost all patients with ALS. In most cases, different genes (TARDP, C9ORF72, …) are affected, which cause a pathological accumulation of certain proteins, which, it is discussed, lead to the death of these nerve cells. However, it remains unclear why this process only specifically affects the alpha-motoneurons of the spinal cord and brain stem.
Ice Bucket Challenge
Behind the term “Ice Bucket Challenge” is a fundraising campaign started in 2014, the exact beginning of which is unknown. The goal was to collect as much money as possible, which was donated to the research and therapy of ALS. The exact challenge was to pour a bucket of ice water over your head and then donate 10€ for this purpose.
Now you also appoint friends to whom you set this challenge. So the fundraising campaign spread all over the world and brought in a total of about 42 million Euros for ALS research.