- ALS is due to the progressive death of motoneurons, the nerve cells that drive and control voluntary muscles.
- Most often, it is the involvement of the respiratory muscles that causes the death of patients.
“My name is William. I’m 52 years old and I was diagnosed with Charcot’s disease a year and a half ago.” In an interview with the Huffpost, a father recounts how he discovered he had Charcot’s disease (or amyotrophic lateral sclerosis, called “ALS”). “The first symptoms were fatigue and loss of balance: I was running a lot and more and more often I fell for no reason. I also had small muscle twitching. At first I wasn’t worried at all, but as it persisted, I was consulted, and very quickly, in 15 days, I was diagnosed. “ he remembers.
Grave degenerative disease
In February 2021, William created his YouTube channel with a very specific goal. “The goal is to leave a mark on my loved ones, children, and grandchildren that I may never know.” he explains in his first video.
Amyotrophic lateral sclerosis is a serious and disabling degenerative disease that leads to death within 3 to 5 years after diagnosis. “The research effort dedicated to it in recent years has significantly developed knowledge of its genetics and biology. And no curative treatment is yet available, the medium-term outlook is encouraging.” indicates the Inserm.
The symptoms of Charcot’s disease are characterized by complete paralysis of the muscles of the arms, legs, mouth and tongue, as well as the respiratory muscles. “This leads to an inability to use one’s arms, to walk, to eat, to talk, and to gradually develop breathing difficulties.” explains the Brain Institute.
Today, ALS affects 5,000 to 7,000 patients in France, with an annual incidence of close to 2.5 per 100,000 inhabitants. “Clinically, ALS begins on average at age 55-60 with a very low male preponderance,” indicates the arsla.