Charcot disease: the majority of patients resolve within three to five years

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  • Today is World Amyotrophic Lateral Sclerosis (ALS) Day.
  • It is estimated that in France there are between 7,000 and 8,000 people with ALS.

The name “Charcot” comes from the French neurologist who first described amyotrophic lateral sclerosis (ALS). In English, it is also called Lou Gehrig’s disease, in honor of a famous baseball player who has this disease….

Quel que soit le nom donné à ce trouble dégénératif, la réalité des patients atteints de cette maladie qui entraîne une paralysie progressive à l’issue fatale est la même : comme il n’existe actuellement aucun gradé trait entà la the body.

ALS takes different forms depending on the nature of the initial damage, as described by Inserm: “In about 30% of cases, it begins in the brainstem. Then we talk about forms with a bulbar appearance, the first manifestations of which are difficulties with expression or swallowing. In other cases, ALS first changes the peripheral motor neurons: in these forms with the beginning of the spine, Weakness and discomfort appear in the arm, leg or hand. The onset of the disease.”

nervous cells

Amyotrophic lateral sclerosis affects the nerve cells (neurons) and prevents the transmission of information between the brain and the muscles of the whole body, which gradually leads to the intensification of the disease and its reach to all muscles, all the way to the muscles of the respiratory system, which leads to its exacerbation. Concretely, there is muscle wasting and coordination disturbances that prevent walking and picking up objects. Increased difficulty swallowing or speaking. The brain remains intact.

Its causes are unknown: in one in ten cases a genetic cause is suspected, but the onset of the disease probably depends on various factors, both environmental and genetic. It generally appears between the ages of 50 and 70, often earlier when it is of family origin. The outcome is fatal after 3 to 5 years of development on average, and it is often the attack of the respiratory muscles that causes the death of patients.

“Only your eyes and your mind work”

In Europe 1, Christophe Malsot, 57, the commercial director who was diagnosed with the disease 18 months ago, details: “Vous commencez à ne plus pouvoir respirer tout seul, vous perdez la parole, et seuls vos yeux et votre cerveau fonctionnent. Aujourd’hui, lorsque le diagnostic tombe, on vous informe que vous allez mourir. distrois des ent 80%” five years.” according to him, “That’s why so many people [atteints] I decided to say, “It’s over, I’d rather leave.” For him, the choice has already been made: “If at some point he becomes very vegan, that’s what I’d like to do.” The case of the famous astrophysicist Stephen Hawking, who was diagnosed at dawn at the age of 21 and died in 2018 at the age of 76, after living fifty years with the disease, remains a mystery.

therapeutic pathways

There is no curative treatment yet, but the prospects in the medium term are encouraging with several therapeutic avenues being studied with the search for relevant biomarkers.

Recognizing them can facilitate early diagnosis of the disease in families that include people with: “Neurodegenerative diseases have in fact been shown to cause biological and morphological changes several years before the first symptoms appear. However, this pre-symptomatic phase probably represents the best window for therapeutic intervention to prevent the neurodegenerative process, before it causes irreversible damage” According to Inserm.

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