the majority of patients disappear in three to five years

THE ESSENTIAL

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

The name “Charcot” comes from the French neurologist who was the first to describe amyotrophic lateral sclerosis (ALS). It is also known as Lou Gehrig’s disease in English, after a famous baseball player who was affected by this disease.

Whatever this degenerative disease is called, the reality for patients suffering from this disease, which leads to progressive paralysis and death, is the same: With no current treatment available, they watch their bodies helplessly.

ALS takes different forms depending on the nature of the initial damage, as stated by Inserm: “About 30% of the time it starts in the brainstem. We then speak of bulbar-onset forms, the first manifestations of which are difficulty in articulation or swallowing. In the other cases, ALS first affects the peripheral motor neurons: in these spinal-onset forms, the disease manifests as weakness and discomfort in an arm, a leg, or a hand.”

neurons

ALS affects the neurons (nerve cells) and prevents the transmission of information between the brain and muscles throughout the body, gradually making the disease worse and reaching all muscles, including the respiratory muscles, speeding up its deterioration. Specifically, there is muscle atrophy, incoordination that prevents walking and picking up objects. Swallowing or articulation difficulties increase. The brain remains intact.

The causes are unknown: a genetic cause is suspected in one in ten cases, but the onset of the disease probably depends on various environmental and genetic factors. It generally occurs between the ages of 50 and 70, often earlier if it is of familial origin. The outcome is fatal on average after 3 to 5 years of evolution and most often it is the respiratory muscle attack that causes the death of the patients.

“Only your eyes and brain work”

On Europe 1, Christophe Malsot, 57, commercial director who discovered he had the disease 18 months ago, details: “You start not being able to breathe on your own, you lose your speech, and only your eyes and brain work. Today, when the diagnosis comes, you’re told you’re going to die. 80% of people disappear between three and five years.” According to him “So a lot of people [atteints] choose to say, ‘It’s over, I’d rather go.'” For him, the choice is already made: “If at some point it becomes too vegetative, I would love to do that.” The case of famed astrophysicist Stephen Hawking, who was diagnosed when he was 21 and died in 2018 at the age of 76 after living with the condition for fifty years, remains a mystery.

Therapeutic Traces

A curative treatment is not yet available, but the medium-term prospects are encouraging as several therapeutic avenues are being explored with the search for relevant biological markers.

Their identification could facilitate early diagnosis of the disease in families of people with the following conditions: “It has indeed been proven that neurodegenerative diseases cause biological and morphological changes several years before the first symptoms appear. However, this pre-symptomatic stage probably represents the best window for therapeutic intervention to prevent the neurodegenerative process before it causes irreversible damage ” after ins.

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