what you need to know about this degenerative disease

2023-04-20 10:09:00

There is no cure for amyotrophic lateral sclerosis. Research is progressing to better understand its appearance.





Par Johanna Amselem

10% of ALS cases are of genetic origin and 90% have no identified cause.
10% of ALS cases are of genetic origin and 90% have no identified cause.
© NIH / IMAGE POINT FR / IMAGE POINT FR / BSIP via AFP

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EBetween 5,000 and 7,000 French people suffer from amyotrophic lateral sclerosis (ALS). Eight years ago, on April 17, 2015, Guilhem “Pone” Gallart, ex-rapper of the Marseille rap group Fonky Family, learned that he too suffered from Charcot’s disease. Paralyzed, he has just taken out a book entirely written with his eyes thanks to an ocular navigation system which makes it possible to capture the movement of his eyes.

Amyotrophic lateral sclerosis – also known as Charcot’s disease – is a serious and disabling degenerative disease. It is manifested by a progressive paralysis of the motor muscles, it also affects speech and swallowing. The evolution is rapid and fatal since it generally leads to death within five years of diagnosis. Generally, the disease appears between 50 and 70 years, younger when it is of family origin.

READ ALSOCharcot’s disease, the fight and the hope

“ALS is caused by the progressive death of motor neurons, the nerve cells that direct and control voluntary muscles. Both types of motor effector motor neurons are affected: those called central, located in the brain, and those called peripheral, located in the brainstem and spinal cord. The latter provide the relay between the central motor neurons and the muscles,” explains Inserm.

Regarding the origin of the disease, no environmental triggering factor has yet been clearly identified. What regarding genetics? A familial origin was observed in 10% of patients. Generally, the diagnosis is made by elimination once the medical team has ruled out certain pathologies.

READ ALSOStephen Hawking, a life of fighting once morest Charcot’s disease

What treatment?

To date, there is no cure for Charcot’s disease. Currently, riluzole is the only drug approved for its action on the evolution of this pathology. Multidisciplinary care can relieve certain consequences of ALS, as summed up by Inserm: “The care of the disease targets the symptoms: technical assistance, physiotherapy and antispastic drugs to counter motor disorders, muscle relaxants and analgesics once morest pain, management of undernutrition, speech therapy for speech and swallowing disorders, psychological support. »

In 2006, a study identified the first three potential biomarkers of amyotrophic lateral sclerosis (ALS). A discovery that paved the way for earlier treatment thanks to faster diagnosis. Researchers at Mount Sinai School of Medicine in New York (USA) have identified three proteins that might be the first potential biomarkers of ALS, offering “the possibility, for the first time, of developing a test to confirm definitely the diagnosis. A model combining these three proteins might improve and accelerate the diagnosis of the disease.

READ ALSONaima’s resistance to Charcot’s disease


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