Charcot’s disease: 4 deaths a day in France, what is it?

4 people die every day in France from Charcot disease. June 21 is World Day dedicated to this incurable neurodegenerative disease. At what age does it occur? What are the first symptoms? what are the reasons? What is the average life expectancy? What is the treatment? Answers and diagnosis.

[Mise à jour le 21 juin 2022 à 10h38] Every day in France, 4 people die of Charcot’s disease And 5 people see their lives shattered when the diagnosis was announced, ARSLA (Association for ALS Research – Charcot’s Disease) recalls marking the occasion International Day dedicated to this incurable disease, which falls on June 21. Throughout this month, ARSLA relies on your mobilization by posting on your social networks and participating in the launched challenges. The Amyotrophic lateral sclerosis (ALS), or Charcot’s disease (named after the physician who described it in the 19th century), is a Rare progressive degenerative disease usually announced for 60 – 65 years old More common in men of women. The the reasons It’s still up for debate but we know it’s changing nervous cells. The symptoms affect the muscles. This is a serious disease Reduces life expectancy. The The famous astrophysicist Stephen Hawking, Suffering from this disease, it has contributed to the talk about ALS. In France, comedian Jean-Yves Lavis, his real name Jean-Yves Lambert died of Charcot’s disease at the age of 64, Thursday, July 22, 2021 in Vans, Brittany. What is this Charcot disease or amyotrophic lateral sclerosis? at what age Can we get it, what is the treatment to treat it? what or what the hope of life ?

Definition: What is Charcot disease?

Amyotrophic lateral sclerosis (ALS) is a rare progressive degenerative disease of motor neurons. Marked by Weakness and then paralysis of the muscles of the legs and armsThe muscles of the respiratory system and the muscles of swallowing and speech. Intellectual and sensory functions are not affected. This serious, progressive disease reduces the life expectancy of those affected.

At what age can you get Charcot disease?

The average age of onset of this disease is about 60 years old with slight male predominance. Highly active people, including top athletes, are more likely to get injured.

How do you get Charcot’s disease?

We do not know the originbut it leads to a change in neurons allowing the transfer of order a movement : We call them motor neurons. These neurons are affected both in the central nervous system, the brain and spinal cord, but also in the peripheral nerves. It inevitably leads to the death of the neurons that allow voluntary muscle movement, followed by paralysis. We still do not know the exact causes of this degeneration, but scientists are currently discussing several theories: A Very high level of glutamate (The “messenger” that interferes with the transmission of nerve messages) disable the growth factor (a substance that promotes the growth of certain cells), an abnormal inflammatory reaction, or a combination of these different hypotheses.

Diagram of Charcot’s disease © blueringmedia – 123RF

What are the first symptoms of Charcot disease?

ALS can appear in two main forms: shape “spine” (which begins with the participation of one of the parties), and “bulb” shape (which begins with damage to the muscles of the mouth). The affected person may show various symptoms of gradual development which extend gradually:

  • subordinate muscle crampsAnd the
  • difficulties in mobilization, progression to paralysis,
  • a decrease in muscle mass, called atrophy,
  • slowed movements,
  • involuntary contractions of certain muscle bundles in the form of fasciculations,
  • swallowing disorders
  • eating difficulties,
  • Speech disturbances.

How is Charcot disease diagnosed?

There is no There is no specific test To diagnose amyotrophic lateral sclerosis. Because the first symptoms can be quite subtle (cramps, hand weakness, voice change), doctors sometimes have difficulty making a diagnosis. Above all, they must “eliminate” diseases close to ALS‘, says Dr. Claire Lewandowski, M.D., who specializes in general medicine. Additional tests, such as Electromyogramas well as a Muscle biopsy is possible lead to increased suspicion. a Brain imaging (MRI) testlumbar puncture and Blood tests It is often offered.

How do we treat Charcot disease?

one medicine riluzoleslows down the progression of the disease. Lowers glutamate levelsThis nerve messenger that can be found in abundance in people with amyotrophic lateral sclerosis. It is usually prescribed as soon as the disease is suspected. In addition, some medications can help treat symptoms (analgesics, antidepressants, laxatives, etc.) Non-pharmacological measures can be taken to relieve and support those affected. a Psychological support sessions, physical therapy, rehabilitation or speech therapy It can help patients maintain muscle flexibility, retain their independence and ability to communicate for as long as possible. Medical and social care and installation of technical assistance are also essential.

How does Charcot disease develop?

Charcot disease significantly reduces life expectancy

ALS is a disabling neurodegenerative disease (at the motor level) Significantly reduces life expectancy. evolve into a The rhythm varies from one infected person to another Without it it is possible to predict the duration of its development, even if the form of ALS with a follicular onset is characterized by a faster development. The most common causes of death are difficulty breathing associated with paralysis of the respiratory muscles and respiratory infections (which can lead to swallowing disorders).

What is the average life expectancy for Charcot disease?

The average life expectancy of a person with amyotrophic lateral sclerosis is approximately 3 to 5 years after diagnosis. However, with improved support, 20% of people live five or more years after diagnosisand 10% live 10 years or more. There are also mild forms of the disease that remain stable for more than 30 years, but they are rare.

Evidence for Charcot’s disease

Thank you to Dr. Claire Lewandowski, M.D., specializing in general medicine.

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