What are the first signs of Charcot’s disease?

Affected by Charcot’s disease, the sports journalist and figure of the Canal+ of the great era Charles Biétry, 81, is the guest of Audrey Crespo-Mara in the portrait of “seven to eight” broadcast this Sunday, January 26 on TF1 .

In April 2023, the latter had revealed to suffer from this incurable evil in daily interview The teamand announced that he had organized his assisted suicide in Switzerland. “We organized everything with my wife and my children. I don’t want to be connected to a machine to breathe while there is nothing left, no more future. I don’t want to suffer and especially make my family suffer (…) I signed up in Switzerland for assisted suicide, all the papers are signed “, he confided. He also mentioned the progression of his illness, which is characterized by a progressive paralysis of the muscles.

What are we talking about?

Also also called amyotrophic lateral sclerosis (SLA), Charcot’s disease is a rare paralyzing neurological pathology with, for example, around 2500 new cases per year in France. It is part of the group of motor neural diseases, which gradually degenerate and lose the patients with control of their muscles, insult them irreversibly in their own body.

As a number of neurological pathologies, first of all, Alzheimer’s disease, treatments are almost non -existent despite decades of research. To date, there is no effective neuroprotective treatment. Age of appearance, evolution speed, presence of cognitive problems … It varies a lot according to the patients. Thus, it generally appears between 50 and 70 years old, but often more early when it is of family origin.

What are the first symptoms of the disease?

Once declared, the disease generally paralyzes the muscles gradually, gradually preventing from walking, feeding, speaking or breathing without help, while the brain remains intact. Most often, it starts with a loss of ability to move your arms and legs, then when the diaphragm and chest wall muscles are affected, patients lose their respiratory capacity and are on assistance. According to medical statistics, death usually occurs after three to five years of evolution on average, specifies Inserm. Most often, it is the inability to breathe that carries the patient.

More in detail, “In around 30% of cases, the disease begins in the brainstem”the first manifestations then being the difficulties in articulating or swallowing, while the other cases, “SLA first affects peripheral snowmobileurons and it is by weakness and discomfort in the arm, one leg or a hand that the start of the disease manifests,” Can we still read on the Inserm website.

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  Affected by Charcot’s disease, former journalist Charles Biétry reveals having prepared his assisted suicide

Usually, Charcot’s disease is accompanied by cognitive disorders with a fronto-temporal lobar degeneration. The patients developing the disease therefore gradually have more and more difficult to swallow and speak. In addition to this lesser ability to express themselves and decreased attention, obsessions, disinhibition and apathy are also observed.