The actor famous for his numerous roles in French series (Tomorrow belongs to us, Sous le Soleil…) died following a “stark” Charcot disease. He was 69 years old.
The announcement was made by his daughters in a press release sent to AFP on May 25. “It is with deep emotion that we announce the death of Pierre Deny on Monday as a result of dazzling ALS (amyotrophic lateral sclerosis known as “Charcot disease”, editor’s note)”they shared. The actor was made famous by numerous roles in French series: Tomorrow belongs to us, Julie Lescaut, Camping Paradis, A woman of honor, Sous le Soleil… He was 69 years old.
Like other stars before him, Pierre Deny had to fight against this terrible neurodegenerative disease which affects the motor neurons of the brain and spinal cord. Initially, ALS begins with muscle weakness, muscle atrophy and gradually leads to paralysis. “It is classified as a “rare” disease but it is considered the most common of rare diseases with around 8,000 patients in France currently affected. The risk is 1 in 400 people in France. 5 new cases are diagnosed per day” We explains Dr Pierre-François Pradat, neurologist and researcher specializing in ALS at the Pitié-Salpêtrière Hospital (Paris).
Charcot disease mainly occurs between the ages of 50 and 70, more often in men. Its survival prognosis is poor: at the time of diagnosis, the life expectancy of the affected person is 3 to 5 years. With the improvement in care, 20% of patients (1 in 5) live 5 years or more. “It happens that some patients live up to 10 or 15 years longer” shares the national association dedicated to this disease, ARSLA. There are also mild forms of the disease that remain stable for more than 30 years, but they are very rare.
Why is life expectancy so short? Mainly because no treatment can currently stop the progression of this disease. The motor deficit is irreversible. However “it is a very heterogeneous disease, underlines the neurologist. There are SLAs and not just one SLA. And fortunately there are also slow forms”. Charcot disease thus progresses at a different pace depending on the patient. “It can stabilize spontaneously (around 15% of patients) at a variable stage of disability” informs ARSLA.
Death occurs when paralysis affects the diaphragm, a muscle essential for breathing. The person will develop respiratory failure and/or a respiratory infection. This is a specificity of this disease. Unlike Parkinson’s disease or Alzheimer’s which are also neurodegenerative diseases, Charcot’s disease affects the respiratory system which is essential for survival. It is hereditary in 10% of cases.
