2024-01-22 12:52:36
By EP
Published 4 hours ago, Updated 2 hours ago
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In Saint-Vaast-en-Chaussée near Amiens, five people living in the same street died from this rare disease, which only affects 8,000 patients in France.
Chance, curse or epidemic? In the village of Saint-Vaast-en-Chaussée, in the Somme, residents are wondering about the occurrence of 5 cases of Charcot’s disease in almost 12 years in the same street, notes BFMTV.
The first case concerns a tire manufacturer who died in 2009 at the age of 67. Diagnosed with Charcot disease in October 2008, he died 6 months later. When a second person, living nearby, is diagnosed with this same neurodegenerative disease, “we thought it was a coincidence», Explains Françoise Gamain, widow of the first case. “But after several cases, we started to say that it was a lot of coincidences“, she explains to BFMTV.
Amyotrophic lateral sclerosis (ALS) – also called Charcot disease – is, of all neurodegenerative diseases, one of the most destructive and devastating. This disease manifests itself as progressive muscular paralysis due to degeneration of motor neurons, the cells that control muscle contraction. Little by little, this paralysis leads to respiratory failure which, in the absence of assistance, most often leads to death within two to five years.
Genetic and environmental factors
In this town of 500 souls near Amiens, as many cases of a rare disease raise questions. Especially since at the national level, the Association for Research on ALS (ARSLA) does not count more than 8,000 cases in total. Each year, the increase is 2.7 new cases per 100,000 inhabitants, specify the Hospices Civils de Lyon.
Requested by the village mayor, the Hauts-de-France Regional Health Agency (ARS) confirmed “a high number of ALS cases in this municipality» and contacted Public Health France, in charge of health monitoring. “Investigations are underway» pour «determine if there are one or more local causes for this clustering of cases, other than chance», Declared the organization to BFMTV.
Although the origin of this disease is still the subject of research, it is probably due to genetic and environmental factors. “It is entirely likely that environmental factors are involved in ALS, but a favorable genetic background is required.», nuances François Pradat, neurologist at the Pitié-Salpêtrière hospital (APHP) and co-president of the ARSL scientific council, recalling that 10% of ALS cases have a family history.
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