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France - 5 cases of amyotrophic lateral sclerosis (ALS) on the same street in a village in the Somme department

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  • France - 5 cases of amyotrophic lateral sclerosis (ALS) on the same street in a village in the Somme department

    Click image for larger version  Name:	image.png Views:	1 Size:	42.6 KB ID:	984744
    Location of Somme in France
    https://en.wikipedia.org/wiki/Somme_(department)


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    Translation Google


    “WHERE CAN IT COME FROM?”: 5 CASES OF CHARCOT DISEASE IN THE SAME STREET, A VILLAGE IN THE SOMME WONDERS

    Céline Hussonnois-Alaya
    THE 01/22/2024 at 6:00

    Five people living in the same street in Saint-Vaast-en-Chaussée (Somme) contracted Charcot disease.

    “My husband was the first one on the street to get sick.” Françoise Gamain's husband - who worked for a tire manufacturer - died in 2009, at the age of 67, from Charcot's disease . “The second patient, we said to ourselves that it was a coincidence.”

    “But after several cases, we started to say to ourselves that it was a lot of coincidences,” she testifies for BFMTV.com.

    Because in a dozen years, five people living in the same street in Saint-Vaast-en-Chaussée, a village of barely more than 500 inhabitants, located about fifteen kilometers from Amiens, were affected by the same disease.

    Charcot disease, or amyotrophic lateral sclerosis (ALS), is a serious and severely disabling degenerative disease that results in progressive muscle paralysis. It leads to the death of the patient within three to five years following diagnosis, recalls Inserm (National Institute of Health and Medical Research).

    “It went very quickly,” remembers Françoise Gamain. “We were diagnosed in October 2008. By the following March, he had died.”

    “A high number of cases”

    The four other patients also lived in the same street in this village in the Somme. A number that calls attention to: ALS affects around 8,000 patients in France, indicates the Association for Research on ALS (ARSLA) . The annual incidence is 2.7 new cases per year per 100,000 inhabitants, specify the Hospices Civils de Lyon .

    How can we explain so many sick people in Saint-Vaast-en-Chaussée? The Hauts-de-France Regional Health Agency (ARS) was contacted by the village mayor. It corroborates all five cases. “The study of the report thus allowed the ARS to confirm a high number of cases of ALS in this town,” she explains to BFMTV.com.

    Public Health France, responsible for ensuring health monitoring and epidemiological surveillance, was thus contacted. “Investigations are underway,” we assure BFMTV.com.

    The agency explains that the principle of investigating “a cluster report” is to verify “whether there actually exists a statistical excess of diseases in the observed population”. And “if this excess exists”, to “determine whether there are one or more local causes for this grouping of cases, other than chance”. For the moment, no cause has been formally identified in the cases in Saint-Vaast-en-Chaussée.

    “The residents are aware, the new residents ask me questions, I don’t know any more,” concludes the mayor, Marc Vignolle, to Courrier Picard .

    “Why doesn’t this concern the other streets?”

    Françoise Gamain, who has lived in Saint-Vaast-en-Chaussée since 1976, wonders. "There are fields around the house, could it come from there? From phytosanitary products? From what we ate? But if it's in the water or in the air, why is that? does not concern the other streets of the village?"

    François Pradat, neurologist at the Pitié-Salpêtrière hospital (APHP) and co-president of the ARSLA scientific council, wants to be reassuring. If the situation in Saint-Vaast-en-Chaussée is “unusual”, he recognizes, it is “statistically possible”: “ALS is a rare disease but not exceptional.”

    “I have three patients who suffer from ALS who are part of the same cycling club,” assures the neurologist. “I also have couples where both members develop ALS. This does not mean that there is a link.” And adds:

    “If I had five people with ALS living on my street, I wouldn’t panic.”

    A “complex” origin to be determined

    The origin of the disease is “complex” to determine, recalls Inserm. The occurrence of the disease would be “multifactorial”, “subject to the influence of genetics and the environment”.

    In addition to the 10% of ALS with a family history, “as with all neurodegenerative diseases, there is a genetic susceptibility,” notes François Pradat. Especially since not all the genes involved in the disease have yet been identified.

    “It is entirely likely that environmental factors play a role in ALS,” recognizes François Pradat. “But you need a favorable genetic background.”

    Regarding the role of the environment, "no triggering factor has been clearly highlighted", even if tobacco, high-level sport, exposure to pesticides, heavy metals or even a toxin present in certain algae are suspected.

    In 2021, a Franco-American study implicated the giant gyromitre, also called false morel, in a cluster of Charcot's disease in Savoie. Between 1991 and 2013, some 14 cases of ALS were identified in the La Plagne-Tarentaise sector. Everyone would have ingested this mushroom.

    An avenue that François Pradat, neurologist at the Pitié-Salpêtrière hospital (APHP) and co-president of the ARSLA scientific council, is exploring. “In Savoie, this cluster has not been proven,” he explains for BFMTV.com. “It actually didn’t make statistical sense.”

    He specifies that it still remains difficult to establish a cluster for this disease. “There are a lot of biases: the age of the population, the duration of possible exposure… We often have stories of ALS clusters, but they rarely hold up.”

    "Where can it come from?"

    In 2013, an epidemiological study published in the scientific journal Plos One on 381 patients who developed ALS in Hérault showed that the number of patients was higher in the municipalities close to the Etang de Thau, a mussel production area. and oysters, recalls Medscape .

    The study incriminated a toxin - BMAA - produced by cyanobacteria found in the pond. This neurotoxin had already been implicated in cases of ALS on the island of Guam, in the Pacific - BMAA was present in cycad seeds consumed by the natives.

    “There is therefore a link between BMAA and ALS,” assures neurologist William Camu, head of the national center of competence for (ALS) at Montpellier University Hospital and author of the study. While specifying that "the consumption of seafood is not the cause of the excess incidence of ALS" and pointing out the inhalation of the toxin during sporting activities around the lake, the products used by the wood industries or the heavy metals contained in organisms.

    In Saint-Vaast-en-Chaussée, the investigation is still ongoing. “We are told nothing, we have no explanation, we have not been contacted by anyone, we have the impression that no research is being done,” laments the daughter of one of the patients, an ambulance driver then a secretary. died in 2022 at the age of 70.

    “We feel completely helpless,” she continues.

    Especially since his father, his aunt and his cousin still live on this street. "Where could this come from? What is intriguing is that all these cases occurred within a few years, or even a few months of each other."
    ...
    Céline Hussonnois-Alaya
    Journalist BFMTV



    ------------------------------------------------------------------------

    Charcot disease: Public Health France seized after five cases in the same village in the Somme

    The Hauts-de-France Regional Health Agency contacted the national public health agency to “determine whether there is in fact a statistical excess of diseases in the observed population” and, if so, the reasons for this excess. .

    Article written byfranceinfo with AFP
    France Televisions
    Publishedon 01/22/2024 8:31 p.m.

    Public Health France was contacted by the Hauts-de-France Regional Health Agency (ARS) after the death of five people suffering from Charcot's disease in two streets in the village of Saint-Vaast-en-Chaussée (Somme) , north of Amiens. “Five cases of residents, living in the same street or in a perpendicular street for one of them, having contracted the disease between 2007 and 2022” have been confirmed, the ARS underlined to AFP. The institution clarified that it was not aware of "other cases declared in the municipality" .

    The ARS contacted the national public health agency to "determine whether there actually exists a statistical excess of diseases in the observed population" and, if this excess exists, "to determine whether there are one or more local causes for this grouping of cases, other than chance, on which it is possible to act,” Public Health France told AFP, confirming information from Courrier Picard . At this stage, "the investigations are underway" and have "first of all the objective of carefully documenting the cases" , an "indispensable" step in the investigation of the cluster.

    “Unusual” situations of grouped cases

    Amyotrophic lateral sclerosis (ALS), another name for Charcot disease, causes progressive paralysis of the muscles, creating a state of confinement in the patient, and generally causes death within three years. To date, there is no effective neuroprotective treatment. Some “unusual” situations of grouped cases of ALS “have been reported to Public Health France over the last 10 years, without it being possible at this stage to hypothesize a common cause for the different cases” .

    For Pierre-François Pradat, neurologist at the Pitié-Salpêtrière hospital (APHP) and co-president of the scientific council of the Association for Research on ALS (ARSLA), clusters are "frequent" and linked to " chance", he puts things into perspective to AFP. According to him, five cases since 2007 "this is not a gigantic excess incidence given a disease that is still quite common", he adds, specifying that the disease was of genetic origin for 10% of patients.

    "Safety and security don't just happen, they are the result of collective consensus and public investment. We owe our children, the most vulnerable citizens in our society, a life free of violence and fear."
    -Nelson Mandela

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