2024-01-24 05:15:00
Amyotrophic lateral sclerosis (ALS), better known as Charcot disease, affects 8,000 patients in France, according to the Association for Research on ALS.
Its annual incidence amounts to 2.7 cases per 100,000 inhabitants. It is a rare neurodegenerative pathology which is characterized by the destruction of the neurons responsible for motor skills, the motor neurons.
The astonishing story of a village in the Somme
In the Somme, the village of Saint-Vaast-en-Chaussée is wondering after the deaths of 5 people since 2009 from Charcot’s disease. Residents of the same town, these people also all lived in the same street.
The Mayor of the town contacted the Hauts-de-France Regional Health Agency to alert it. “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 thus been confirmed”, notes to Destination Santé the ARS, which continues: “the study of the report allowed the ARS to confirm a high number of cases of ALS in this municipality”.
Public Health France was contacted to determine whether there is a Charcot disease cluster in Saint-Vaast-en-Chaussée.
“The epidemiological objectives of responding to a suspected cluster are to determine whether there is in fact a statistical excess of diseases in the observed population and to determine whether there are one or more local causes for this grouping of cases, other than the chance, on which it is possible to act,” explains the national public health agency.
“At this stage, investigations are ongoing. Their first objective is to carefully document the cases. This documentation is an essential first step in investigating the cluster,” the agency continues.
The precedent of Montchavin, in Savoie
“It is not exceptional in itself but it is nevertheless a source of high incidence”, notes for Destination Santé, Timothée Lenglet, neurologist at the Pitié-Salpêtrière hospital.
“Having several cases in a limited geographical area, we have already seen that. In the same street, in the same building, even two cases within a couple. This is often attributed to chance, but we know that there can be environmental factors that can influence the risk of developing an ALS-type illness. And this is why it is important to be concerned about this type of situation because investigations will be able to shed light on factors that we do not yet know.”
The specialist recalls that in 2021, an abnormally high number of victims of Charcot disease – 14 cases per 900 inhabitants in 22 years – was observed in Savoie, in the village of Montchavin.
An epidemiology study, the results of which were published in the Journal of Neurological Sciences, showed that the consumption of a mushroom, the giant gyromitre, was responsible for the cluster.
Multifactorial causes
According to Inserm, no environmental factor has been formally highlighted. However, “tobacco, high-level sport, exposure to pesticides, heavy metals or even a toxin present in certain algae (BMAA cyanotoxin) are suspected”.
A genetic mutation would also be involved, i.e. “the random (and not transmitted) mutation of a causal gene or of one or more susceptibility genes (which would increase the risk of occurrence of the disease)”.
“These environmental factors increase the risk of developing a neurological disease, associated with a personal susceptibility factor,” adds Dr. Lenglet. And we do not know how to clearly establish the role of these environmental factors in the occurrence of the disease compared to an innate factor.”
Symptomatic management
The disease usually occurs between the ages of 50 and 70. ALS is a progressive pathology that leads to death within 3 to 5 years following the first signs. It is most often damage to the respiratory muscles that leads to the patient’s death.
It can first manifest itself as difficulty articulating or swallowing, in 30% of cases. Otherwise, the disease may begin “by weakness and discomfort in an arm, leg or hand that manifests itself at the beginning of the disease.
Then, the disease progresses with:
- Contractures, stiffness of muscles and joints;
- muscle wasting and coordination problems hamper walking, grasping objects and difficulty swallowing or articulating
- At an advanced stage of the disease, the respiratory muscles are no longer able to perform their function.
To date, there are no curative treatments for Charcot disease. The care is symptomatic and multidisciplinary: “technical assistance, physiotherapy and anti-spastic medications to counter motor disorders, muscle relaxants and analgesics for pain, management of malnutrition, speech therapy for speech and swallowing disorders, support psychological…”, lists Inserm.
To note : ALS is also familial in one in 10 cases.
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