Serial cases of Charcot disease in a single village street

In Saint-Vast-en-Chaussy, a small village of 500 inhabitants on the Somme, what terrible disaster is on the rue du Château d’Eau? In just fifteen years, five residents of this street have developed Charcot disease, an incurable and fatal neurodegenerative disease. According to the BFM, the city’s mayor contacted the regional health agency (ARS) about this unusually high number of cases, and Public Health France launched an investigation.

“Amyotrophic lateral sclerosis (ALS), also known as Charcot disease, is a severe neurodegenerative disease that results in progressive paralysis of the muscles involved in voluntary motor skills,” we explain on Inserm’s website. There is no cure for ALS and patients have a particularly poor prognosis, with a life expectancy of three to five years.

A particularly high incidence of disease

According to the Hospice Civil de Leon (HCL) website, the annual incidence of Charcot disease is “2.7 new cases per 100,000 inhabitants” and “the risk of being affected by ALS is estimated at a scale of 1/350 for life. for males and 1/400 for females”. Five cases in fifteen years in this street in this small town fifteen kilometers north-west of Amiens is a cause for concern as it is 24 times higher than the incidence reported by HCL.

Especially since Charcot disease finds its origin in family history in only 10% of cases, refer to the Hospice Civil de Lyon. “90% of patients with ALS, however, have no family history and are said to be ‘sporadic'”, it mentioned, adding that “environmental factors” may also come into play.

ARS, however, is not there yet. The health police must first determine whether these figures actually represent a “statistical increase”. ARS explained to BFM that it would be necessary to find out if there were “one or more local causes”, if necessary.

Mikael Libert