We know the person responsible for Charcot’s disease in Savoie

Charcot’s disease or sclerosis amyotrophic lateral (SALAD) is a neurodegenerative disease rare. In France, there are about 2.5 cases per 100,000 inhabitants. But in a hamlet in the French Alps, it is much more common. Between 1990 and 2018, 14 cases of Charcot disease were diagnosed in unrelated people. An exception that prompted several French doctors to launch an epidemiological investigation. With the help of an American colleague from the University of Oregon, the person responsible was identified a few weeks ago.

A fungus responsible for Charcot disease

The genetic trail ruled out from the start, the doctors analyzed the soil, the water, theair or the vegetables grown by the sick, without success. A link with lifestyle habits has also been considered, some patients suffering from Charcot’s disease were smokers or engaged in high-intensity athletics. Again, nothing.

The investigation takes a new breath thanks to Peter S. Spencer, neurologist at the University of Oregon, who suggests they look on the side of the food. French doctors end up finding a point in common between the 14 Savoyard cases, the consumption of mushroom Gyromitra gigas Where false morel.

False morels are common in highland forests and are poisonous, even fatal, to humans if eaten raw. This mushroom is banned for sale in France, but still regularly picked and prepared in mountain regions. Cooking reduces its toxicity, but it is not recommended to eat it several times in a row or serve it to children. The 14 patients with Charcot’s disease in Savoie repeatedly consumed false morels before the onset of Charcot’s disease. Half of them developed sudden and acute symptoms after eating these mushrooms. The toxins of the false morel would be at the origin of the degeneration of motorboats typical of Charcot’s disease.