Montchavin is a picturesque village in the French Alps where many are employed in the skiing industry. The region is home to Paradiski, one of the world's largest ski resorts. Thousands of people visit every year, but Montchavin itself only has a couple hundred permanent residents. In 2009, a woman from Montchavin was referred to neurologist Emmeline Lagrange, who diagnosed amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease. Dr. Lagrange contacted the Montchavin general practitioner, who told her he was well aware of ALS, because he knew of three other cases in the village.
Lagrange was puzzled, because ALS is fairly rare, with two or three new cases diagnosed per 100,000 people in a year. She eventually uncovered 16 cases of ALS in Montchavin, an extraordinary cluster of diagnoses. The patients were not all related, and several came from other countries, although they had lived in Montchavin for years. Of 12 patients who had blood tests, none had genetic markers for ALS susceptibility. Was it an environmental pollutant? Many possibilities were investigated, but no risk factor was identified as being common to all the patients. However, a 2017 report mentioned that six of the patients reported eating wild mushrooms. Lagrange spotted that detail and went back to the 13 patients she was studying. She discovered that all of them had eaten Gyromitra esculenta, a delicious wild mushroom that is so toxic that it is illegal to sell them in France. Read about the investigation, and the state of research into the causes of ALS at Knowable magazine.
(Image credit: Audrey OT)
