The inhabitants of Montchavin in the French Alps knew something was killing them. They just didn’t know what.
Some wondered whether lead from a disused mine in the village had leaked into the water supply. Others thought mobile phone masts were to blame. One woman believed the area was cursed.
Between 1991 and 2019, 16 people – almost a tenth of the area’s 200-strong population – were diagnosed with amyotrophic lateral sclerosis, a fatal condition more commonly known as motor neurone disease (MND).
In other words, if you lived in Montchavin, you were 20 times more likely to contract MND than anywhere else in Europe.
This hideous disease causes the body’s nervous system to shut down, slowly paralysing the patient until they are unable to breathe. Tragically, there is no cure or treatment.
As the cases piled up and the death toll rose, French scientists tried to track down what was causing this unprecedented cluster. Finally, in 2019, they stumbled across a shocking possible conclusion.
Could it be that a rare toxic mushroom – considered a delicacy in Montchavin – was to blame?
In the latest twist to this extraordinary story, the Mail can today reveal that the only known MND patient still alive from the Montchavin area is a British man named Steve Isaac.
Steve Isaac (right) with his son Fraser, pictured on holiday shortly after Steve was diagnosed with motor neurone disease
Steve pictured again in 2015 with his son Fraser who had begun recording his deterioration
In 2009, Steve was given two years to live. But, like the late scientist Stephen Hawking who lived with MND for 55 years, he has defied medical science despite being fully paralysed except for his eyes. But more on Steve later.
For now, this is the remarkable story of Montchavin: the cursed village over-run by a ‘plague’ for which there was seemingly no explanation.
Dr Valerie Foucault served as the only GP in her adopted hometown of Montchavin since the early 1990s.
The first patient she knew to have MND was diagnosed in 1991 and the second not until nine years later. The two cases, while tragic, seemed wholly unrelated.
However, by 2009, Dr Foucault knew of five patients with the disease. The fifth was a lightbulb moment. Something clearly wasn’t right.
‘I just couldn’t handle it any more,’ she told the Mail this week. ‘I get attached to my patients and it affects me personally.
‘No one wants to see their wife in a wheelchair or their father paralysed, communicating via a computer. But there was nothing I could do for them.’
Not only was the emotional burden taking its toll, but Dr Foucault understood perfectly that five cases out of 200-odd patients was anything but normal. ‘I looked at the records and thought, ‘this is just not possible’. I called other GPs in the valley but I was the only one with these cases.’
Dr Valerie Foucault served as the only GP in her adopted hometown of Montchavin since the early 1990s
Montchavin, a small village in the French Alps, 1250 meters high and with a population of around 200 people
There is no single cause of MND, but up to 90 per cent of cases are attributed to environmental factors, with the other 10 per cent being genetic.
Dr Foucault knew she had to identify what it was in Montchavin that was causing this terrible disease or more people would die.
She reached out to the regional health authority for help but, initially, was dismissed out of hand.
‘They told me there was a cluster of cases, but that it happens sometimes and there was nothing to be done about it,’ she recalled.
Indeed, clusters of MND do occur. In the Australian city of Wagga Wagga, cases are seven times higher than the national average. A lakeside village in New Hampshire recently reported cases up to 25 times higher than the US national average.
In 2010, a further three people were diagnosed around Montchavin – taking the total to eight. This cluster was surely no coincidence.
The anomalies piqued the interest of Dr Emmeline Lagrange, a neurologist at the Grenoble Alpes University Hospital – an hour’s drive from Montchavin.
Having secured government support, Dr Lagrange hastily assembled a team of researchers to begin what would become a gruelling ten-year investigation.
In 2009, Dr Foucault had five patients who had been diagnosed with the disease
Dr Foucault knew she had to identify what was causing this terrible disease or more people would die
‘The first investigations were carried out on the water networks, the reservoirs, the distribution system,’ local mayor Jean-Luc Boch said.
‘There were no symptoms, no signs, no trace whatsoever of the possibility of infection through the water. Then we looked at whether the incineration plants could be the cause. Everything was analysed.’
When Mr Boch says everything, he means everything. Soil from residents’ gardens was examined for toxins and heavy metals. Radon meters were brought into homes to test for radioactive gas emissions.
Further up the mountain, samples of the artificial snow used to prolong the skiing season were taken off to laboratories.
Hunks of wood – repurposed from old train carriages to build the village infrastructure – were chipped off and forensically scrutinised.
Every resident was asked to fill out a form – so long it took some three hours to complete – detailing their diet, work, habits, hobbies and everything in between. What on earth, thought Dr Lagrange, did all the patients have in common?
One early theory suggested that the villagers may have been inbred over decades and therefore share a common gene that causes MND.
This was ruled out when it was discovered that not a single victim had a parent, grandparent or even great-grandparent who had been affected.
The scientists had come up blank. Years passed and cases rose into double figures before a major breakthrough in 2017, courtesy of US-based neurologist Dr Peter Spencer.
Researchers worked for years in Montchavin to try and work out the cause of the disease
Samples of the artificial snow used to prolong the skiing season were taken off to laboratories
He told the Mail: ‘Whether in the jungles of New Guinea, an isolated Pacific island, or the pristine ski slopes of Montchavin, medical research is directed at identifying possible environmental triggers of brain diseases.’
Indeed, Dr Spencer had his eye on one particular ‘environmental trigger’: mushrooms.
Having previously investigated an MND cluster on the Pacific island of Guam, where the consumption of cyad seeds was to blame, Dr Spencer knew some mushrooms contained toxins capable of manipulating DNA and destroying the nervous system.
He joined Dr Lagrange’s team in 2018 and carried out a fresh series of interviews about the community’s consumption of mushrooms. One thing immediately stood out. The majority of people with MND reported eating the notorious ‘false morel’ mushroom, with half saying it had at some point made them acutely sick.
What’s more, not one of the 48 healthy ‘control’ subjects reported having tried the mushroom. The scientists took a number of specimens and concluded their research in June 2021 with the publication of a ground-breaking paper that claimed: ‘Fungal genotoxins could induce degeneration of motor neurons.’
However, Dr Spencer stressed to the Mail that, while the mushroom had been shown to be ‘associated’ with the disease, further research was required to prove a ’cause-effect relationship’.
So what do the people of Montchavin believe?
Edmond Clement-Guy was in his early 60s when he shot himself in the head following a diagnosis of MND 17 years ago.
The majority of people with MND reported eating the notorious ‘false morel’ mushroom
Half of the residents with MND had said trying the mushroom had even made them sick
His niece, 70-year-old Ginette Blanchet, still lives in Montchavin. She said: ‘He did indeed eat these mushrooms.
‘But he didn’t live to excess. Didn’t drink too much. What all the sufferers had in common was that they were out in nature a lot. A lot of them were hunters, woodcutters and also ski instructors.
‘We all wondered what it could be in our village that was causing all this. Could it be the water? The game the hunters brought in? We even thought it might be the fireworks we set off to celebrate Bastille Day. But when we found out it was likely the mushrooms, we were shocked.’
More shocked than most was Edmond’s sister, 76-year-old Mireille Marchand. ‘I used to eat them every spring for at least 20 years,’ she said cheerily in the kitchen of a wooden chalet.
‘I’m not convinced they are the cause of the disease,’ she added in a suddenly hushed tone. ‘I think it remains a mystery.’
Despite her reservations, Mireille has stopped eating false morel mushrooms. She continues, however, to forage for other fungi and has four large trays of verpa bohemica mushrooms drying out – which they must do for six weeks – by her open window.
‘I put them in omelettes, usually,’ she says. ‘But you need to cook these ones for 20 minutes first or they can make you very sick. My brother almost died when he cooked them only for a couple of minutes.’
Despite the risks, Mireille – for whom foraging is a way of life – isn’t worried. ‘The false morels are rounder with less of a stalk. And they’re a rusty colour. I’m not worried about picking the wrong ones.’
Herve Fino,64, has lived in Montchavin for 43 years and has become an expert at picking out the right mushrooms
Scientists concluded their research in June 2021 with the publication of a ground-breaking paper about the links between mushrooms and the disease
The Mail went foraging with 64-year-old Herve Fino, who has lived in Montchavin for 43 years.
‘We’ve got the Italians driving over and stealing our mushrooms now,’ he complained.
‘Some people have started slashing their tyres so now they leave lookouts by their cars and forage higher up the mountains where we can’t see them.’
Herve bends down to run his palm through a patch of cowslip. ‘Luckily, I can tell a false morel straight away. But it’s too dry today. You need rain to bring mushrooms out.’
Fifteen years ago, Herve’s friend, Marie Paul, died in her 40s from MND. According to Herve, however, she never knowingly ate false morels.
‘I have my doubts that it’s the mushrooms that caused it. Maybe she mistook a false morel for something else… but I think it was more to do with the stress from her failing restaurant. I just don’t know.’
What about Edmond Clement-Guy? ‘He once fell into freezing cold water up to his neck,’ Herve said. ‘Maybe that is what caused his condition.’
One thing is clear, despite the conclusions of the research team from Grenoble University, the residents of Montchavin seem keen to blame almost anything but mushrooms. Does this apparently charming village hold an even deeper secret?
Perhaps Steve Isaac – the last remaining MND sufferer from the Montchavin cluster – could shed further light on this mystery?
Residents of Montchavin seem keen to blame almost anything but mushrooms
The now 66-year-old Steve bought a property in Montchavin with his wife, Debbie, and two children in 2007.
‘We’re a skiing family and love entertaining,’ he told the Mail, typing the words on a computer system by fluttering his eyes. ‘Running a catered chalet combines our passions and makes a living. Montchavin is gorgeous and our chalet has impressive views over the valley and the mountains beyond.’
When I ask Steve whether he believes the false morel mushroom is to blame, he gives a surprising answer: ‘To my knowledge I’ve never had false morel mushrooms. And I don’t know why so many people got the disease in the area. Perhaps it’s just a random anomaly.’
In the past six years, during which time the locals have reportedly stopped eating false morels, there have been no further diagnoses of MND. But, as Dr Spencer pointed out, correlation does not always mean causation.
And, after hearing Steve Isaac’s story, I cannot help but feel that the mystery of the Montchavin cluster goes even deeper than fungi. As deep, perhaps, as the coal mines this village was built to support.
The eerie Montchavin mines were declared ‘permanently closed’ in 1995 – around the same time the first cases of MND were declared in the village.
Perhaps – like the mushrooms – that is just another uncomfortable coincidence.
Additional reporting: Rory Mulholland
