TY - JOUR
T1 - An amyotrophic lateral sclerosis hot spot in the French Alps associated with genotoxic fungi
AU - Lagrange, E.
AU - Vernoux, J. P.
AU - Reis, J.
AU - Palmer, V.
AU - Camu, W.
AU - Spencer, P. S.
N1 - Funding Information:
We thank the patients and inhabitants of Montchavin, notably Mayor Mr. M. Girod, who provided assistance across 10 years of research on ALS in his community, as did our colleague Dr. V. Foucault, General Practitioner. We also acknowledge with gratitude “la longue route des patients SLA”. Valuable assistance was also provided by Dr. J.L. Chappert (INVS SFP), by Prof. C. Casellas in regard to epidemiology speciation, by Prof. C. Andary and Prof. P. Saviuc in regard to mushroom speciation and information on acute toxicity, by Prof. S. Banack, and Prof L. Brient in regard to cyanobacteria concentration and analysis, by Prof. A. Maître in regard to heavy metal speciation, and Dr. J. Boutonnat in regard to autopsy speciation. Prof. V. Bonneterre and Dr. G. Besson are thanked for moral support, Prof. Gunther Sebastian (Greifswald University, Germany), Prof. Mélanie Bourjot and Prof. Eric Marchioni (University of Strasbourg, France) for assistance, and Prof. Glen Kisby (Western University of Sciences, USA) for discussion.
Publisher Copyright:
© 2021 Elsevier B.V.
PY - 2021/8/15
Y1 - 2021/8/15
N2 - Between 1990 and 2018, 14 cases of amyotrophic lateral sclerosis (ALS) were diagnosed in residents of, and in visitors with second homes to, a mountainous hamlet in the French Alps. Systematic investigation revealed a socio-professional network that connected ALS cases. Genetic risk factors for ALS were excluded. Several known environmental factors were scrutinized and eliminated, notably lead and other chemical contaminants in soil, water or home-grown vegetation used for food, radon and electromagnetic fields. Some lifestyle-related behavioral risk factors were identified: Prior to clinical onset of motor neuron disease, some patients had a high degree of athleticism and smoked tobacco. Recent investigations on site, based on a new hypothesis, showed that all patients had ingested wild mushrooms, notably poisonous False Morels. Half of the ALS cohort reported acute illness following Gyromitra gigas mushroom consumption. This finding supports the hypothesis that genotoxins of fungal origin may induce motor neuron degeneration.
AB - Between 1990 and 2018, 14 cases of amyotrophic lateral sclerosis (ALS) were diagnosed in residents of, and in visitors with second homes to, a mountainous hamlet in the French Alps. Systematic investigation revealed a socio-professional network that connected ALS cases. Genetic risk factors for ALS were excluded. Several known environmental factors were scrutinized and eliminated, notably lead and other chemical contaminants in soil, water or home-grown vegetation used for food, radon and electromagnetic fields. Some lifestyle-related behavioral risk factors were identified: Prior to clinical onset of motor neuron disease, some patients had a high degree of athleticism and smoked tobacco. Recent investigations on site, based on a new hypothesis, showed that all patients had ingested wild mushrooms, notably poisonous False Morels. Half of the ALS cohort reported acute illness following Gyromitra gigas mushroom consumption. This finding supports the hypothesis that genotoxins of fungal origin may induce motor neuron degeneration.
KW - False morels
KW - Genotoxins
KW - Southeast Central France, Auvergne-Rhône-Alpes
KW - Sporadic amyotrophic lateral sclerosis
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U2 - 10.1016/j.jns.2021.117558
DO - 10.1016/j.jns.2021.117558
M3 - Article
C2 - 34216974
AN - SCOPUS:85109199231
SN - 0022-510X
VL - 427
JO - Journal of the Neurological Sciences
JF - Journal of the Neurological Sciences
M1 - 117558
ER -