Scientific Committee
Title:
Environmental Neurology and Primary Disease Prevention: Sporadic ALS/MNDBiography
60+ years of basic, applied and clinical neurotoxicology research, initially in London (Institute of Neurology), subsequently in New York City (Albert Einstein College of Medicine) and presently in Oregon (OHSU). Research support received from the U.S. National Institutes of Health; National Science Foundation; Center for Disease Control & Prevention, National Institute for Occupational Safety & Health: U.S. Department of Veterans Affairs; and by several private organizations and corporate entities. Recipient of numerous national and international awards, with honorary professorships in Africa (n=1), Asia (5) and South America (1).
Research Interests: Global Environmental Neurology, Neurotoxicology, Neurodegenerative Disorders.
Abstract
Most cases of motor neuron disease (MND)/amyotrophic lateral sclerosis (ALS) are sporadic and unassociated with mutant genes. Disease is therefore likely to result from prior exposure to an environmental trigger, whether chemical, physical or biological. Epidemiological study of ALS clusters and conjugal cases, ideally with an examination of lifetime exposomes, carries the promise of discovering disease etiology. Application of these principles is illustrated first by the former very high incidence of ALS among the Chamorro people of Guam, where the disease slowly disappeared after World War II when food reliance on the poisonous seed of cycad plants was progressively replaced by a Western diet. Guam ALS was strongly associated with residual cycasin in washed cycad seed used for flour, which was daily consumed in the form of tortillas (tatiyas) [Spencer et al., J. Neurol. Sci. 2020;419:117185]. Related to methylazoxymethanol, the aglycone metabolite of cycasin, is the compound methylhydrazine, the metabolite of gyromitrin from Gyromitra esculenta (False Morel). A subsequent case control study of a cluster of European ALS cases in the French Alps revealed a strong association with a history of False Morel consumption [Lagrange E. et al. eNeurologicalSci. 2024;35:100502]. Avoidance of exposure to such mycotoxins/phytotoxins should result in primary disease prevention and, thus, maintenance of brain health. Furthermore, hydrazinic compounds, whether of natural or anthopogenic origin, are suspect etiological agents for MND/ALS [Spencer et al. Front. Neurosci. 2023:17].
Audience Take-Aways:
1. A progressive and uniformly fatal neurodegenerative disease is preventable.
2. Close enquiry of the exposure history of sALS patients can reveal etiologic clues.
3. The foregoing environmental neurology principles should be incorporated into teaching and research in Neurology.
