Peter Spencer, PhD, FANA, FRCPath is Professor of Neurology and Occupational Health Sciences of Oregon Health & Science University (OHSU), where he has also served as founding director of the Center for Research on Occupational and Environmental Toxicology and the OHSU Global Health Center. Prior to joining OHSU in 1988, he was professor of Neuroscience, Neurology and Pathology (Neuropathology), and director, Institute of Neurotoxicology, of New York’s Albert Einstein College of Medicine, which he joined after leaving the Royal Free Hospital School of Medicine in 1971. Over the past 50 years, his research has focused mainly on the mechanisms underlying environmental neurological diseases, with heavy focus on self-limiting and progressive neurodegenerative disorders, notably Western Pacific ALS-PDC. His continuously funded research, which is published in >400 papers, has been recognized by numerous international awards and honorary professorial appointments.
Western Pacific amyotrophic lateral sclerosis and parkinsonism-dementia complex (ALS-PDC) is a familial and sporadic neurodegenerative disease featured neuropathologically by a tau-dominated polyproteinopathy. Over the past half-century in the hyperendemic communities of Guam, USA and Kii Peninsula, Japan, disease incidence has progressively declined as age of clinical onset has increased. Some Japanese investigators view ALS-PDC primarily as a genetic disease, with manifestation of clinical phenotypes modified by other genes or environmental factors . Similarly, on Guam, it is suggested that family histories with multi-incident pedigrees (ALS, atypical parkinsonism, dementia and PDC) are compatible with genetic transmission of each syndrome, while shifting environmental factors cause disease postponement [2,3]. My position is that ALS-PDC is dominated by an environmental aetiology , the chemical identity of which is relevant to, and can guide research on, the aetiology of related neurodegenerative disorders (e.g., ALS, progressive supranuclear palsy .