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The western Pacific form of ALS has been of interest for over 50 years because its incidence, prevalence, and mortality rates were initially 50 to 100 times those of sporadic ALS. The male:female ratio approximated 2:1, the median age at onset was 44 years, familial aggregation was recognized, and ALS was associated frequently with a parkinsonism/dementia complex (PDC). Three foci of western Pacific ALS/PDC were identified: in Guam, in Japan, and in western New Guinea.1 More recently, the frequency of western Pacific ALS has declined.2 The implication—that there was a temporary exposure to an environmental risk factor, possibly in a genetically susceptible population—has fueled decades of research and speculation. Several factors supported an environmental etiology for western Pacific ALS/PDC. A deficiency of essential minerals was excluded.3 Thus, the leading hypothesis remained exposure to an environmental toxin, presumed to have peaked during World War II. According to this hypothesis, individuals exposed to higher doses of the toxin developed ALS at a relatively early age, and individuals exposed to a lesser dose developed neurodegenerative disease at a later date.
Marjorie Whiting, a nutritionist and anthropologist who …
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