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- W3203838843 endingPage "120021" @default.
- W3203838843 startingPage "120021" @default.
- W3203838843 abstract "Recognized worldwide as an unusual overlap syndrome, Parkinsonism and motor neuron disease, with or without dementia, is best exemplified by the former high-incidence clusters of Amyotrophic Lateral Sclerosis and Parkinsonism-Dementia Complex (ALS/PDC) in Guam, USA, in the Kii Peninsula of Honshu Island, Japan, and in Papua, Indonesia, on the western side of New Guinea. Western Pacific ALS/PDC is a disappearing neurodegenerative disorder with multiple and sometime overlapping phenotypes (ALS, atypical parkinsonism, dementia) that appear to constitute a single disease of environmental origin, in particular from exposure to genotoxins/neurotoxins in seed of cycad plants (Cycas spp.) formerly used as a traditional source of food (Guam) and/or medicine (Guam, Kii-Japan, Papua-Indonesia). Seed compounds include the principal cycad toxin cycasin, its active metabolite methylazoxymethanol (MAM) and a non-protein amino acid β-N-methylamino-L-alanine (L-BMAA); each reproduces components of ALS/PDC neuropathology when individually administered to laboratory species in single doses perinatally (MAM, L-BMAA) or repeatedly for prolonged periods to young adult animals (L-BMAA). Human exposure to MAM, a potent DNA-alkylating mutagen, also has potential relevance to the high incidence of diverse mutations found among Guamanians with/without ALS/PDC. In sum, seven decades of intensive study of ALS/PDC has revealed field and laboratory approaches leading to discovery of disease etiology that are now being applied to sporadic neurodegenerative disorders such as ALS beyond the Western Pacific region. This article is part of the Special Issue Parkinsonism across the spectrum of movement disorders and beyond edited by Joseph Jankovic, Daniel D. Truong and Matteo Bologna." @default.
- W3203838843 created "2021-10-11" @default.
- W3203838843 creator A5076619105 @default.
- W3203838843 date "2022-02-01" @default.
- W3203838843 modified "2023-10-01" @default.
- W3203838843 title "Parkinsonism and motor neuron disorders: Lessons from Western Pacific ALS/PDC" @default.
- W3203838843 cites W1518040978 @default.
- W3203838843 cites W1910374374 @default.
- W3203838843 cites W1966962735 @default.
- W3203838843 cites W1967195211 @default.
- W3203838843 cites W1973066999 @default.
- W3203838843 cites W1973237516 @default.
- W3203838843 cites W1977144473 @default.
- W3203838843 cites W1978168248 @default.
- W3203838843 cites W1980046499 @default.
- W3203838843 cites W1987784344 @default.
- W3203838843 cites W1993776743 @default.
- W3203838843 cites W1994925296 @default.
- W3203838843 cites W1994948661 @default.
- W3203838843 cites W1996140948 @default.
- W3203838843 cites W1996572616 @default.
- W3203838843 cites W1997188060 @default.
- W3203838843 cites W2004004906 @default.
- W3203838843 cites W2004682018 @default.
- W3203838843 cites W2008317554 @default.
- W3203838843 cites W2016150506 @default.
- W3203838843 cites W2020174505 @default.
- W3203838843 cites W2025425510 @default.
- W3203838843 cites W2027903092 @default.
- W3203838843 cites W2036444151 @default.
- W3203838843 cites W2037470187 @default.
- W3203838843 cites W2037731224 @default.
- W3203838843 cites W2040426212 @default.
- W3203838843 cites W2041992717 @default.
- W3203838843 cites W2043111031 @default.
- W3203838843 cites W2045460240 @default.
- W3203838843 cites W2046746252 @default.
- W3203838843 cites W2048328872 @default.
- W3203838843 cites W2054339810 @default.
- W3203838843 cites W2054839608 @default.
- W3203838843 cites W2056338504 @default.
- W3203838843 cites W2060134039 @default.
- W3203838843 cites W2064572695 @default.
- W3203838843 cites W2065343408 @default.
- W3203838843 cites W2067933131 @default.
- W3203838843 cites W2072711194 @default.
- W3203838843 cites W2073222492 @default.
- W3203838843 cites W2077083529 @default.
- W3203838843 cites W2077131862 @default.
- W3203838843 cites W2079086843 @default.
- W3203838843 cites W2079408121 @default.
- W3203838843 cites W2081689111 @default.
- W3203838843 cites W2084541096 @default.
- W3203838843 cites W2087574528 @default.
- W3203838843 cites W2092929009 @default.
- W3203838843 cites W2095617813 @default.
- W3203838843 cites W2106206610 @default.
- W3203838843 cites W2110097095 @default.
- W3203838843 cites W2113491588 @default.
- W3203838843 cites W2114088312 @default.
- W3203838843 cites W2114594510 @default.
- W3203838843 cites W2117756648 @default.
- W3203838843 cites W2119494689 @default.
- W3203838843 cites W2125287392 @default.
- W3203838843 cites W2125300464 @default.
- W3203838843 cites W2128536162 @default.
- W3203838843 cites W2129686157 @default.
- W3203838843 cites W2134181639 @default.
- W3203838843 cites W2137077630 @default.
- W3203838843 cites W2144977714 @default.
- W3203838843 cites W2147231862 @default.
- W3203838843 cites W2148407847 @default.
- W3203838843 cites W2154825122 @default.
- W3203838843 cites W2157999333 @default.
- W3203838843 cites W2159300760 @default.
- W3203838843 cites W2163864270 @default.
- W3203838843 cites W2166650045 @default.
- W3203838843 cites W2167611139 @default.
- W3203838843 cites W2168539226 @default.
- W3203838843 cites W2285664856 @default.
- W3203838843 cites W2309442902 @default.
- W3203838843 cites W2318730042 @default.
- W3203838843 cites W2397460217 @default.
- W3203838843 cites W2564558365 @default.
- W3203838843 cites W2591335640 @default.
- W3203838843 cites W2619782882 @default.
- W3203838843 cites W2622679763 @default.
- W3203838843 cites W2752529139 @default.
- W3203838843 cites W2756127351 @default.
- W3203838843 cites W2759404612 @default.
- W3203838843 cites W2765183304 @default.
- W3203838843 cites W2789293490 @default.
- W3203838843 cites W2791391969 @default.
- W3203838843 cites W2905489823 @default.
- W3203838843 cites W2930781493 @default.
- W3203838843 cites W2948741248 @default.
- W3203838843 cites W2989702225 @default.
- W3203838843 cites W2990007946 @default.