Title: Western Pacific amyotrophic lateral sclerosis and parkinsonism–dementia complex
Long Title: IUPAC Gold Book - Western Pacific amyotrophic lateral sclerosis and parkinsonism–dementia complex
DOI: 10.1351/goldbook.12179
Status: current

Definition
Neurodegenerative disease observed in the islands of Guam, Indonesia, Japan and Papua New Guinea, showing combinations of characteristics of amyotrophic lateral sclerosis, atypical parkinsonism with dementia, or dementia alone that may present sequentially during life.

Notes
1) Neurotoxins of the cycad plant, such as β-N-methylamino-L-alanine (L-BMAA) produced by symbiotic cyanobacteria, and methylazoxymethanol (MAM), are suspected causative agents.
2) Polyproteinopathy, notably tauopathy, is a common causative feature underlying the different clinical manifestations.

Related Terms
- Neurodegenerative disease: https://goldbook.iupac.org/terms/view/11926
- Neurotoxins: https://goldbook.iupac.org/terms/view/11952
- dementia: https://goldbook.iupac.org/terms/view/11704
- parkinsonism: https://goldbook.iupac.org/terms/view/11998
- tauopathy: https://goldbook.iupac.org/terms/view/12121

Source
- PAC, 2015, 87, 841. 'IUPAC Glossary of terms used in neurotoxicology (IUPAC Recommendations 2015)' on page 916 (https://doi.org/10.1515/pac-2015-0103)

Other Outputs
- html: https://goldbook.iupac.org/terms/view/12179/html
- json: https://goldbook.iupac.org/terms/view/12179/json
- xml: https://goldbook.iupac.org/terms/view/12179/xml

Citation: Citation: 'Western Pacific amyotrophic lateral sclerosis and parkinsonism–dementia complex' in IUPAC Compendium of Chemical Terminology, 5th ed. International Union of Pure and Applied Chemistry; 2025. Online version 5.0.0, 2025. 10.1351/goldbook.12179
License: The IUPAC Gold Book is licensed under Creative Commons Attribution-ShareAlike CC BY-SA 4.0 International (https://creativecommons.org/licenses/by-sa/4.0/) for individual terms.
Collection: If you are interested in licensing the Gold Book for commercial use, please contact the IUPAC Executive Director at executivedirector@iupac.org .
Disclaimer: The International Union of Pure and Applied Chemistry (IUPAC) is continuously reviewing and, where needed, updating terms in the Compendium of Chemical Terminology (the IUPAC Gold Book). Users of these terms are encouraged to include the version of a term with its use and to check regularly for updates to term definitions that you are using.
Accessed: 2026-05-14T12:26:50+00:00