https://doi.org/10.1351/goldbook.12179
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:
- 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.
- Polyproteinopathy, notably tauopathy, is a common causative feature underlying the different clinical manifestations.